Orthognathic Surgery

1291
CHAPTER 31  Selected Complex Malformations that Frequently Require Maxillofacial Reconstruction: Evaluation & Treatment
F
G
Maxillary counter-
clockwise rotation
Mandibular counter-
clockwise rotation
E
E, Occlusal views before and after reconstruction. F, Articulated dental
casts that indicate analytic model planning. G, Lateral cephalometric radiographs before and after
reconstruction.
• Figure 31-12, cont’d 
References
Klippel–Feil Anomaly
1. Agris J, Dingman RO, Varon J: Correction
of webbed neck defects. Ann Plast Surg
2:299, 1983.
2. Baba H, Maezawa Y, Furusawa N, et al: The
cervical spine in the Klippel–Feil syndrome:
A report of 57 cases. Int Orthop 19:204,
1995.
3. Baird PA, Robinson GC, Buckler WS:
Klippel–Feil syndrome: A study of
mirror movements detected by
electromyography. Am J Dis Child
113:546, 1967.
4. Bauman GI: Absence of the cervical spine:
Klippel–Feil syndrome. JAMA 98:129, 1932.
5. Bavinck JN, Weaver DD: Subclavian artery
supply disruption sequence: Hypothesis of a
vascular etiology for Poland, Klippel–Feil,
and Moebius anomalies. Am J Med Genet
23:903, 1986.
6. Brill CB, Peyster RG, Keller MS, et al:
Isolation of the right subclavian artery with
subclavian steal in a child with Klippel–Feil
anomaly: An example of the subclavian
artery supply disruption sequence. Am J Med
Genet 26:933–940, 1987.
7. Brown MW, Templeton AW, Hodges FJ,
3rd: The incidence of acquired and
congenital fusions in the cervical spine. Am J
Roentgenol 92:1255, 1964.
8. Chandler FA: Webbed neck (pterygium
colli). Am J Dis Child 53:798, 1937.
9. Chemke J, Nisani R, Fischel RE: Absent
ulna in the Klippel–Feil syndrome: An
unusual associated malformation. Clin Genet
17:167, 1980.
10. Cohney BC: The association of cleft palate
with the Klippel–Feil syndrome. Plast
Reconstr Surg 31:179, 1963.

1292 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
11. Daniilidis J, Maganaris T, Dimitriadis A,
et al: Stapes gusher and Klippel–Feil
syndrome. Laryngoscopy 88:1178, 1978.
12. Da Silva EO: Autosomal recessive Klippel–
Feil syndrome. J Med Genet 19:130, 1982.
13. De Bruin M: Pterygium colli congenitum
(congenital webbing). Am J Dis Child
36:333, 1928.
14. Duncan PA: Embryologic pathogenesis of
renal agenesis associated with cervical
vertebral anomalies (Klippel–Feil phenotype).
Birth Defects Orig Artic Ser 13:91, 1977.
15. Erskine CA: An analysis of the Klippel–Feil
syndrome. Arch Pathol 41:269, 1946.
16. Everberg G, et al: Wildervanck syndrome:
Klippel–Feil syndrome associated with
deafness and retraction of the eyeball. Br J
Radiol 36:562, 1963.
17. Fietti VG, Jr, Fielding W: The Klippel–Feil
syndrome: Early roentgenographic
appearance and progression of the deformity:
A report of two cases. J Bone Joint Surg Am
58:891, 1976.
18. Foucar HO: Pterygium colli and allied
conditions. Can Med Assoc J 59:251, 1948.
19. Fragoso R, Cid-Garcia A, Hernandez A,
et al: Frontonasal dysplasia in the Klippel–
Feil syndrome: A new associated
malformation. Clin Genet 22:270, 1982.
20. Gardner WJ: Klippel–Feil syndrome,
iniencephalus, anencephalus, hindbrain
hernia, and mirror movements:
Overdistention of the neural tube. Childs
Brain 5:361, 1979.
21. Gienapp R von: Zur erbbiologie der
Klippel–Feilschen krankheit. Nervenarzt
21:74, 1950.
22. Gilmour JR: The essential identity of the
Klippel–Feil syndrome and iniencephaly.
J Pathol Bacteriol 53:117, 1941.
23. Ginrup PA, Ginrup L: Klippel–Feil
syndrome. Danish Med Bull 11:50, 1964.
24. Gorlin RJ, Cohen MM, Jr, Levin LS:
Syndromes of the head and neck, ed 3, New
York, 1990, Oxford University Press.
25. Gray SW, Romaine CB, Skandalakis JE:
Congenital fusion of the cervical vertebrae.
Surg Gynecol Obstet 118:373, 1964.
26. Guille JT, Miller A, Bowen JR, et al: The
natural history of Klippel–Feil syndrome:
Clinical, roentgenographic, and magnetic
resonance imaging findings at adulthood.
J Pediatr Orthop 15:17, 1995.
27. Gunderson CH, Greenspan RH, Glaser GH,
et al: The Klippel–Feil syndrome: Genetic
and clinical evaluation of cervical fusion.
Medicine 46:491, 1967.
28. Gunderson CH, Solitaire GB: Mirror
movements in patients with the Klippel–Feil
syndrome. Arch Neurol 18:675, 1968.
29. Heiner H: Uber die kombination von
medianer gaumenspalte und Klippel–Feil
syndrom. Dtsch Stomatol 10:92, 1960.
30. Helmi C, Pruzansky S: Craniofacial and
extracranial malformations in the Klippel–
Feil syndrome. Cleft Palate J 17:65, 1980.
31. Hensinger RN, Lang JE, MacEwen GD:
Klippel–Feil syndrome: A constellation of
associated anomalies. J Bone Joint Surg Am
56:1246, 1974.
32. Jarcho S, Levin PM: Hereditary
malformation of the vertebral bodies. Bull
Johns Hopkins Hosp 62:216, 1938.
33. Juberg RC, Gershank JJ: Cervical vertebral
fusion (Klippel–Feil) syndrome with
consanguineous parents. J Med Genet
13:246, 1976.
34. Klippel M, Feil A: Un cas d’absence des
vertebres cervicales. Nouv Iconogr Salpet
25:223, 1912.
35. Lauerman WC: Maurice Klippel. Spine
20:157, 1995.
36. Lowry RB: The Klippel–Feil anomalad as
part of the fetal alcohol syndrome. Teratology
16:53, 1977.
37. Luftman II, Weintraub S: Klippel–Feil
syndrome in a full-term newborn infant. NY
State J Med 51:2035, 1951.
38. Mahajan PV, Bharucha BA: Evaluation of
short neck: New neck length percentiles and
linear correlations with height and sitting
height. Indian Pediatr 31:1193, 1994.
39. McLay K, Maran AGD: Deafness and the
Klippel–Feil syndrome. J Otolaryngol 83:175,
1969.
40. Mecklenburg RS, Krueger PM: Extensive
genitourinary anomalies associated with
Klippel–Feil syndrome. Am J Dis Child
128:92, 1974.
41. Menick FJ, Furnas DW, Achauer BM:
Lateral cervical advancement flaps for the
correction of webbed-neck deformity. Plast
Reconstr Surg 73:223, 1984.
42. Moore WB, Matthews TJ, Rabinowitz R:
Genitourinary anomalies associated with
Klippel–Feil syndrome. J Bone Joint Surg Am
57:355, 1975.
43. Morrison SG, Perry LW, Scott LP, 3rd:
Congenital torticollis (Klippel–Feil
syndrome) and cardiovascular anomalies. Am
J Dis Child 115:614, 1968.
44. Mosberg WH: The Klippel–Feil syndrome:
Etiology and treatment of neurologic signs.
J Nerv Ment Dis 117:479, 1953.
45. Nagib MG, Larson DA, Maxwell RE, et al:
Neuroschisis of the cervical spinal cord in a
patient with Klippel–Feil syndrome.
Neurosurgery 20:629, 1987.
46. Nagib MG, Maxwell RE, Chou SN:
Identification and management of high-risk
patients with Klippel–Feil syndrome.
J Neurosurg 61:523, 1984.
47. Nagib MG, Maxwell RE, Chou SN:
Klippel–Feil syndrome in children: Clinical
features and management. Childs Nerv Syst
1:255, 1985.
48. Neidengard L, Carter TE, Smith DW:
Klippel–Feil malformation complex in fetal
alcohol syndrome. Am J Dis Child 132:929,
1978.
49. Nobel TP, Frawley JM: Klippel–Feil
syndrome: Numerical reduction of cervical
vertebrae. Ann Surg 82:728, 1925.
50. Nora JJ, et al: Klippel–Feil syndrome with
congenital heart disease. Am J Dis Child
102:858, 1961.
51. Palant DI, Carter BL: Klippel–Feil syndrome
and deafness: A study with polytomography.
Am J Dis Child 123:218, 1972.
52. Pizzutillo PD, Woods M, Nicholson L, et al:
Risk factors in Klippel–Feil syndrome. Spine
19:2110, 1994.
53. Posnick JC: Other craniofacial syndromes:
Klippel–Feil anomaly, neurofibromatosis,
Down syndrome, micro-ophthalmia,
hemifacial hyperplasia (hemi-hypertrophy).
In Posnick JC, editor: Craniofacial and
maxillofacial surgery in children and young
adults, Philadelphia, 2000, WB Saunders Co,
24, pp 503–527.
54. Raas-Rothschild A, Goodman RM,
Grunbaum M, et al: Klippel–Feil anomaly
with sacral agenesis: Additional subtype,
type IV. J Craniofac Genet Dev Biol 8:297,
1988.
55. Schwarze K: Zur frage des Klippel–Feilschen
fehlers der wirbelsule. Arch Orthop Unfallchir
41:47, 1941.
56. Shoul MJ, Ritvo M: Clinical and
roentgenological manifestations of
Klippel–Feil syndrome (congenital fusion of
the cervical vertebrae brevicollis): Report of
8 additional cases and review of the
literature. Am J Roentgenol 68:369, 1952.
57. Slate RK, Posnick JC, Armstrong DC,
Buncic JR: Cervical spine subluxation
associated with congenital muscular
torticollis and craniofacial asymmetry. Plast
Reconstr Surg 91(7):1187–1195, 1993.
58. Sommerfeld RM, Schweiger JW: Cleft palate
with Klippel–Feil syndrome. Oral Surg
27:737, 1969.
59. Stadnicki G, Rassumowski D: The
association of cleft palate with the
Klippel–Feil syndrome. Oral Surg 33:335,
1972.
60. Stewart EJ, O’Reilly BF: Klippel–Feil
syndrome and conductive deafness.
J Laryngol Otol 103:947, 1989.
61. Tveter KJ, Kluge T: Cor triloculare biatrium
associated with Klippel–Feil syndrome. Acta
Paediatr Scand 54:489, 1965.
62. Weber V, Schneider HJ, Haberlandt W:
Pathogenetische und genetische fragen beim
Klippel–Feil syndrom–vergleichende
betrachtungen bei zwei vettern. Fortschr Geb/
Roentgenstr/Nuklearmed 119:209, 1973.
63. Willemsen WN: Combination of the
Mayer–Rokitansky–Kuster and Klippel–Feil
syndrome—A case report and literature
review. Eur J Obstet Gynecol Reprod Biol
13:229, 1982.
64. Windle-Taylor PC, Emery PJ, Phelps PD:
Ear deformities associated with the
Klippel–Feil syndrome. Ann Otol Rhinol
Laryngol 90:210, 1981.
Down Syndrome
65. Adams MM, Erickson JD, Layde PM, et al:
Down syndrome: Recent trends in the
United States. JAMA 246:758, 1981.
66. Ardran GM, Harker P, Kemp FH: Tongue
size in Down’s syndrome. J Ment Defic Res
16:160, 1972.

1293
CHAPTER 31  Selected Complex Malformations that Frequently Require Maxillofacial Reconstruction: Evaluation & Treatment
67. Balkany TJ, Downs MP, Jafek BW, et al:
Hearing loss in Down syndrome. Clin
Pediatr 18:116, 1979.
68. Bjuggren G, Jensen R, Strombeck JO:
Macroglossia and its surgical treatment:
Indications and postoperative experiences
from the orthodontic, phoniatric, and
surgical points of view. Scand J Plast Reconstr
Surg 2:116, 1968.
69. Brousseau K: Mongolism, Baltimore, Md,
1928, Williams & Wilkins.
70. Dios PD, Feijoo JF, Ferreiro MC, et al:
Functional consequences of partial
glossectomy. J Oral Maxillofac Surg 52:12,
1994.
71. Donaldson JD, Redmond WM: Surgical
management of obstructive sleep apnea in
children with Down syndrome. J Otolaryngol
17:398, 1988.
72. Down JL: Observation on an ethnic
classification of idiots. Ment Retard
33(1):54–56, 1866.
73. Farkas LG, Munro IR, Kolar JC: Abnormal
measurements and disproportions in the face
of Down syndrome patients: Preliminary
report of an anthropometric study. Plast
Reconstr Surg 75:159, 1985.
74. Gisel EG, Lange LJ, Niman CW: Tongue
movements in 4 and 5 year old Down
syndrome children during eating: A
comparison with normal children. Am J
Occup Ther 38:660, 1984.
75. Gorlin RJ, Cohen MM, Jr, Levin LS:
Syndromes of the head and neck, ed 3, New
York, 1990, Oxford University Press.
76. Hinderer U: Malar implants for
improvement of the facial appearance. Plast
Reconstr Surg 56:157, 1975.
77. Hohler H: Changes in facial expression as a
result of plastic surgery in mongoloid
children. Aesthetic Plast Surg 1:245, 1977.
78. Hultcrantz E, Svanholm H: Down syndrome
and sleep apnea—a therapeutic challenge.
Int J Pediatr Otorhinolaryngol 21:263,
1991.
79. Katz S, Kravetz S: Facial plastic surgery for
persons with Down syndrome: research
finding and their professional such
implications. Am J Ment Retard 94(2):101–
110, 1989.
80. Lefavre J-F, Cohen SR, Burstein FD, et al:
Down syndrome: Identification and surgical
management of obstructive sleep apnea. Plast
Reconstr Surg 99:629, 1997.
81. Lejeune J, Turpin R, Gautier M: Le
mongolisme, maladie chromosomique
(trisomie). Bull Acad Natl Med 143:256,
1959.
82. Lemperle G: Plastic surgery in children with
Down syndrome. In Lane D, Stratford B,
editors: Current approaches to Down
syndrome, Eastbourne, UK, 1985, Holt,
Rinehart & Winston, p 131.
83. Lemperle G, Nievergelt J: Plastisch–
chirurgische korrekturen im geicht von
kindern mit Down–syndrom. In Plastische
Chirurgie bei Menschen mit Down
Syndrom. Lebenshilfe Marburg 9:19, 1983.
84. Lemperle G, Radu D: Facial plastic surgery
in children with Down syndrome. Plast
Reconstr Surg 66:337, 1980.
85. Lemperle G, Spitalny HH: Long-term
experience with silicone implants in the face.
In Caronni EP, editor: Craniofacial surgery,
Boston, 1985, Little, Brown, p 490.
86. Marcus CL, Keens TG, Bautista DB, et al:
Obstructive sleep apnea in children with
Down syndrome. Pediatrics 88:132, 1991.
87. Margar-Bacal F, Witzel MA, Munro IR:
Speech intelligibility after partial glossectomy
in children with Down syndrome. Plast
Reconstr Surg 79:44, 1987.
88. Michejda M, Menolascino FJ: Skull base
abnormalities in Down syndrome. Ment
Retard 13:24,1975.
89. Martinez-Frias ML: The real earliest
historical evidence of Down syndrome. Am J
Med Genet A 132A(2):231, 2005.
90. Mearig JS: Facial surgery and an active
modification approach for children with
Down syndrome: Some psychological and
ethical issues. Rehabil Lit 46:72, 1985.
91. Olbrisch RR: Plastic surgical management of
children with Down syndrome: Indications
and results. Br J Plast Surg 35:195, 1982.
92. Olbrisch RR: Plastic surgery in 250 children
with Down syndrome: Indications and results.
Transactions of the Eighth International
Congress on Plastic and Reconstructive Surgery,
Montreal, 1983, International Plastic Surgery
Society, p 702.
93. Otermin A: Mongolism (Down syndrome)
and plastic surgery. Prensa Meica Argentina
60:27, 970–972, 1967.
94. Patterson RS, Munro IR, Farkas LG:
Transconjunctival lateral canthopexy in
Down syndrome patients: A nonstigmatizing
approach. Plast Reconstr Surg 79:714, 1987.
95. Posnick JC: Other craniofacial syndromes:
Klippel–Feil anomaly, neurofibromatosis,
Down syndrome, micro-ophthalmia,
hemifacial hyperplasia (hemi-hypertrophy).
In Posnick JC, editor: Craniofacial and
maxillofacial surgery in children and young
adults, Philadelphia, 2000, WB Saunders Co,
24, pp 503–527.
96. Pueschel S, Rynders J, editors: Down
syndrome: Advances in biomedicine and the
behavioral sciences, Cambridge, Mass, 1982,
Ware Press.
97. Rand Y, Mintsker Y, Feuerstein R:
Reconstructive plastic surgery in Down
syndrome children and adults: Parents’
evaluations—follow-up data. Jerusalem,
Hadassah-Wizo-Canada Research Institute,
1984.
98. Rolfe CR, Montague JC, Jr, Tirman RM,
et al: Pilot perceptual and physiological
investigation of hypernasality in Down
syndrome adults. Folia Phoniatr 31:177,
1979.
99. Rozner L: Facial plastic surgery for Down
syndrome. Lancet 1:132, 1983.
100. Silverman M: Airway obstruction and sleep
disruption in Down syndrome. Br Med J
296:1618, 1988.
101. Strome M: Down syndrome: A modern
otorhinolaryngological perspective.
Laryngoscope 91:1581, 1981.
102. Strome M: Obstructive sleep apnea in Down
syndrome children: A surgical approach.
Laryngoscope 96:1340, 1986.
103. Wexler MR, Peled IJ: Plastic surgery in
Down syndrome. Down Syndrome 6:7, 1983.
104. Wexler MR, Peled IJ, Rand Y, et al:
Rehabilitation of the face in patients with
Down syndrome. Plast Reconstr Surg 77:383,
1986.
Neurofibromatosis
105. Abuelo DN, Meryash DL:
Neurofibromatosis with fully expressed
Noonan syndrome. Am J Med Genet 29:937,
1988.
106. Afifi AK, Dolan KD, Van Gilder JC, et al:
Ventriculomegaly in neurofibromatosis.
Neurofibromatosis 1:299–305, 1988.
107. Baden E, Pierce HE, Jackson WF: Multiple
neurofibromatosis with oral lesions: Review
of the literature and report of a case. Oral
Surg Oral Med Oral Pathol 8:268, 1955.
108. Baden E, Jones JR, Khedekar R, et al:
Neurofibromatosis of the tongue: A light and
electronmicroscopic study with review of the
literature from 1849 to 1981. J Oral Med
39:157, 1984.
109. Bader JL: Neurofibromatosis and cancer. Ann
NY Acad Sci 486:57, 1986.
110. Barker D, Wright E, Nguyen K, et al: Gene
for von Recklinghausen neurofibromatosis is
in the pericentric region of chromosome 17.
Science 236:1100, 1987.
111. Benedict PH, Szabo G, Fitzpatrick TB, et al:
Melanotic macules in Albright syndrome and
in neurofibromatosis. JAMA 205:618, 1968.
112. Binet FF, Kieffer SA, Martin SH, et al:
Orbital dysplasia in neurofibromatosis.
Radiology 93:829, 1969.
113. Bloem JJ, van der Meulen JC:
Neurofibromatosis in plastic surgery. Br J
Plast Surg 31:50, 1978.
114. Brooks B, Lehman EP: The bone changes in
Recklinghausen’s neurofibromatosis. Surg
Gynecol Obstet 38:587, 1924.
115. Buntin PT, Fitzgerald JF: Gastrointestinal
neurofibromatosis. Am J Dis Child 119:521,
1970.
116. Carey JC, Laub JM, Hall BD: Penetrance
and variability in neurofibromatosis: A
genetic study of 60 families. Birth Defects
Orig Artic Ser 15:271, 1979.
117. Chao DH-C: Congenital neurocutaneous
syndromes in childhood: I.
Neurofibromatosis. J Pediatr 55:189, 1959.
118. Clark RD, Hutter JJ, Jr: Familial
neurofibromatosis and juvenile chronic
myelogenous leukemia. Hum Genet 60:230,
1982.
119. Cohen MM, Jr, Hayden PW: A newly
recognized hamartomatous syndrome. Birth
Defects Orig Artic Ser 15:291, 1979.
120. Crawford AH: Neurofibromatosis in
children. Acta Orthop Scand Suppl 218:1,
1986.

1294 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
121. Crowe FW, Schull WJ: Diagnostic
importance of cafe-au-lait spot in
neurofibromatosis. Arch Intern Med 91:758,
1953.
122. D’Ambrosio JA, Langlais RP, Young RS: Jaw
and skull changes in neurofibromatosis. Oral
Surg 66:391, 1988.
123. Davis WB, Edgerton MT, Hoffmeister SF:
Neurofibromatosis of the head and neck.
Plast Reconstr Surg 14:186, 1954.
124. Diehl SR, Boehnke M, Erickson RP, et al:
A refined genetic map of the region of
chromosome 17 surrounding the von
Recklinghausen neurofibromatosis (NFI)
gene. Am J Hum Genet 44:33, 1989.
125. Dreyfuss U, Ben-Arieh JY, Hirshowitz B:
Liposarcoma: A rare complication in
neurofibromatosis: Case report. Plast Reconstr
Surg 61:287, 1978.
126. Dunn DW: Neurofibromatosis in childhood.
Curr Probl Pediatr 17:445, 1987.
127. Farag MZ: Vascular malformation of the
parotid gland in von Recklinghausen’s
disease. J Laryngol Otol 97:571, 1983.
128. Fienman NL, Yakovac W: Neurofibromatosis
in childhood. J Pediatr 76:339, 1970.
129. Freeman AG: Proptosis and
neurofibromatosis. Lancet 1:1032,
1987.
130. Gorlin RJ, Cohen MM, Jr, Levin LS:
Syndromes of the head and neck, ed 3, New
York, 1990, Oxford University Press.
131. Grabb WC, Reed O, Dingman RMO, et al:
Facial hamartomas in children:
Neurofibroma, lymphangioma, and
hemangioma. Plast Reconstr Surg 66:509,
1980.
132. Grant WM, Walton DS: Distinctive
gonioscopic findings in glaucoma due to
neurofibromatosis. Arch Ophthalmol 79:127,
1968.
133. Griffith BH, Lewis YL, Jr, McKinney P:
Neurofibromas of the head and neck. Surg
Gynecol Obstet 160:534, 1985.
134. Hall BD: Congenital lid ptosis associated
with neurofibromatosis [letter to the editor].
Am J Med Genet 25:595, 1986.
135. Holt JF: Neurofibromatosis in children. Am
J Roentgenol 130:615, 1978.
136. Holt JF, Wright EM: The radiologic features
of neurofibromatosis. Radiology 51:647,
1948.
137. Horwich A, Riccardi VM, Francke U: Brief
clinical report: Aqueductal stenosis leading
to hydrocephalus—an unusual manifestation
of neurofibromatosis. J Med Genet 14:577,
1983.
138. Hunt JC, Pugh DG: Skeletal lesions in
neurofibromatosis. Radiology 76:1,
1961.
139. Jackson IT, Carbonnel A, Potparic Z, et al:
Orbitotemporal neurofibromatosis:
Classification and treatment. Plast Reconstr
Surg 92:1, 1993.
140. Jackson IT, Laws ER, Martin RD: The
surgical management of orbital
neurofibromatosis. Plast Reconstr Surg
71:751, 1983.
141. Jacobs MH: Oral manifestations in von
Recklinghausen’s disease (neurofibromatosis).
Am J Orthod Oral Surg 32:28, 1946.
142. Janecka IP: Correction of ocular dystopia.
Plast Reconstr Surg 97:892, 1996.
143. Kaplan P, Rosenblatt B: A distinctive facial
appearance in neurofibromatosis von
Recklinghausen. Am J Med Genet 21:463,
1985.
144. Koblin I, Reil B: Changes in the facial
skeleton in cases of neurofibromatosis.
J Maxillofac Surg 3:23, 1975.
145. Kragh LV, Soule EH, Masson JK:
Neurofibromatosis (von Recklinghausen’s
disease) of the head and neck: cosmetic and
reconstructive aspects. Plast Reconstr Surg
Transplant Bull 25:565, 1960.
146. Laue L, Comite F, Hench K, et al:
Precocious puberty associated with
neurofibromatosis and optic gliomas. Am J
Dis Child 139:1097, 1985.
147. Lewis RA, Gerson LP, Axelson KA, et al:
Von Recklinghausen neurofibromatosis: II.
Incidence of optic gliomata. Ophthalmology
91:929, 1984.
148. Maceri DS, Saxon KG: Neurofibromatosis of
the head and neck. Head Neck Surg 6:842,
1984.
149. Marchac D: Intracranial enlargement of the
orbital cavity and palpebral remodelling for
orbitopalpebral neurofibromatosis. Plast
Reconstr Surg 73:534, 1984.
150. Martuza RL, Eldridge R: Neurofibromatosis
2 (bilateral acoustic neurofibromatosis). N
Engl J Med 318:684, 1988.
151. Miller M, Hall JG: Possible maternal effect
in severity of neurofibromatosis. Lancet
2(8099):1071–1073, 1978.
152. Munro IR, Martin RD: The management of
gigantic benign craniofacial tumors: The
reverse facial osteotomy. Plast Reconstr Surg
65:777, 1980.
153. Opitz JM, Weaver DD: The
neurofibromatosis-Noonan syndrome. Am J
Med Genet 21:477, 1985.
154. Parker DA, Skalko RG: Congenital
asymmetry: Report of 10 cases with
associated developmental abnormalities.
Pediatrics 44:584, 1969.
155. Pollack MA, Shprintzen RJ: Velopharyngeal
insufficiency in neurofibromatosis. Int J
Pediatr Otorhinolaryngol 3:257, 1981.
156. Posnick JC: Other craniofacial syndromes:
Klippel–Feil anomaly, neurofibromatosis,
Down syndrome, micro-ophthalmia,
hemifacial hyperplasia (hemi-hypertrophy).
In Posnick JC, editor: Craniofacial and
maxillofacial surgery in children and young
adults, Philadelphia, 2000, WB Saunders Co,
24, pp 503–527.
157. Riccardi VM, Kleiner B, Lubs ML:
Neurofibromatosis: Variable expression is not
intrinsic to the mutant gene. Birth Defects
Orig Artic Ser 15:283, 1979.
158. Rittersma J, ten Kate LP, Westerink P:
Neurofibromatosis with mandibular
deformities. Oral Surg Oral Med Oral Pathol
33:718, 1972.
159. Shapiro SD, Abramovitch K, Van Dis ML,
et al: Neurofibromatosis: Oral and
radiographic manifestations. Oral Surg
58:493, 1984.
160. Sorensen SA, Mulvihill JJ, Nielsen A: On the
natural history of von Recklinghausen
neurofibromatosis. Ann N Y Acad Sci 486:30,
1986.
161. Spencer WG, Shattock SG: A case of
macroglossia neurofibromatosa. Proc R Soc
Med 1:8, 1908.
162. Tibbles JA, Cohen MM, Jr: The Proteus
syndrome: The Elephant Man diagnosed. Br
Med J (Clin Res Ed) 293:683, 1986.
163. Van Damme PA, Freihofer HPM, De Wilde
CM: Neurofibroma in the articular disc
of the temporomandibular joint: A case
report. J Craniomaxillofac Surg 24:310,
1996.
164. Van der Meulen JC: Orbital
neurofibromatosis. Clin Plast Surg 14:123,
1987.
165. Von Recklinghausen FD: Uber die multiplen
fibrome der haut und ihre Bezichung zu den
multiplen neuromen: festschrift fur Rudolph
Virchow, Berlin, 1882, Hirschwald.
166. Weber FP: Neurofibromatosis of the tongue
in a child, together with a note on the
classification of incomplete and anomalous
cases of Recklinghausen disease. Br J Child
Dis 7:13, 1910.
167. Westerhof W, Delleman JW, Wolters E, et al:
Neurofibromatosis and hypertelorism. Arch
Dermatol 120:1579, 1984.
Hemihypertrophy of the Face
168. Andermann E, et al: A syndrome of
hemihypertrophy, hemihypesthesia,
hemiareflexia and scoliosis in three unrelated
girls. Fifth International Conference on Birth
Defects, Montreal, Quebec, Canada, August
21–27, 1977.
169. Arnold EB: Case of hemiacromegaly. Int J
Orthodont 22:1228, 1933.
170. Bell RA, McTigue DJ: Complex congenital
hemihypertrophy: A case report and
literature review. J Pedod 8:300, 1984.
171. Bergman JA: Primary hemifacial
hypertrophy: Review and report of a case.
Arch Otolaryngol 97:490, 1973.
172. Boxer LA, Smith DL: Wilm’s tumor prior to
onset of hemihypertrophy. Am J Dis Child
120:564, 1970.
173. Cohen MM, Jr: Overgrowth syndromes. In
El-Shafie M, Klippel CH, editors: Associated
congenital malformations, Baltimore, Md,
1981, Williams & Wilkins, pp 71–104.
174. Cohen MM, Jr: A comprehensive and
critical assessment of overgrowth and
overgrowth syndromes. Adv Hum Genet
18:181, 1989.
175. Eisenberg RL, Pfister RC: Medullary sponge
kidney associated with congenital
hemihypertrophy (asymmetry). Am J
Roentgenol 116:773, 1972.
176. Fraumeni JF, Jr, Geiser CF, Manning MD:
Wilms’ tumor and congenital
hemihypertrophy: Report of five new cases

1295
CHAPTER 31  Selected Complex Malformations that Frequently Require Maxillofacial Reconstruction: Evaluation & Treatment
and review of literature. Pediatrics 40:886,
1967.
177. Fraumeni JF, Miller RW: Adrenocortical
neoplasms with hemihypertrophy, brain
tumors, and other disorders. J Pediatr
70:129, 1967.
178. Furnas DW, Soper RT, Nickman NJ, et al:
Congenital hemihypertrophy of the face:
Impersonator of childhood facial tumors.
J Pediatr Surg 5:344, 1970.
179. Gesell A: Hemihypertrophy and twinning:
Further study of the nature of
hemihypertrophy with report of a new case.
Am J Med Sci 173:542, 1927.
180. Gonzalez-Crussi F, Lee SC, McKinney M:
The pathology of congenital localized
gigantism. Plast Reconstr Surg 59:411,
1977.
181. Gorlin RJ, Cohen MM, Jr, Levin LS:
Syndromes of the head and neck, ed 3, New
York, 1990, Oxford University Press.
182. Gorlin RJ, Meskin LH: Congenital
hemihypertrophy. J Pediatr 61:870, 1962.
183. Haicken BN: Congenital hemihypertrophy.
Am J Dis Child 120:373, 1970.
184. Harkin JC, Reed RJ: Tumors of the
peripheral nervous system. In Atlas of Tumor
Pathology, series 2, fascicle 3, Washington,
DC, 1969, Armed Forces Institute of
Pathology.
185. Hennessy WT, Cromie WJ, Duckett JW:
Congenital hemihypertrophy and associated
abdominal lesions. Urology 18:576, 1981.
186. Hoyme HE, et al: The incidence of neoplasia
in children with isolated congenital
hemihypertrophy. David Smith Meeting on
Malformations and Morphogenesis,
Burlington, Vt, August 11–14, 1986.
187. Khanna JN, Andrade NN: Hemifacial
hypertrophy: Report of two cases. Int J Oral
Maxillofac Surg 18:294, 1989.
188. Kogon SL, Jarvis AM, Daley TD, et al:
Hemifacial hypertrophy affecting the
maxillary dentition. Oral Surg Oral Med Oral
Pathol 58:549, 1984.
189. Lenstrup E: Eight cases of hemihypertrophy.
Acta Pediatr Scand 6:205, 1926.
190. Loh HS: Congenital hemifacial hypertrophy.
Br Dent J 153:111, 1982.
191. Meadows AT: Wilms’ tumor in three
children of a woman with hemihypertrophy.
N Engl J Med 291:23, 1974.
192. Meckel JF: Uber die seitliche asymmetrie im
tierichen korper, anatomische physiologische
beobachtungen und untersuchungen, Renger,
1822, Haile, p 147.
193. Obwegeser HL: Descriptive terminology for
jaw anomalies. Oral Surg Oral Med Oral
Pathol 75:138–140, 1993.
194. Obwegeser HL: Mandibular growth
anomalies: Terminology-aetiology-diagnosis-
treatment, Berlin, Heidelberg, NY, 2001,
Springer-Verlag.
195. Obwegeser HL, Mekek M: Hemimandibular
hyperplasia—hemimandibular elongation.
J Maxillofac Surg 14:183–208, 1986.
196. Pollock RA, Newman MH, Burdi AR, et al:
Congenital hemifacial hyperplasia: An
embryologic hypothesis and case report. Cleft
Palate J 22:173, 1985.
197. Posnick JC: Other craniofacial syndromes:
Klippel–Feil anomaly, neurofibromatosis,
Down syndrome, micro-ophthalmia,
hemifacial hyperplasia (hemi-hypertrophy).
In Posnick JC, editor: Craniofacial and
maxillofacial surgery in children and young
adults, Philadelphia, 2000, WB Saunders Co,
24, pp 503–527.
198. Reed EA: Congenital total hemihypertrophy.
Arch Neurol Psychiatry 14:824, 1925.
199. Ringrose RE, Jabbour JT, Keele DK:
Hemihypertrophy. Pediatrics 36:434, 1965.
200. Rudolph DC, Norvold RW: Congenital
partial hemihypertrophy involving marked
malocclusions. J Dent Res 23:133, 1944.
201. Sculerati N, Jacobs JB: Congenital facial
hemihypertrophy: Report of a case with
airway compromise. Head Neck Surg 8:124,
1985.
202. Spielman WR, Marano PD, Kolodny SC,
et al: True hemifacial hypertrophy: Report of
case. J Oral Surg 29:592, 1971.
203. Tommasi AF, Jitomirski F: Crossed
congenital hemifacial hemihyperplasia. Oral
Surg Oral Med Oral Pathol 67:190, 1989.
204. Viljoen D, Pearn J, Beighton P:
Manifestations and natural history of
idiopathic hemihypertrophy: A review of
eleven cases. Clin Genet 26:81, 1984.
205. Viteporn S: Surgical-orthodontic treatment
in hemifacial hypertrophy: A case report. Int
J Adult Orthodon Orthognath Surg 8:55,
1993.
206. Wagner R, see Kottmeier HL: Uber
hemihypertrophia und heniatrophia corporis
totalis nebst spontane extremitatenganrane
bei saugliegen im anschluss zu einem
ungewohnlichen fall. Acta Paediatr Scand
20:543, 1938.
207. Wakefield EG, Hines EA, Jr: Congenital
hemihypertrophy: A report of eight cases.
Am J Med Sci 195:493, 1933.
208. Ward J, Lerner HH: A review of the subject
of congenital hemihypertrophy and a
complete case report. J Pediatr 31:403,
1947.
Vascular Malformations
209. Arneja JS, Gosain AK: Vascular
malformations. Plast Reconstr Surg
121:195e–206e, 2008.
210. Berenguer B, Burrows PE, Zurakowski D,
et al: Sclerotherapy of craniofacial venous
malformations: Complications and results.
Plast Reconstr Surg 104:1, 1999.
211. Burns AJ, Kaplan LC, Mulliken JB: Is there
association between hemangioma and
syndromes with dysmorphic features?
Pediatrics 88:1257–1267, 1991.
212. Burrows PE, Mulliken JB, Fellows KE, et al:
Childhood hemangiomas and vascular
malformations: Angiographic differentiation.
AJR Am J Roentgenol 141:483, 1983.
213. Cohen MM, Jr: Vasculogenesis, angiogenesis,
hemangiomas, and vascular malformations.
Am J Med Genet 108:265–274, 2002.
214. Cohen MM, Jr: Vascular update:
Morphogenesis, tumors, malformations, and
molecular dimensions. Am J Med Genet
140A:2013–2038, 2006.
215. Cohen MM, Jr, Neri G, Weksberg R:
Overgrowth syndromes, New York, 2002,
Oxford University Press.
216. Enjolras O, Mulliken JB: The current
management of vascular birthmarks. Pediatr
Dermatol 10:311, 1993.
217. Enjolras O, Mulliken JB: Vascular tumors
and vascular malformations. Adv Dermatol
13:375–423, 1998.
218. Folkman J, D’Amore PA: Blood vessel
formation: What is its molecular basis? Cell
87:1153, 1996.
219. Hein KD, Mulliken JB, Kozakewich HPW,
et al: Venous malformations of skeletal
muscle. Plast Reconstr Surg 110:1625, 2002.
220. Jackson IT, Carreno R, Potparic Z, et al:
Hemangiomas, vascular malformations, and
lymphovenous malformations: Classification
and methods of treatment. Plast Reconstr
Surg 91:1216, 1993.
221. Jackson IT, Keskin M, Yavuzer R, et al:
Compartmentalization of massive vascular
malformations. Plast Reconstr Surg 115:10,
2005.
222. Marler JJ, Mulliken JB: Current
management of hemangiomas and vascular
malformations. Clin Plast Surg 32:99,
2005.
223. Mulliken JB: Cutaneous vascular anomalies.
Semin Vasc Surg 6:204–218, 1993.
224. Mulliken JB: Vascular anomalies. In Aston
SJ, Beasley RW, Thorne CHM, editors:
Grabb and Smith’s plastic surgery, ed 5,
Philadelphia, 1997, Lippincott-Raven,
p 191.
225. Mulliken JB, Fishman SJ, Burrows PE:
Vascular anomalies. Curr Probl Surg 37:517,
2000.
226. Mulliken JB, Glowacki J: Hemangiomas and
vascular malformations in infants and
children: A classification based on
endothelial characteristics. Plast Reconstr Surg
69:412–422, 1982.
227. Mulliken JB, Murray JE, Castaneda AR,
et al: Management of a vascular
malformation of the face using total
circulatory arrest. Surg Gynecol Obstet
146:168, 1978.
228. Mulliken JB, Young AE: Vascular birthmarks:
Hemangiomas and malformations,
Philadelphia, 1998, Saunders.
229. Posnick JC: Hemangiomas and vascular
malformations of the head and neck:
Evaluation and management. In Posnick JC,
editor: Craniofacial and maxillofacial surgery
in children and young adults, Philadelphia,
2000, WB Saunders Co, 26, pp 564–596.
230. Pribaz JJ, Morris DJ, Mulliken JB:
Three-dimensional folded free-flap
reconstruction of complex facial defects
using intraoperative modelling. Plast Reconstr
Surg 93:285, 1994.
231. Takahashi K, Mulliken JB, Kozakewich
HPW, et al: Cellular markers that distinguish

1296 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
the phases of hemangioma during infancy
and childhood. J Clin Invest 93:2357, 1994.
232. Vikkula M, Boon LM, Mulliken JB:
Molecular genetics of vascular
malformations. Matrix Biol 20:327, 2001.
Velocardiofacial Syndrome
233. Antshel KM, Fremont W, Kates WR: The
neurocognitive phenotype in velo-cardio-
facial syndrome: a developmental perspective.
Dev Disabil Res Rev 14:43–51, 2008.
234. Carotti A, Digilio MC, Piacentini G, et al:
Cardiac defects and results of cardiac surgery
in 22q11.2 deletion syndrome. Dev Disabil
Res Rev 14:35–42, 2008.
235. Cuneo BF: 22q11.2 deletion syndrome:
DiGeorge, velocardiofacial, and conotruncal
anomaly face syndromes. Curr Opin Pediatr
13:465–472, 2001.
236. De Smedt B, Swillen A, Ghesquiere P, et al:
Pre-academic and early academic
achievement in children with velocardiofacial
syndrome (del22q11.2) of borderline or
normal intelligence. Genet Couns 14:15–29,
2003.
237. Digilio MC, Angioni A, De Santis M, et al:
Spectrum of clinical variability in familial
deletion 22q11.2: from full manifestation to
extremely mild clinical anomalies. Clin Genet
63:308–313, 2003.
238. Dyce O, McDonald-McGinn D, Kirschner
RE, et al: Otolaryngologic manifestations of
the 22q11.2 deletion syndrome. Arch
Otolaryngol Head Neck Surg 128:1408–1412,
2002.
239. Eberle P, Berger C, Junge S, et al: Persistent
low thymic activity and non-cardiac
mortality in children with chromosome
22q11.2 microdeletion and partial DiGeorge
syndrome. Clin Exp Immunol 155:189–198,
2009.
240. Goldberg R, Motzkin B, Marion R, et al:
Velo-cardio-facial syndrome: a review of 120
patients. Am J Med Genet 45:313–319,
1993.
241. Goldmuntz E: DiGeorge syndrome: new
insights. Clin Perinatol 32:963–978, 2005.
242. Goldmuntz E, Clark BJ, Mitchell LE, et al:
Frequency of 22q11 deletions in patients
with conotruncal defects. J Am Coll Cardiol
32:492–498, 1998.
243. Kelly D, Goldberg R, Wilson D, et al:
Confirmation that the velo-cardio-facial
syndrome is associated with
haplo-insufficiency of genes at chromosome
22q11. Am J Med Genet 45:308–312, 1993.
244. McDonald-McGinn DM, Zackai EH:
Genetic counseling for the 22q11.2 deletion.
Dev Disabil Res Rev 14:69–74, 2008.
245. McLean-Tooke A, Spickett GP, Gennery AR:
Immunodeficiency and autoimmunity in
22q11.2 deletion syndrome. Scand J
Immunol 66:1–7, 2007.
246. Mehendale FV, Sommerlad BC: Surgical
significance of abnormal internal carotid
arteries in velocardiofacial syndrome in 43
consecutive hynes pharyngoplasties. Cleft
Palate Craniofac J 41:368–674, 2004.
247. Ryan AK, Goodship JA, Wilson DL, et al:
Spectrum of clinical features associated with
interstitial chromosome 22q11 deletions: a
European collaborative study. J Med Genet
34:798–804, 1997.
248. Shprintzen RJ: Velo-cardio-facial syndrome:
30 years of study. Dev Disabil Res Rev
14:3–10, 2008.
249. Shprintzen RJ, Goldberg RB, Lewin ML,
et al: A new syndrome involving cleft palate,
cardiac anomalies, typical facies, and learning
disabilities: velo-cardio-facial syndrome. Cleft
Palate J 15:56–62, 1978.

1297

SECTION 5  Cleft Jaw Deformities
32
Cleft–Orthognathic Surgery:
The Unilateral Cleft Lip and
Palate Deformity
JEFFREY C. POSNICK, DMD, MD
• Facial Growth Implications of Cleft Palate
Repair in the Infant with Unilateral Cleft Lip
and Palate
• Coordinated Team Approach
• Treatment Protocol
• Timing of Orthognathic Surgery
• Residual Deformities in the Adolescent with
Unilateral Cleft Lip and Palate
• Orthodontic Considerations in the Patient
with Unilateral Cleft Lip and Palate with
a Jaw Deformity
• Immediate Presurgical Re-Assessment
• Orthognathic Approach for Unilateral Cleft
Lip and Palate Deformities
• Avoiding Pitfalls
• Clinical Management after Initial Surgical
Healing
• Orthognathic Surgery for Unilateral Cleft Lip
and Palate: Review of Study
• Skeletal Stability after Modified Le Fort I
for Unilateral Cleft Lip and Palate: Review
of Study
• Controversies and Unresolved Issues
• Conclusions
In 1880, the pioneer orthodontist Normal W. Kingsley
stated the following in his remarks about surgical operations
as a means of correcting cleft palate: “Although the practice
[of cleft palate repair] has been tested in thousands of cases
by the most eminent surgeons of their time, it has resulted
in such uniformity of failure [i.e., severe jaw deformities],
that it should have been utterly abandoned years ago.”85
In 1920, another preeminent orthodontist of his day,
Calvin Case, voiced a similar sentiment: “[L]et us hope that
the proportion of [cleft palate] surgical failures will be
greatly lessened in the future and that well-informed, honest
surgeons … accept only the most favorable cases with a
determination to follow them through with proper
interest.”16
In 1965, Vilray Kazanjian, who was a leading maxillofa­
cial surgeon of the first half of the twentieth century,
reminded us that “the relative value of surgery to repair a
cleft palate has been intelligently questioned for over 150
years.”83
Facial Growth Implications of Cleft
Palate Repair in the Infant with
Unilateral Cleft Lip and Palate
Effects of Cleft Palate Repair in Infancy
The management of individuals with unilateral cleft lip and
palate (UCLP) presents specific clinical challenges for the
maxillofacial surgeon, the orthodontist, and the restorative
dental team. Orthognathic surgery is a procedure that
should be considered as part of the treatment algorithm for
patients with UCLP. Ross completed a multicenter, long-
term facial growth study to assess the need for orthognathic
surgery among individuals born with complete UCLP
who had undergone primary lip and palate repair during
The successful reconstruction of cleft lip and palate is inex­
tricably connected with orthodontic care and skeletal
surgery. The negative effects of the primary surgical repair
of cleft palate during childhood have been known for the
past 200 years.

1298 SECTION 5  Cleft Jaw Deformities
childhood. He concluded that, even by the most conserva­
tive standards and in conjunction with maximum compen­
sating orthodontic camouflage maneuvers, at least 25% of
adolescents with UCLP required orthognathic surgery to
achieve even the limited objective of a neutralized occlu­
sion.148 His research indicated that only 25% of adolescents
with UCLP had near-normal maxillary growth and that
another 50% were in a borderline category with some
degree of maxillary hypoplasia. Ross stated that individuals
who were born with a cleft lip and palate have an intrinsic
deficiency in the midfacial skeleton that is made worse by
operations. More recently, Mulliken and colleagues reviewed
the prevalence of Le Fort I osteotomies among patients with
cleft lip and palate who were treated at Boston Children’s
Hospital.55 They found that 48% of UCLP patients who
underwent repair in infancy later required orthognathic
surgery. The study also showed that the need for orthogna­
thic surgery is dependent on the severity of the cleft type as
well as the number and extent of previous operative proce­
dures. Similarly, The Hospital for Sick Children in Toronto,
Ontario, Canada, found that 48.3% of their patients (i.e.
treated since infancy) with complete UCLP required
orthognathic surgery. When they looked at all patients with
UCLP who were referred to their center, they found that
59.4% needed jaw surgery.27 A retrospective cohort study
of five prominent cleft palate centers in North America
compared the maxillomandibular relationships of individu­
als with non-syndromal complete UCLP (n = 169).63 The
one center that incorporated primary alveolar bone grafting
showed especially poor maxillary growth, with 66% of its
patients requiring orthognathic surgery. Interestingly, at the
one center in which a single surgeon performed all of the
surgeries with the use of a more delayed approach to cleft
palate repair and whose patients underwent no revisions
until they were 14 years old showed the lowest need for
orthognathic surgery at less than 25%.
Saperstein and colleagues described the facial growth of
children with complete clefting of the primary palate (i.e.,
the lip through the incisal foramen) but with an intact
secondary palate (i.e., the incisal foramen through the
uvula).151 This was a retrospective, cross-sectional analysis
of non-syndromal patients with unilateral complete clefting
of the primary palate as compared with those with unilateral
complete clefting of both the primary and secondary palates.
Angular and linear measurements of the midfacial region
were made on lateral cephalograms. The study groups
included those with unilateral complete clefting of the
primary palate (n = 25) and those with unilateral complete
clefting of the primary and secondary palate (n = 18). The
study documented that individuals with a cleft of only the
primary palate who underwent lip repair during infancy
typically had a normal or even slightly forward maxillary
position as compared with age-matched controls. This was
in contrast with children with a cleft of both the primary
and secondary palates who underwent lip repair followed
by palate repair before they were 1 year old; this group
showed a high incidence of maxillary deficiency. The study
clarified that the cleft palate repair carried out before 1 year
of age—and not the cleft lip repair itself—was responsible
for the high incidence of midface hypoplasia.
Effects of Achieving Bone Fusion Across the
Cleft Alveolus during Infancy
Reconstruction of the alveolar process in patients with
UCLP is an essential part of cleft care. Accomplishing this
goal provides support for the alar base of the nose; the teeth
in the cleft region; and the periodontium that surrounds
those teeth (Figs. 32-1 and 32-2). There are three basic
methods that have been described for the closure of the cleft
alveolus: 1) primary bone grafting; 2) secondary bone graft­
ing; and 3) gingival periosteoplasty (GPP).56 The method
of primary bone grafting is now generally recognized to
result in severe midface growth disturbance and has there­
fore been universally abandoned throughout the world.63,148
The use of secondary (mixed dentition) bone grafting is
generally recognized as an effective method to avoid the
problem of additional midface growth disturbance and to
successfully achieve support for the alar base; to provide
bone for the eruption of the canine through the grafted
cleft site; and to establish effective periodontal support
(Figs. 32-3 through 32-6).1
The technique of GPP was first described by Skoog in
1965 as a method to achieve fusion across the cleft alveolus
at the time of lip repair. The goal was to “remove” the cleft
during infancy with the hope that no harm would result.156
Millard included the use of the Latham device to position
the alveolar ridges close to one another before going forward
with the GPP procedure at the time of lip repair.105,106
Grayson and Cutting later proposed the use of a nasoalveo­
lar molding device before the GPP and primary lip repair
to accomplish the same alveolar ridge fusion. Several studies
confirm that the GPP procedure has a high osteogenic
potential that results in the deposition of bone to achieve
successful fusion of the alveolar ridge. Unfortunately, clini­
cal studies do not confirm sufficient bone fill to consistently
allow for the eruption of the canine through the ridge and
to provide ideal periodontal support. Since the late 1970s,
clinical studies have been carried out to assess the long-term
effects of GPP on midfacial growth in cleft patients.
In 1999, Millard and colleagues used serial dental casts
to evaluate the effects of GPP on maxillomandibular rela­
tionships.106 They found a greater frequency of anterior
crossbite in the GPP group than in the non-GPP group.
They also confirmed that the GPP group had a shorter
anteroposterior length of the maxilla as compared with the
non-GPP group at 6 years of age. Similar findings of poor
midface growth have been documented by Berkowitz and
colleagues,9 Matic and Powers,99 and others.70,169 In 2005,
Renkielska and colleagues evaluated the impact of GPP on
occlusal relationships with the use of the Goslon Yardstick
occlusal grading system.143 They found that patients treated
with GPP had a poor occlusal relationship and a Goslon
Yardstick score of 4 and 5, thus indicating need for

• Figure 32-1  A, The maxillofacial skeleton of an adult with unrepaired unilateral cleft lip and palate
(UCLP) is shown. Typical UCLP deformities of the hard palate, the alveolus, the nasal septum, the
floor of the nose, the nasal spine, and the pyriform rims are demonstrated. B, An occlusal view of a
child born with UCLP who presented during the mixed dentition with a residual alveolar/palatal skeletal
defect and a labial and palatal oronasal fistula is also shown.
A
B
• Figure 32-2  A 6-year-old boy who was born with a UCLP is shown after soft tissue lip and palate
repair in infancy. A, Frontal and B, oblique facial and computed tomography scan views during the
mixed dentition demonstrate residual cleft skeletal defects and deformities that were present before
grafting and fistula closure.
Or
Or
Or
Or
Or
Or
Or
Or
Orthththththththt ogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatathicicicicicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C. Pos
os
os
os
os
os
os
o nicicicicick
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Orththththththththththththththththththththththt ogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatathicicicicicicicicicicicicicicicici Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
osnicicicicicicicick
A
B

1300 SECTION 5  Cleft Jaw Deformities
• Figure 32-3  An example of the suboptimal management of a unilateral alveolar/palatal cleft. A child
who was born with UCLP underwent soft tissue lip and palate repair during infancy. She underwent
orthodontic treatment and achieved a satisfactory overjet and overbite. The alveolar/palatal cleft
skeletal defects and the residual labial and palatal oronasal fistula were ignored. The maxillary lateral
incisor tooth was inadequate and required extraction. The canine erupted adjacent to the cleft, and
the dental gap was orthodontically retained. Dental rehabilitation was accomplished with a three-unit
bridge to manage the cleft–dental gap. Limited periodontal (bone) support of the cleft-adjacent teeth
(the canine and the central incisor) resulted in a periodontic–endodontic problem with a loss of the
canine and poor support of the central incisor. These problems would not have occurred had effective
bone grafting and fistula closure been carried out during the mixed dentition. A, Occlusal and palatal
views during the mixed dentition before the full eruption of the canine through the non-grafted alveolar
cleft. B, Occlusal and palatal views are also shown during orthodontic alignment (extraction of right
bicuspid was carried out). C, Occlusal view at the completion of orthodontic alignment. A periapical
radiograph indicates limited bony support of the cleft-adjacent teeth. D, An occlusal view is next
shown after the placement of a three-unit bridge across the cleft–dental gap. A periapical radiograph
confirms that, after several years, a periodontal–endodontic problem resulted in irreversible injury to
the cleft-adjacent canine.
C
D
A
B

1301
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
Alveolar mucosa
(non-keratinized)
Alveolar bone
Mucogingival junction
Attached gingiva (keratinized)
Free marginal gingiva (keratinized)
Periodontal sulcus (pocket)
Periodontal ligament
Junctional epithelial attachment
Probing depth
(pocket)
Cemental enamel junction
Clinical
attachment
loss
Crown (enamel)
B
A
C
• Figure 32-4  The importance of maintaining keratinized tissue adjacent to the cervical margin of each tooth
for long-term periodontal health cannot be overemphasized (see Chapter 6). A, Illustration of a cross-section of
dentoalveolar anatomy that indicates the location of the gingiva (keratinized mucosa) adjacent to the tooth surface.
B, Low-power histomicrograph cross-section of the dentoalveolar anatomy that indicates the microscopic character
of the keratinized mucosa adjacent to the tooth surface. Just deep to the keratinized epithelium rete pegs, organized
fibrous tissue can be seen. C, Intraoperative view of an elevated mucogingival flap advanced anteriorly for the closure
of the labial aspect of the cleft oronasal fistula. The flap brings the gingiva into the region where the canine will erupt.
Continued

1302 SECTION 5  Cleft Jaw Deformities
the GPP procedure. As a result of their study, the Chang
Gung Cleft Center no longer uses the GPP technique.
Coordinated Team Approach
Care of the patient with UCLP is best delivered by an
integrated group of specialists who evaluate and provide
comprehensive definitive care. It is no longer acceptable
for individual practitioners (e.g., surgeons, orthodontists,
restorative dentists, speech pathologists, otolaryngologists)
to carry out extended treatment without considering all
aspects of the patient’s care and without discussing options
with members of the team.82,90
A frequent road block to successful reconstruction and
dental rehabilitation of the midface-deficient adolescent
with UCLP—especially in those who present with other
residual clefting problems (see the section about residual
deformities later in this chapter)—is disagreement between
clinicians about the indications, the most effective tech­
niques, and the timing of intervention.8,104,123 The surgeon,
the orthodontist, the dental and medical team, and the
orthognathic surgery. They reaffirmed that the inclusion of
the alveolar process in the primary lip repair increased severe
occlusal maldevelopment.
More recently, Hsieh and colleagues completed a retro­
spective clinical study to evaluate the effects of GPP on
facial growth in patients with UCLP.74 Sixty-two consecu­
tive patients with non-syndromal complete UCLP with
records from when they were 5 years old were included in
the study. All of the patients had received nasoalveolar
molding treatment before primary lip repair. Those indi­
viduals who underwent GPP at the time of lip repair (n =
26) were placed in one group. Those that did not undergo
GPP at the time of lip repair (n = 36) were placed in another
group. Cephalometry was used to evaluate facial growth at
5 years of age in the two treatment groups. GPP was found
to have significant negative effects on the maxillary position
(i.e., diminished horizontal projection), the intermaxillary
position (i.e., negative overjet), the maxillary length, and
the maxillary alveolar length at the age of 5 years. The
authors concluded that, in patients with UCLP, the sagittal
growth of the maxilla was significantly adversely affected by
Incision line
Cleft
A
B
C
D
E
Backcut
Tongue
Mucogingival junction
E
D
F
G
D, Illustration of incision placement for the elevation of the mucogingival flap. The flap splits the attached gingiva posteriorly
adjacent to the first molar so that the advancing flap brings keratinized tissue into the cleft site without denuding the attached gingiva from the
posterior teeth. E, Low-power histomicrograph that demonstrates keratinized epithelium, organized fibrous tissue, and rete pegs. F, Palatal view
of a patient with UCLP during the mixed dentition. This patient underwent the closure of an oronasal fistula using a buccal “finger” flap by another
surgeon. This is not the preferred way to rearrange the intraoral tissue for fistula closure; the labial vestibule is destroyed, and non-keratinized
buccal mucosa is brought into the region where the canine will erupt. G, Low-power histomicrograph of non-keratinized buccal mucosa. Note the
thin layer of non-keratinized epithelium with the underlying loose areolar connective tissue. Part D modified from an original illustration by Bill Winn.
• Figure 32-4, cont’d

1303
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
C
D
Hard palate
Palatal flaps
Palatal flaps
Nasal
mucosa
Oronasal fistula
Backcut
Incision line
Cleft
A
B
C
D
E
Backcut
Tongue
Mucogingival junction
A
B
• Figure 32-5  Illustrations of basic technique for bone grafting and the management of residual oronasal fistula during the mixed dentition of a
child with a UCLP. If required, orthodontic expansion to correct the arch width precedes mixed-dentition bone grafting. A, A view of the left labial
mucogingival incision in progress. B, Attention is turned to the sharp palate for the separation of the oral and nasal mucosa at the site of the fistula
and then the elevation of the flaps. C, Subperiosteal elevation of the palatal flaps. D, With the palatal flaps elevated, the nasal mucosa is separated
from the floor of the nose on each side. Left and right labial flaps have already been elevated. The nasal mucosa is further separated from the
bony surface along the distal aspect of the central incision for later closure. Part A modified from an original illustration by Bill Winn.
Continued

1304 SECTION 5  Cleft Jaw Deformities
a minimum by an orthodontist, an orthognathic surgeon,
an otolaryngologist, and a speech pathologist. Additional
consultations with other dental (ex. prosthodontist, pediat­
ric or general dentist, periodontist) and medical (i.e., sleep
specialist, geneticist) specialists are carried out as indicated.
For the evaluation of a cleft dentofacial deformity, records
and tests should include medical-quality photographs,
cephalometric and dental radiographs, dental models, direct
facial measurements, speech and velopharyngeal (VP)
assessment (e.g., nasoendoscopic instrumentation), and a
thorough evaluation of the upper airway.
The primary surgeon not only performs repair of the cleft
lip and palate and corrects VP insufficiency but also plays
a role in directing the patient’s overall care. If the primary
patient and his or her family must first agree about the
dental, occlusal, speech, upper airway, and aesthetic objec­
tives; only then can effective treatment go forward.
The advantage of coordinated care was confirmed by the
Eurocleft Study, which found a lack of association between
high-intensity disjointed treatment and favorable results. In
other words, the greater the number of operations and the
greater the number of years of orthodontic appliances worn
(i.e., heavy burden of care), the worse the outcome.25,108
Treatment Protocol
Patients who present with a UCLP jaw deformity after
being referred for possible orthognathic surgery are seen at
Cancellous bone graft
Labial mucogingival
advancement flap
A
B
C
D
E
Palatal flaps
E
Nasal mucosa
Labial mucosa
F
G
E, Suturing of the nasal flaps for watertight closure is in progress. A hemostat is placed through the left
nostril to demonstrate the partially sutured nasal floor. The palatal flaps have been sutured together for fistula closure. F, After the
nasal flaps are sutured, iliac cancellous bone graft is packed into the palatal, alveolar, and floor-of-the-nose skeletal defects. G, Left
and right labial and palatal flaps are advanced for watertight closure. By advancing the left labial mucogingival flap, the keratinized
tissue has been placed over the alveolar ridge, where the canine will eventually erupt. This is accomplished by splitting the attached
gingiva at the last molar.
• Figure 32-5, cont’d

1305
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
• Figure 32-6  A 16-year-old girl was born with van der Woude syndrome, including UCLP and lower lip pits. She has
been managed by this surgeon since her birth. She underwent lip and palate repair during her early childhood, which
was followed by successful bone grafting and fistula closure during the mixed dentition. She had normal maxillary
growth and underwent standard orthodontic treatment with the maintenance of the cleft–dental gap. She underwent
an open rhinoplasty that included the use of a septal cartilage (caudal strut) graft when she was 15 years old. There
is sufficient bone volume and attached gingiva for a dental implant, which is planned for when she is 18 years old.
A, Facial views at the time of birth and during the mixed dentition, just before alveolar/palatal bone grafting. B, Facial
and occlusal views taken when the patient was a teenager after rhinoplasty and before dental implant placement. A
removable retainer that includes the missing lateral incisor is in place. C, Panorex image taken when the patient was
a teenager during orthodontic treatment indicates successful bone grafting of the alveolar cleft.
B
A
C
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
O thththththt ogn
gn
g atata hicicic Su
Su
Su
Su
S rgery
ry
ry
ry
ry; J.C.
C. Pos
osnicick
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Orthththththththththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatata hicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
S rgery
ry
ry
ry; J.C.
C.
C.
C. Pos
os
os
osnicici k
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
O ththththththththththththththththogn
gn
gn
g athicicici Su
Su
Su
S rgery
r ; J.C. Pos
os
os
osnicicicicicicicicicicicicicicicicicicicici k

1306 SECTION 5  Cleft Jaw Deformities
anterior nasal spine) should be carried out at the time of
orthognathic surgery (see Chapters 10 and 15).150
Discussions among the treating medical and dental con­
sultants, the patient, and the family clarify the need for and
the extent of orthognathic and intranasal procedures. The
overall plan for speech, jaw, upper airway, and dental reha­
bilitation and for the enhancement of facial aesthetics is
agreed upon before the initiation of treatment.
Timing of Orthognathic Surgery
Definitive correction of the jaw deformity is best carried out
when the skeleton is mature and before the patient finishes
high school.82,90 Maxillofacial growth is generally complete
between the ages of 14 and 16 years in girls and 16 and
18 years in boys. However, skeletal growth is variable and
may be further gauged by an analysis of sequential lateral
cephalometric radiographs taken at 6-month intervals (see
Chapter 17). The patient’s and the family’s preferences for
the timing of the operation on the basis of psychosocial and
functional needs are also taken into account.
As early as 1986, investigators showed that, if maxillary
advancement is performed during the mixed dentition in a
patient with a cleft palate, then another orthognathic pro­
cedure will be necessary for definitive correction after skel­
etal maturity is reached.181-184 All research to date indicates
that a Le Fort I osteotomy carried out during the mixed
dentition in a patient with clefting—whether with standard
or distraction osteogenesis (DO) techniques—results in no
further horizontal maxillary growth (see the controversies
section later in this chapter).21,41,40,75
Residual Deformities in the Adolescent
with Unilateral Cleft Lip and Palate
Correction of the residual skeletal, soft-tissue, and dental
deformities in the adolescent patient with UCLP challenges
the ingenuity and skill of the orthognathic surgeon and the
cleft team. The central deformity is maxillary hypoplasia
(Figs. 32-7, 32-8, and 32-9), and it is frequently combined
with residual oronasal fistula, bone defects, intranasal
obstruction, soft-tissue scarring, and, occasionally, VP dys­
function (Figs. 32-10 through 32-21).84,167 In addition, the
maxillary lateral incisor at the cleft site is usually congeni­
tally absent or deficient, thereby resulting in a cleft–dental
gap. Secondary deformities of the nose, the mandible, and
the chin region are also common.
The prevalence of these residual clefting deformities in
mature patients with UCLP varies widely depending on
the primary cleft surgeon’s philosophy, available expertise,
the individual’s intrinsic biologic growth potential, and the
patient’s and family’s interests. Published clinical surveys
of individuals who were born with complete UCLP and
treated at established cleft centers provide insight into alveo­
lar cleft management. The studies indicate that, despite a
cleft surgeon is not trained in skeletal procedures, then a
timely and seamless transition to the orthognathic maxil­
lofacial surgeon should occur. The surgeon who is caring for
the patient with clefting and a skeletal deformity should
have a fundamental understanding of the patient’s dental,
speech, upper airway, and aesthetic needs. He or she should
request consultation with appropriate specialists, evaluate
the clinical information, and be prepared to perform
orthognathic and intranasal procedures.
The orthodontist provides interceptive treatment during
the mixed dentition in association with bone grafting and
carries out definitive orthodontic treatment in conjunction
with orthognathic surgery, when indicated. From the mixed
dentition phase, the cleft orthodontist should recognize the
patient with UCLP who may require orthognathic surgery.*
The institution of extensive camouflage (dental compensa­
tory) treatment is likely to jeopardize periodontal health
and lead to late dental relapse. Proceeding with a compro­
mised (camouflage) orthodontic approach should only be
entered into with full disclosure to the family and other
treating clinicians.
Before orthognathic surgery, a speech pathologist evalu­
ates the patient to characterize VP function and to identify
articulation errors that result from the cleft palate jaw defor­
mity and the dental malocclusion (see Chapter 8). A base­
line evaluation is important, because VP function may
deteriorate after maxillary advancement. A nasoendoscopic
guided speech assessment is useful to provide maximum
objective data.180 VP closure that is adequate before surgery
may become borderline afterward, and VP closure that is
borderline may become inadequate. Studies document
that only a small percentage of patients require a primary
pharyngeal flap or flap revision after maxillary advance­
ment.19,20,57,60,79,86,91,95,102,125,170,180 Articulatory distortions
that result from malocclusion are also identified and cause-
and-effect relationships are determined. The successful
orthodontic and surgical correction of crossbites, open bite,
cleft–dental gaps, negative overjet, and residual oronasal
fistulas represents the most effective way to correct the
identified articulation distortions (see Chapter 8). Unfortu­
nately, the use of “oral–motor therapy” is still often applied
to manage both jaw-deformity–related articulation errors
and VP insufficiency; however, this type of treatment has
not been proven beneficial for dynamic speech.
A thorough evaluation of the upper airway is conducted
to assess for areas of obstruction (see Chapter 10). Studies
suggest an increased prevalence of sleep-disordered breath­
ing and obstructive sleep apnea in patients with cleft palate,
especially in the presence of Robin sequence. A formal sleep
study (i.e., an attended polysomnogram) is performed if
there is a suggestion of obstructive sleep apnea (see Chapter
26). If indicated, simultaneous intranasal procedures (e.g.,
septoplasty; reduction of the inferior turbinates; recontour­
ing of the nasal apertures, the floor of the nose, and the
*References 14, 15, 28, 29, 42, 47-49, 54, 80, 98, 96, 97, 109-111, 121,
144, 147, 152-154, 157, 161
Text continued on p. 1355

1307
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
Orth
r ognathic Surgery; J.C. Posnick
Orth
r ognathic Surgery; J.C. Posnick
Orth
r ognathic Surgery; J.C. Posnick
Orth
r ognathic Surgery; J.C. Posnick
A
B
• Figure 32-7  A 19-year-old woman was born with UCLP on the left side and underwent lip and palate repair followed by effective bone
grafting and fistula closure during the mixed dentition. She has a useful lateral incisor at the cleft site but is missing the lateral incisor on
the non-clefted side. A bicuspid has also been removed on the cleft side. As a teenager, she was referred to this surgeon and underwent
a combined orthodontic and orthognathic surgical approach. The procedure included a standard Le Fort I osteotomy (horizontal advance­
ment, vertical lengthening) and interpositional grafting. A, Frontal views with smile before and after reconstruction. B, Profile views before
and after reconstruction.
Continued

1308 SECTION 5  Cleft Jaw Deformities
C
D
E
C, Occlusal and palatal views before and after reconstruction. D, Articulated dental
casts that indicate analytic model planning. E, Lateral cephalometric radiographs before and after recon­
struction. Note that, by limiting surgery to the maxilla, the cant (which also involved the mandible) cannot
be corrected.
• Figure 32-7, cont’d

1309
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
• Figure 32-8  A 20-year-old man was born with
UCLP on the right side. Primary lip and palate repair
were carried out at another institution. He was
referred to this surgeon and underwent successful
bone grafting and fistula closure during the mixed
dentition. He underwent orthodontic closure of the
cleft–dental gap and limited alignment by the time
he was 14 years old. Final orthodontic decompensa­
tion was later carried out in combination with orthog­
nathic surgery when he was 19 years old. The
patient’s procedures included maxillary Le Fort I
osteotomy (horizontal advancement, vertical short­
ening, midline correction, cant correction, and clock­
wise rotation) with interpositional grafting; sagittal
split ramus osteotomies (counterclockwise rotation
and asymmetry correction); osseous genioplasty
(horizontal advancement); and septoplasty, inferior
turbinate reduction, and recontouring of the floor of
the nose. A, The patient is shown during the mixed
dentition after successful bone grafting and fistula
closure. B, Frontal views in repose before and after
reconstruction. C, Frontal views with smile before
and after reconstruction.
B
C
©Elsevier Inc.
Or
Or
Orthththththththththththththththththththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatata hicicicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
osnicicicicicicicicicicicicicicicicicicicicicick
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Orthththththththththththththththth
r ogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatathicicicicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C. Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
osnicicicicicicicicicicicicicicick
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Or
Orththththththththththththththththththththththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatathicicicicicicicicicicicici Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
o nicicicicicicicici k
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Orthththththththththththt ogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatathicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
o nicicicick
©Elsevier Inc.
Or
Or
Or
Orthththththththththththththththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatatatatatatathicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C. Pos
os
os
os
os
os
os
o nicicicicicicicici k
A
Continued

1310 SECTION 5  Cleft Jaw Deformities
D
E
Orth
r ognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orth
r ognathic Surgery; J.C. Posnick
Orth
r ognathic Surgery; J.C. Posnick
D, Oblique facial views before and after reconstruction. E, Profile views before and after reconstruction.
• Figure 32-8, cont’d

1311
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
F
Prior to treatment with successful mixed dentition graft
Pre surgery
After treatment
F, Occlusal views before definitive orthodontics, with decompensation in progress before surgery, and after
reconstruction.
• Figure 32-8, cont’d 
Continued

1312 SECTION 5  Cleft Jaw Deformities
G
H
Vertical
lengthening
(4mm)
Mandibular
adjustment
G, Articulated dental casts that indicate analytic model planning. H, Lateral cephalometric radiographs before and after
reconstruction.
• Figure 32-8, cont’d

1313
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
©Elsevier Inc.
Or
Or
O thththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gnatatatatatatathicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
o nicicicicicicicicick
A
• Figure 32-9  A high school senior was born with
a complete UCLP on the left side. He underwent lip
and palate repair at another institution. He was then
referred to this surgeon and underwent successful
bone grafting and fistula closure during the mixed
dentition. He developed a jaw deformity that was
characterized by maxillary deficiency and secondary
deformities of the mandible and the intranasal cavity.
He underwent a combined orthodontic and surgical
approach. Orthodontic decompensation included
cleft–dental gap closure (absent lateral incisor). He
then underwent jaw reconstruction. The patient’s
procedures included maxillary Le Fort I osteotomy
(horizontal advancement, vertical shortening, midline
correction, cant correction, and clockwise rotation)
with interpositional grafting; sagittal split ramus oste­
otomies (clockwise rotation and asymmetry correc­
tion); osseous genioplasty (vertical shortening); and
septoplasty, inferior turbinate reduction, and recon­
touring of the floor of the nose. Six months after
successful orthognathic surgery, the patient under­
went cleft rhinoplasty including rib cartilage (caudal
strut) grafting. A, The patient is shown during the
mixed dentition before bone grafting and fistula
closure. B, Frontal views in repose before and after
reconstruction. C, Frontal views with smile before
and after reconstruction.
Continued
B
C
©Elsevier Inc.
Or
Or
Or
Or
O ththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gnatatatatatatatatatatatatatatatathicicicicicicicicicicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
osnicicicici k
©Elsevier Inc.
Or
Or
Or
Or
O thththththogn
gn
gn
gn
gn
gn
gn
gn
gnatatatata hicicicicic Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry; J.C.
C Pos
os
os
os
os
os
osnicicick
©Elsevier Inc.
Or
Orththogn
gn
gn
gn
gn
gn
g atatatatatatatata hicicicicicicicicicicicicicicicicicicicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C. Pos
os
os
os
os
os
os
os
os
o nicicicici k
©Elsevier Inc.
Or
Or
Orthththogn
gn
gn
gn
gn
gn
gnatatatatatatatathicicicic Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C Pos
os
os
os
o nicici k

1314 SECTION 5  Cleft Jaw Deformities
D
E
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
D, Oblique facial views before and after reconstruction. E, Profile views before and after reconstruction.
• Figure 32-9, cont’d

1315
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
F
Prior to treatment with successful mixed dentition graft
Pre surgery
After treatment
F
F, Occlusal views before definitive orthodontics, with orthodontic dental decompensation in progress, and
after reconstruction.
• Figure 32-9, cont’d 
Continued

1316 SECTION 5  Cleft Jaw Deformities
H
I
Maxillary cant
correction
Mandibular
adjustment
G
Maxillary clockwise
rotation
Mandibular
adjustment
G & H, Articulated dental casts that indicate analytic model planning. Note that, with the clockwise rotation
of the mandible, the pogonion moves posterior while the incisors do not. I, Lateral cephalometric radiographs before and after
reconstruction.
• Figure 32-9, cont’d

1317
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
Circumvestibular
incision in UCLP with
residual cleft/fistula
Separation of oral and
nasal mucosa at fistula
Separation of labial and
palatal mucosa at cleft
UCLP with secondary and residual jaw/
intranasal deformities
Modified LeFort I in two segments
Bone graft
A
B
• Figure 32-10  Illustrations of modified Le Fort I osteotomy in two segments as carried out in a
patient with UCLP who did not undergo successful grafting during the mixed dentition and who
presents as an adult with a jaw deformity. A, Frontal view of maxillofacial skeleton before and after
Le Fort I osteotomy in two segments. The inferior turbinates have been reduced, and a submucous
resection of the deviated septum has been performed. The nasal floor and the nasal spine have
been recontoured with a rotary drill. Cancellous iliac bone graft has also been placed along the
cleft nasal floor. Corticocancellous iliac graft will also be placed in gaps along the anterior maxilla
on each side. B, Circumvestibular and perifistular incisions for exposure to complete osteotomies
for down-fracture and later fistula closure.
Continued

1318 SECTION 5  Cleft Jaw Deformities
After modifed Le
Fort I in 2 segments
Closure of cleft dental
gap with approximation
of attached gingiva
UCLP with residual cleft skeletal deformities prior to and
after modified Le Fort I in 2 segments
UCLP maxilla after down fracture
Closure of nasal mucosal layer
C
Maxillary segments repositioned into
splint with closure of cleft dental gap
D
E
C, Down-fractured Le Fort I osteotomy in two segments after the submucosal resection of
the septum, the reduction of the inferior turbinates through the nasal mucosa opening, and then watertight nasal-side
closure. D, Wound closure of both the labial and palatal aspects after differential segmental repositioning. E, Palatal
view of bony segments before and after repositioning. Parts A-E modified from an original illustration by Bill Winn.
• Figure 32-10, cont’d

1319
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
A
B
• Figure 32-11  A 17-year-old girl who was born with UCLP. She underwent lip and palate repair during childhood, but she did not undergo
effective bone grafting during the mixed dentition. A useful lateral incisor is not present at the cleft site. She was referred to this surgeon and
underwent a comprehensive orthodontic and orthognathic surgical approach. The patient’s procedures included a modified Le Fort I osteotomy in
two segments (differential repositioning of the segments) with interpositional grafting as well as closure of the oronasal fistula, the cleft–dental gap,
and the alveolar defect; an osseous genioplasty (vertical reduction and horizontal advancement); and septoplasty, inferior turbinate reduction, nasal
floor recontouring. A, Frontal views with smile before and after reconstruction. B, Oblique views before and after reconstruction.
Continued

1320 SECTION 5  Cleft Jaw Deformities
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
C
D
C, Profile views before and after reconstruction. D, Worm’s-eye views before and after reconstruction.
• Figure 32-11, cont’d

1321
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
F
X
Pre surgery with residual cleft defects
After treatment
E
E, Occlusal views with orthodontics in progress and after reconstruction. F, Palatal views with orthodontics in
progress and after reconstruction. Note: The maxillary dentition is reconstructed with only eleven teeth. This provides a low maintenance
dentition for the patient going forward.
• Figure 32-11, cont’d 
Continued

1322 SECTION 5  Cleft Jaw Deformities
G
Cant correction
H
I
G and H, Views of articulated dental casts that indicate analytic model planning. I, Lateral cephalometric
radiographs before and after reconstruction. A, E (top Center), E (bottom center), F, From Posnick JC, Tompson B: Modification
of the maxillary Le Fort I osteotomy in cleft–orthognathic surgery: the unilateral cleft lip and palate deformity, J Oral Maxillofac Surg
50:666-675, 1992.
• Figure 32-11, cont’d

Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
A
B
• Figure 32-12  A 19-year-old man who was born with UCLP. He underwent lip and palate repair during childhood, but he did not undergo effec­
tive bone grafting during the mixed dentition. A useful lateral incisor is not present at the cleft site. He was referred to this surgeon and underwent
a combined orthodontic and orthognathic surgical approach. The patient’s procedures included a modified Le Fort I osteotomy in two segments
(differential repositioning of the segments) with interpositional grafting, the correction of occlusal canting, and the closure of the oronasal fistula,
the alveolar defect, and the cleft–dental gap; bilateral sagittal ramus osteotomies (correction of asymmetry); osseous genioplasty (vertical reduction
and horizontal advancement); and septoplasty, inferior turbinate reduction, and nasal floor recontouring. A, Frontal views in repose before and after
reconstruction. B, Frontal views with smile before and after reconstruction.
Continued

1324 SECTION 5  Cleft Jaw Deformities
Orthognathic Surgery; J.C. Posnick
Orth
r ognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orth
r ognathic Surgery; J.C. Posnick
C
D
C, Oblique views before and after reconstruction. D, Profile views before and after reconstruction.
• Figure 32-12, cont’d

1325
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
F
Roll Orientation Correction
E
E, Occlusal and palatal views with orthodontics in progress and after reconstruction.
F and G, Articulated dental casts that indicate analytic model planning.
• Figure 32-12, cont’d 
Continued

1326 SECTION 5  Cleft Jaw Deformities
G
H
H, Lateral cephalometric radiographs before and after reconstruction. B, D,
E, From Posnick JC, Tompson B: Modification of the maxillary Le Fort I osteotomy in cleft–orthognathic
surgery: the unilateral cleft lip and palate deformity, J Oral Maxillofac Surg 50:666-675, 1992.
• Figure 32-12, cont’d

1327
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
A
B
• Figure 32-13  A 17-year-old boy who was born with UCLP. He underwent lip and palate repair during childhood, but he did not undergo effec­
tive bone grafting during the mixed dentition. The lateral incisor is not present at the cleft site. He was referred to this surgeon as a teenager and
underwent a combined orthodontic and orthognathic surgical approach. The patient’s procedures included a modified Le Fort I osteotomy in two
segments (differential repositioning of the segments) with interpositional grafting and closure of the oronasal fistula, the alveolar defect, and the
cleft–dental gap; and septoplasty, inferior turbinate reduction, and nasal floor recontouring. A, Frontal views in repose before and after reconstruc­
tion. B, Frontal views with smile before and after reconstruction.
Continued

1328 SECTION 5  Cleft Jaw Deformities
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
C
D
C, Oblique views before and after reconstruction. D, Profile views before and after reconstruction.
• Figure 32-13, cont’d

1329
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
F
G
Pre surgery with residual cleft defects
After treatment
Before and After Treatment
E
E, Occlusal views with orthodontics in progress and after reconstruction. F, Palatal
views with orthodontics in progress and after reconstruction. G, Lateral cephalometric radiographs before and
after reconstruction. B, D, E, F, From Posnick JC, Dagys AP: Skeletal stability and relapse patterns after Le Fort
I maxillary osteotomy fixed with miniplates: the unilateral cleft lip and palate deformity, Plast Reconstr Surg
94:924-932, 1994.
• Figure 32-13, cont’d

1330 SECTION 5  Cleft Jaw Deformities
C
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
O ththththththththththththththththththththththththththththththththththththththththththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatatatatathicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C. Pos
os
os
os
os
os
os
os
os
os
os
os
osnicicicicicicicicicicicicicicicicicicicicicicicicicicicicicick
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
O thththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththt ogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatathicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C. Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
osnicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicici k
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Orththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththt ogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatata hicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicici Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
osnicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicick
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Orthththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatata hicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicici Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
osnicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicick
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
O ththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatata hicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
osnicicicicicicicicicick
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
O thththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatata hicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C. Pos
os
os
os
os
os
o nicicicicicicick
A
B
• Figure 32-14  A 20-year-old man who was
born with UCLP on the left side. He underwent
lip and palate repair during childhood, but he
did not undergo effective bone grafting during
the mixed dentition. A useful lateral incisor is
not present at the cleft site. He has a retained
primary molar and an absent lateral incisor on
the non-cleft side. He was referred to this
surgeon as a young adult and underwent
a combined orthodontic and orthognathic
surgical approach. The patient’s procedures
included a modified Le Fort I osteotomy in two
segments (differential repositioning of the seg­
ments) with interpositional grafting and closure
of the oronasal fistula, the alveolar defect, and
the cleft–dental gap; and septoplasty, inferior
turbinate reduction, and nasal floor recontour­
ing. A, Frontal views with smile before and
after reconstruction. B, Oblique views before
and after reconstruction. C, Profile views
before and after reconstruction.

1331
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
E
Pre surgery with residual cleft defects
After treatment
Before and After Treatment
D
D, Occlusal views with orthodontics in progress and after reconstruction. E, Palatal views with orthodontics in
progress and after reconstruction.
• Figure 32-14, cont’d 
Continued

1332 SECTION 5  Cleft Jaw Deformities
G
H
F
Closure of cleft
dental gap
F and G, Articulated dental casts that indicate analytic model planning. H, Lateral cepha­
lometric radiographs before and after reconstruction. A, C, D, F, From Posnick JC, Dagys AP: Skeletal stability and
relapse patterns after Le Fort I maxillary osteotomy fixed with miniplates: the unilateral cleft lip and palate deformity,
Plast Reconstr Surg 94:924-932, 1994.
• Figure 32-14, cont’d

1333
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
A
B
• Figure 32-15  A 24-year-old schoolteacher who was born with UCLP. She underwent 4 bicuspid extractions as part of earlier orthodontic treat­
ment. The retained lateral incisor at the cleft does not have an adequate root and required extraction. She was referred to this surgeon as an adult
and underwent a combined orthodontic and orthognathic surgical approach. The patient’s procedures included a modified Le Fort I osteotomy in
two segments (differential repositioning of the segments) and closure of the oronasal fistula, the alveolar cleft, and the cleft–dental gap; bilateral
sagittal split ramus osteotomies; osseous genioplasty; and septoplasty, inferior turbinate reduction, and nasal floor recontouring. A, Frontal views
in repose before and after reconstruction. B, Frontal views with smile before and after reconstruction.
Continued

1334 SECTION 5  Cleft Jaw Deformities
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
C
D
C, Oblique views before and after reconstruction. D, Profile views before and after reconstruction.
• Figure 32-15, cont’d

1335
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
F
Closure of cleft
dental gap
G
8.5
Pre surgery with residual cleft defects
After treatment
E
X
X
E, Occlusal views with orthodontics in progress and after reconstruction and dental
rehabilitation. F, Articulated dental casts that indicate analytic model planning. G, Lateral cephalometric
radiographs before and after reconstruction.
• Figure 32-15, cont’d

1336 SECTION 5  Cleft Jaw Deformities
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
A
B
• Figure 32-16  A 17-year-old boy who was born with UCLP. He was referred to this surgeon during the late mixed dentition and underwent
effective bone grafting. He was congenitally missing his lateral incisor at the cleft site. There is transposition of the canine and the first bicuspids
on each side of the upper jaw. He underwent a combined orthodontic and orthognathic surgical approach. The patient’s procedures included a
standard Le Fort I osteotomy (horizontal advancement) with interpositional grafting; osseous genioplasty (vertical reduction and horizontal advance­
ment); and septoplasty, inferior turbinate reduction, and nasal floor recontouring. A, Frontal views in repose before and after reconstruction.
B, Frontal views with smile before and after reconstruction.

1337
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
C
D
C, Oblique views before and after reconstruction. D, Profile views before and after reconstruction.
• Figure 32-16, cont’d 
Continued

1338 SECTION 5  Cleft Jaw Deformities
F
E
Pre surgery
After treatment
E, Occlusal views with orthodontics in progress and after reconstruction. F, Palatal views with orthodontics in progress
and after reconstruction.
• Figure 32-16, cont’d

1339
CHAPTER 32  Cleft–Orthognathic Surgery: The Unilateral Cleft Lip and Palate Deformity
G
H
G, Articulated dental casts that indicate analytic model planning. H, Lateral cephalometric radiographs before and after
reconstruction. Note that cosmetic recontouring and modification of the maxillary dental crowns would be beneficial.
• Figure 32-16, cont’d