Plastic and Reconstructive Surgery Board Review Pearls of Wisdom 3th

CHAPTER 63 Pharyngoplasty/Pharyngeal Flaps/Speech T erapy
617

Is SP better than PFF?
It is not clear. wo randomized trials ound no di erence between the two procedures in terms o speech
improvement, operative complications, or incidence o sleep apnea. However, a meta-analysis showed possible
improved outcomes with a pharyngeal f ap. T e velopharyngeal closure pattern may impact the relative e cacy
o these procedures. SP is thought to be easier to per orm than PPF, with no need to divide the so t palate. SP is
also easily revisable, in that the f aps can be reelevated and made tighter (i hypernasality persists) or looser (i sleep
apnea becomes a problem).

For each o the ollowing velopharyngeal closure patterns, what treatment is (theoretically) most
appropriate?
Coronal pattern, with little lateral wall movement

Sphincter pharyngoplasty
Sagittal pattern, with good lateral movement but poor velar
movement
Pharyngeal f ap
Sphincteric pattern, with small gap

Sphincter pharyngoplasty
Sphincteric pattern, with large gap

Pharyngeal f ap
No closure (poor velar and lateral movement)

Large pharyngeal f ap or obturator
Good velar movement, small gap, anteriorly directed velar muscles

Double-opposing Z-plasty (Furlow)
REFERENCES
Abyholm F, D’Antonio L, Davidson Ward SL, et al. Pharyngeal f ap and sphincteroplasty or velopharyngeal insu ciency have equal
outcome at 1 year postoperatively: results o a randomized trial. Cleft Palate Craniofac J. 2005;42(5):501–511.
Armour A, Fischbach S, Klaiman P, Fisher DM. Does velopharyngeal closure pattern a ect the success o pharyngeal f ap pharyngoplasty?
Plast Reconstr Surg. 2005;115(1):45–52.
Collins J, Cheung K, Farrokhyar F, Strumas N. Pharyngeal f ap versus sphincter pharyngoplasty or the treatment o velopharyngeal
insu ciency: a meta-analysis. J Plast Reconstr Aesthet Surg. 2012:65(7):864–868.
Fisher DM, Sommerlad BC. Cle t lip, cle t palate, and velopharyngeal insu ciency. Plast Reconstr Surg. 2011;128(4):342e–360e.
Khosla RK, Mabry K, Castiglione CL. Clinical outcomes o the Furlow Z-Plasty or primary cle t palate repair. Cleft Palate Craniofac J.
2008;45(5):501–510.
Kirschner RE, Baylis AL. Velopharyngeal dys unction. In: Neligan PC, ed. Plastic Surgery. New York, NY: Elsevier; 2013.
Sullivan SR, Vasudavan S, Marrinan EM, Mulliken JB. Submucous cle t palate and velopharyngeal insu ciency: comparison o speech
outcomes using three operative techniques by one surgeon. Cleft Palate Craniofac J. 2011;48(5):561–570.
Ysunza A, Pamplona C, Ramírez E, Molina F, Mendoza M, Silva A. Velopharyngeal surgery: a prospective randomized study o
pharyngeal f aps and sphincter pharyngoplasties. Plast Reconstr Surg. 2002;110(6):1401–1407.

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619
Pieter G.L. Koolen, MD and
Samuel J. Lin, MD, MBA, FACS

When was the f eld o cranio acial surgery established?
In 1967 a ter publication o Paul essier’s work in the paper “Osteotomies totales de ace, Syndrome de Crouzon,
Syndrome D’Apert, Oxycephalies, Scaphocephalies, urricephalies.”

What are the main insights that have helped to overcome the limitations in cranio acial surgery over the past
25 years?
Understanding o the pathologic anatomy through better imaging (3D C scan), extensive bone gra ting o
bony de ects, rigid xation o osteotomized segments, and most recently, the evolving applications o distraction
osteogenesis.

What is distraction osteogenesis?
A concept o xation that actively mobilizes osteotomy segments and promotes osteogenesis. Also known as
callostasis (stretching o callus, as in a racture).

What is the concept o distraction osteogenesis?
1. Distraction zone: location o the bone separation by osteotomy or corticotomy
2. Latency period: time allowed or reparative callus ormation in the distraction zone
3. Activation period: gradual distraction orces applied to callus, elongation o callus under tension
4. Consolidation period: external xation maintained a ter activation, allows consolidation o distraction generate.

What are the advantages o distraction osteogenesis?
Reduced risk o in ection seen in nonvascularized bone gra ts, eliminated need or a donor site hence no donor site
morbidity or limitations, simple design, more predictable bone survival, potential or three-dimensional changes,
reduced operative blood loss, reduced number o subsequent procedures, possibility o repeated applications,
avoids in situ metal plates and screws or maxillomandibular xation (MMF), early closure o tracheostomy in cases
o micrognathia, ability to be used in irradiated bone, well tolerated, no known evidence o growth retardation,
possibility o better aesthetic results.
Distraction Osteogenesis
CHAPTER 64

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rue/False: Distraction osteogenesis has a higher rate o morbidity and greater need or secondary mid ace
procedures compared to bone gra ting.
False.

rue/False: Distraction osteogenesis actually causes enlargement o the overlying surrounding tissue via
histiogenesis.
rue.

What are the disadvantages o mandibular distraction osteogenesis?
Hypertrophic scarring at pin site (most common disadvantage), pin tract in ection (osteomyelitis not reported
to date), pin extrusion or migration, acial nerve injury, sensory de cits o in erior alveolar nerve, intolerance or
noncompliance (uncommon), dentigerous cyst ormation, ankylosis, tooth bud injury in patients younger than 1
year, may still require secondary procedures.

How is new bone ormed during distraction osteogenesis?
Intramembranous ossi cation.

rue/False: Distraction is a unique orm o racture healing that occurs in the cranio acial skeleton without a
cartilaginous intermediate.
rue.

What are the zones o distraction osteogenesis that result in mature bone ormation?
Zone o brous tissue (zone I): the brous interzone (FIZ) is composed o highly organized, longitudinally
oriented, parallel strands o collagen with spindle-shaped broblasts and undi erentiated mesenchymal precursor
cells through the matrix. Central region o the distraction gap.
Zone o extending bone ormation (zone II): the primary mineralization ront (PMF) ound on both the edges
o the FIZ composed o broblasts and undi erentiated mesenchymal precursor cells in direct continuity
with osteoblasts on the sur ace o bone spicules (osteoblasts longitudinally oriented and parallel to direction
o distraction). Spindle-shaped broblastic cells trans orm into bone- orming cells. Increased levels o alkaline
phosphatase, pyruvic acid, and lactic acid. Also known as the transitional zone.
Zone o bone remodeling (zone III): advancing elds o bone resorption and apposition, increased number o
osteoclasts.
Zone o mature bone (zone IV): early compact cortical bone located adjacent to mature bone in unexpanded areas.
Bone spicules thicker, less longitudinal orientation.

When are chondrocytes present during distraction osteogenesis?
When there is excessive motion, brocartilage nonunion occurs.

What growth actor is currently thought to play a key role in the process o distraction osteogenesis?
rans orming growth actor-beta 1.

rue/False: T e blood supply in the distraction zone is decreased compared to the normal side.
False.

CHAPTER 64 Distraction Osteogenesis
621

How long does it take the bone in the distraction zone to achieve 90% o normal bony structure?
Usually within 8 months.

What are the clinical phases o distraction osteogenesis?
Latency phase: 5 to 7 days a ter corticotomy or osteotomy, initial racture healing bridges the cut bony sur aces
be ore initiating distraction.
Distraction phase: 3- to 5-week period o active stretching o the FIZ at 1 mm/day.
Mineralization phase: T e period o consolidation, a 7- to 9-week period a ter distraction when the PMF advances
rom each end toward the center, bridging the FIZ with bone.

rue/False: Failure o distraction osteogenesis has not been reported clinically in the cranio acial skeleton.
rue.

Although ailure o distraction osteogenesis has not been recorded in the cranio acial skeleton, what are
some o the actors Aronson has suggested could contribute to ailure?
Ischemic brogenesis: inadequate local blood supply, brous tissue ormation without bone ormation.
Cystic degeneration: caused by blockage o venous out ow, distraction gap lls with cystic cavity.
Fibrocartilage nonunion: caused by unstable xation, cartilage lls distraction gap.
Buckling o regenerate bone: xation device destabilized or removed prematurely.

At what age are bones potentially too so t to allow distraction?
Be ore age 18 months distraction is used with caution. Distraction is primarily used in the mandible between 18
months and 22 years o age. Recent literature supports the use o mandibular distraction in neonates with tongue-
based airway compromise as seen in Pierre Robin sequence.

What is the only indication or distraction in patients younger than 2 years?
ongue-based airway compromise secondary to mandibular hypoplasia. Pulling the mandible orward pulls the
tongue orward relieving the obstruction. Good outcomes have been seen in multiple institutions.

What are the specif c clinical indications or mandibular distraction in patients with Pierre Robin sequence?
• Syndromic patients with poor mandibular growth.
• Beyond 9 months and continued airway compromise secondary to tongue base obstruction.
• Candidates or tracheostomy.
• Failure o nonsurgical management.
• Age 2 to 4 dif cult time to distract.
• May also need adenoidectomy or tracheal reconstruction.

rue/False: Children younger than 2 years with hypoplasia or aplasia o the mandible without airway
compromise should not undergo distraction osteogenesis o the mandible because o the risk or permanent
dental injury.
rue.

In which grades o mandibular hypoplasia is distraction osteogenesis indicated?
Grades I, IIA, and IIB. In grade III, elongation is not possible because the ramus is absent.

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rue/False: Distraction can be accomplished regardless o so t-tissue def ciency since the so t tissues are
simultaneously expanded with bone distraction.
rue.

What is included in the clinical assessment o patients or mandibular distraction?
Anthropometric measurements, acial nerve unction, dental occlusion, dental cast impressions, preoperative
photos ( ull ace, pro le, three- ourth, basal views, biting tongue blade or occlusal slant), cephalometric
radiographs (lateral, AP), Panorex and or 3D C scans—most accurate measure o mandible size and de ciency.

rue/False: Bone gra ting is pre erred over distraction osteogenesis or advancements o more than 10 mm
because a more stable result that is less prone to relapse can be attained.
False.

Why is distraction less prone to relapse than bone gra ting in advancements larger than 10 mm?
T ere is a more gradual stretching o the so t tissues, which makes relapse less likely.

What are the principles o mandibular distraction?
Preserve periosteum i possible, place osteotomy and pin sites with adequate bone stock away rom tooth buds,
corticotomy pre erred to osteotomy (to avoid in erior alveolar nerve injury).

What are the surgical approaches to the mandible?
Extraoral approach (modi ed Risdon) or internal approach.

What is the primary advantage o an external distraction device versus an internal distraction device?
External distraction device can be removed in the of ce avoiding a second operative procedure. Better control o
the distraction vector.

What is a common protocol or mandibular distraction a ter device placement?
Begin distraction on th to seventh postoperative day by rotating the screw 0.25 mm our times a day (better
tolerated than 0.5 mm twice a day) or 1 mm o distraction a day. Distraction completed in 3 to 5 weeks, slight
overcorrection use ul to prevent postoperative relapse. Pins and device le t in place 8 to 9 weeks longer or until
radiographic evidence o new bone bridging seen.

When should the distraction device be removed?
Only when new bone has a radiodensity equivalent to the host bone sur aces and a macrostructure resembling
host bone with equivalent cross-sectional area and ormation o cortex and medullary canal.

How is the posterior open bite achieved during distraction maintained?
Bite block or similar orthodontic appliance until maxillary growth and tooth eruption lls the gap.

What are the complications o mandibular distraction?
Injury to the in erior alveolar nerve (direct injury or slow traction when distraction distance is signi cant)—
usually temporary, pin tract in ection, loosening o pins, relapse a ter device removal, injury to tooth buds,
ankylosis o temporal mandibular joint ( MJ), acial nerve paresis, ailure o distraction (not reported).

CHAPTER 64 Distraction Osteogenesis
623

rue/False: Osteomyelitis o the mandible during distraction has not been reported.
rue.

What patient population may benef t most rom the clinical application o distraction osteogenesis o the
mid ace?
Cle t palate patients with maxillary hypoplasia. Maxillary advancement may cause speech problems; distraction
may limit velopharyngeal compromise.

What patient population may benef t rom orbital expansion by distraction in the uture?
Patients with anophthalmos, congenital absence o ocular tissue. It is predicted to result in ewer surgical
procedures, better t o orbital prosthesis, and improved cosmetic outcome.

What are the indications or palatal distraction?
Anatomic or relative maxillary de ciency, nasal stenosis when conchae are compressed against the septum, all types
o class III occlusion, mature cle t patient, anteroposterior maxillary de ciency, selected arch length problems.
REFERENCES
Aronson J. Experimental and clinical experience with distraction osteogenesis. Cleft Palate Craniofac J. 1994;31:473–482.
Cruz MJ, Kerschner JE, Beste DJ, Conley SF. Pierre Robin sequence: secondary respiratory dif culties and intrinsic eeding abnormalities.
Laryngoscope. 1999;109:1632–1636.
Denny AD, alisman R, Hanson PR, Recinos RF. Mandibular distraction osteogenesis in very young patients to correct airway
obstruction. Plast Reconstr Surg. 2001;108(2):302–311.
Gantous A, Phillips JH, Catton P, Holmberg D. Distraction osteogenesis in the irradiated canine mandible. Plast Reconstr Surg.
1994;93:164–168.
Glat PM, McCarthy JG. Distraction o the mandible: experimental studies. In: McCarthy JG, ed. Distraction of the Craniofacial Skeleton.
New York, NY: Springer-Verlag; 1999:67–79.
Gosain AK; Plastic Surgery Educational Foundation DA A Committee. Distraction osteogenesis o the cranio acial skeleton. Plast
Reconstr Surg. 2001;107:278–280.
Haas AJ. Long-term post treatment evaluation o rapid palatal expansion. Angle Orthod. 1980;50:189–217.
Ilizarov GA. T e tension-stress e ect on the genesis and growth o tissues. Part I. T e in uence o stability o xation and so t-tissue
preservation. Clin Orthop Relat Res. 1989;(238):249–281.
Ilizarov GA. T e tension-stress e ect on the genesis and growth o tissues. Part II. T e in uence o rate and requency o distraction.
Clin Orthop Relat Res. 1989;(239):263–285.
Karp IS, McCarthy JG, Schreiber JS, Sissons HA, T rone CH. Membranous bone lengthening a serial histological study. Ann Plast
Surg. 1992;29:2–7.
McCarthy JG, Schrieber J, Karp N, T rone CH, Grayson BH. Lengthening the human mandible by gradual distraction. Plast Reconstr
Surg. 1992;89:1–8.
McCarthy JG, Stelnicki EJ, Mehrara BJ, Longaker M . Distraction osteogenesis o the cranio acial skeleton. Plast Reconstr Surg.
2001;107:1812–1827.
McCarthy JG. Principles o cranio acial distraction. In: T orne CH, Beasley RW, Aston SJ, Bartlett SP, Gurtner GC, Spear SL, eds.
Grabb and Smith’s Plastic Surgery. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007:96–102.
Personal notes rom Presymposia on Pierre Robin Sequence. ACPA. 2009.
Singhal VK, Losken HW, Patterson G. Cranio acial distraction. In: Benz ML, ed. Pediatric Plastic Surgery. 1st ed. Stam ord, C :
Appleton & Lange; 1998:341–357.

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625
Steven R. Sewall, DDS and
Kyle Smith, DDS

What is orthognathic surgery?
Orthognathic surgery is the surgical correction o abnormalities o the mandible, maxilla, or both. T e underlying
abnormality may be present at birth or may become evident as the patient grows and develops or may be the result
o traumatic injuries. T e severity o these de ormities precludes adequate treatment through dental treatment
alone.

What are the main osteotomies per ormed in orthognathic surgery?
1. Maxilla:
a. Le Fort I osteotomy
i. One-piece
ii. Segmented
2. Mandible:
a. Sagittal split ramus osteotomy
b. ransoral vertical ramus osteotomy
c. ranscutaneous vertical ramus osteotomy

What are the acial categorizations?
Mesocephalic: equal vertical acial thirds
Brachycephalic: broader, shorter, square ace
Dolichocephalic: ovoid and narrow ace with an increased lower third
Orthognathic Surgery
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What are the di erent classi cations o skeletal relationships?
See Figure 65-1.
A

B

C
Figure 65-1 A: Class I orthognathic. B: Class II retrognathic. C: Class III prognathic. (Reproduced with permission rom Ferraro JW.
Fundamentals o Maxillo acial Surgery. New York, NY: Springer-Verlag Inc.; 1997.)

How do you per orm a dento acial analysis?
A dento acial analysis is a clinical examination o so t and hard tissue o the dento acial complex assessing vertical
and transverse proportions in a thorough, systematic, and consistent approach. Direct measurement o the
patient’s resting and dynamic relationships is per ormed.
See Figure 65-2.
1/5
1/5
1/5
1/5
1/5
1/3
1/3
1/3
1/3
2/3
A

B
Figure 65-2 A: Reproduced with permission rom Booth PW, Schendel SA, Hausamen JE. Maxillo acial Surgery. 2nd ed. Vol. 2. St Louis, MO:
Churchill Livingstone; 2007. B: Reproduced with permission rom Ferraro JW. Fundamentals o Maxillo acial Surgery. New York, NY: Springer-Verlag
Inc.; 1997.

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627

What records are needed or treatment planning or orthognathic surgery?
1. Photographs:
a. Frontal view at rest
b. Frontal view with smile
c. Pro le view at rest
d. Dental occlusal relationship
2. Plaster study models mounted on an articulator utilizing a ace-bow trans er
3. Centric relation record
4. Cephalometric prediction
5. Radiographs:
a. Cephalometric radiographs
i. Lateral
ii. Posteroanterior
b. Panorex

What are the most pertinent points/angles that should be traced on a lateral cephalometric radiograph?
1. Sella (S): Center o the pituitary ossa o the sphenoid bone.
2. Nasion (N): Intersection o the internasal suture with the naso rontal suture in the midsagittal plane.
3. Glabella (G): Most anterior portion o the rontal bone.
4. Orbitale (Or): Lowest point o the roo o orbit; most in erior point o the external border o the orbital
cavity.
5. Subnasale (Sn): Point where the nose connects to the center o upper lip.
6. Anterior nasal spine (ANS): T e tip o the ANS.
7. A-point: Deepest point o the curve o the maxilla between the ANS and the dental alveolus.
8. B-point: Most posterior point in the concavity along the anterior border o the symphysis.
9. Stomion superius: Most in erior point on the curve o the upper lip.
10. Stomion in erius: Most superior point on the curve o the lower lip.
11. Pogonion (Pg): Most anterior point on the midsagittal symphysis.
12. Gnathion (Gn): Midpoint between the most anterior and the in erior point on the bony chin.
13. Menton (Me): Most in erior point o the symphysis.
14. Porion (Po): Highest point o the ear canal; most superior point o the external auditory meatus.
15. Condylion (Co): Most posterior superior aspect o the condyle.
16. Gonion (Go): Most convex point along the in erior border o the ramus.
17. SNA:
a. Angle o sella, nasion, and A-point.
b. Relationship o maxilla to the cranial base—mean is 82 ± 2 degrees.
18. SNB:
a. Angle o sella, nasion, and B-point.
b. Relationship o the mandible to the cranial base—mean is 80 ± 2 degrees.

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19. ANB:
a. Angle o A-point, nasion, to B-point.
b. Relative position o maxilla/mandible to each other—mean is 2 ± 2 degrees.
c. N-ANS (mm) and ANS-Me (mm): Hard tissue proportion o the middle acial height to the lower acial
height—mean ratio is 7:8.
20. MP-FH: Divergence o the mandible rom the horizontal plane—mean is 25 degrees.
21. G-Sn-Pg′: So t-tissue angle relative to acial orm—mean is 12 ± 4 degrees.
22. G-Sn/Sn-Me: So t-tissue proportion o the middle acial height to the lower acial height—mean ratio is 1:1
(Fig. 65-3).
Frankfort-horizontal
Nasion
1/3
1/3
Nasofrontal
angle
130–134°
10–17°
1/3
1/3
2/3
Profile line
A
B
Figure 65-3 A: Lateral cephalometric radiograph. B: Pro le o ace demonstrating vertical and sagittal relationships. (B: Reproduced with
permission rom Ferraro JW. Fundamentals o Maxillo acial Surgery. New York, NY: Springer-Verlag Inc.; 1997.)

CHAPTER 65 Orthognathic Surgery
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What does a cephalometric prediction accomplish in treatment planning or orthognathic surgery?
A cephalometric prediction anticipates hard and so t-tissue changes o the planned surgery that cannot be
visualized on the plaster model surgery. T e purpose is to develop an accurate surgical treatment plan that will
achieve the best unctional and aesthetic results.

What are the key eatures and surgical correction o short ace syndrome?
T e short ace syndrome is characterized by a reduced acial height secondary to vertical maxillary def ciency.
Examination notes a square-shaped ace, a low mandibular plane angle, little or no show o the maxillary, anterior
teeth with lips reposed, a deep labiomental old, and an acute nasolabial angle. Dental analysis varies rom an
Angle Class I to an Angle Class II malocclusion. Surgical correction o the problem is with a Le Fort I osteotomy
with bone gra ting to position the maxilla in eriorly.

What are the key eatures and surgical correction or long ace syndrome?
T e long ace syndrome is characterized by vertical maxillary excess resulting in a narrow nose, narrow alar bases,
a prominent nasal dorsum, and depressed paranasal areas. Analysis o the lower third o the ace usually reveals
excessive exposure o the maxillary anterior teeth and gingiva on smiling, lip incompetence, long lower third
acial height, a retropositioned chin, and a normal-to-obtuse nasolabial angle. Examination o the dentition
notes a high, constricted palatal vault with a wide space between the root apices and the nasal oor, as well as a
steep mandibular plane. An Angle Class II malocclusion is a requent nding with or without the presence o an
anterior open bite. Surgical correction o the long ace syndrome decreases the anterior acial height with a Le Fort
I maxillary impaction, autorotation o the mandible, and sagittal osteotomies o the mandible i necessary.

What are the key characteristics and surgical correction o mandibular excess without mid ace de ciency?
Mandibular excess is characterized by a prominent lower third o the ace. Analysis o the data notes normal so t
and hard tissue position o the mid ace; maxillary anterior teeth that are protrusive to the alveolus; the mandibular
anterior teeth are upright to lingually inclined relative to the alveolus; bilateral, posterior crossbites are usually
present; an Angle Class III molar and cuspid malocclusion, and cephalometric measurements greater than the
mean or anteroposterior position o the mandible. Surgical correction is aimed at reducing the mandible and
dentition with either transoral vertical or sagittal ramus osteotomies or transcutaneous vertical ramus osteotomies.

What are key characteristics and surgical correction o anteroposterior maxillary de ciency without
mandibular excess?
Anteroposterior maxillary de ciency unrelated to one o the anomalous syndromes is characterized by a retrusive
upper lip; atness o the paranasal areas, a narrow nose, an obtuse nasolabial angle, nasal “prominence” in the
anteroposterior plane, normal hard and so t-tissue position o the mandible, anterior and posterior crossbites,
maxillary anterior teeth are protrusive to the alveolus, mandibular anterior teeth are upright to the alveolus,
an Angle Class III malocclusion, and cephalometric data less than the mean or anteroposterior position o the
maxilla. Surgical correction is with a Le Fort I maxillary advancement osteotomy.

What are the key characteristics and surgical correction o mandibular de ciency?
T e so t-tissue characteristics are variable depending on the severity o the de ciency; however, a retruded position
o the chin in pro le view is a constant eature. Dental relationships are also variable depending on the severity o
the de ciency; however, the Angle Class II malocclusion is a constant characteristic. Unusual inclinations o the
cranial base are common or patients with mandibular de ciency and there ore the standard cranial base planes
may not be satis actory re erences. For patients with suspected mandibular de ciency, it is important to take
cephalometric lms in a natural head position and utilize the patient’s physiologic horizontal re erence, the visual
axis, as a re erence plane. Surgical correction is with transoral sagittal ramus osteotomies to advance the mandible.

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What are the key characteristics and surgical correction o an open bite de ormity?
Skeletal characteristics include long lower third o the ace, narrow V-shaped maxilla, excessive exposure o
maxillary anterior teeth show at rest, normal or short mandibular ramus, tipped palatal plane which is higher in
ront than in back, steep mandibular plane angle with the mandible rotated down and backward causing increased
acial height.
Surgical correction is Le Fort I maxillary osteotomy with superior repositioning, when the extrusion is
primarily a maxillary problem, possibly combined with mandibular advancement i there is an anteroposterior
de ciency.

What is the most important clinical measurement when treatment planning or vertical maxillary changes?
T e amount o maxillary incisor exposure with the lips in a relaxed position is the most important clinical
measurement. Approximately 2 to 4 mm o incisor exposure is normal.

What are the osteotomies needed to complete surgically assisted rapid palatal expansion?
Horizontal osteotomy rom pyri orm rim to pterygomaxillary ssure, midline palatal osteotomy rom ANS to
posterior nasal spine then extending interdentally between the maxillary incisors, pterygoid, and nasal septal
separation.

A 17-year-old boy s/p cle t lip and palate repair presents to your of ce or management o malocclusion.
Evaluation notes a signi cant de ciency o SNA, a normal SNB, and a negative 15-mm overjet. What
surgical procedure is the most appropriate or the correction?
1. A Le Fort I advancement by distraction osteogenesis.
2. Expanding the so t-tissue envelope is o ten the rate-limiting step in large maxillary advancements. Distraction
osteogenesis should be considered or patients, with repaired cle t lip and palate, who require greater than
10 mm advancement.

What are the phases o distraction osteogenesis in the cranio acial region?
1. Latency period: 3 to 7 days depending on the surgical site.
2. Activation:
• Distraction rate: 1.0 mm per day (0.5–2.0 mm)
• Distraction rhythm: Continuous orce application is best, but device activation two times a day allows or
better patient compliance.
3. Consolidation: Until cortical outline can be seen radiographically across the distraction gap, usually
6 weeks.

CHAPTER 65 Orthognathic Surgery
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A 22-year-old woman undergoes a down racture o the maxilla during Le Fort I osteotomy. Pro use bleeding
is noted in the posterior aspect o the lateral nasal wall. Which artery is most likely involved?
Descending palatine artery, which is a branch o the maxillary artery, descends vertically within the perpendicular
laminae o the palatine bone along the in erior lateral nasal wall.

What are the potential complications when per orming mandibular osteotomies?
1. Ramus ractures o the proximal and distal segments o a sagittal osteotomy.
2. Nerve injuries:
a. In erior alveolar and lingual nerves with transoral osteotomies.
b. In erior alveolar and acial nerves with transcutaneous osteotomies.
3. emporomandibular joint dys unction.
4. In ection.
5. Necrosis o the proximal segment with a vertical ramus osteotomy.
6. Relapse secondary to malposition o the proximal segment.
Nasopalatine
artery
Descending
palatine artery
Lesser palatine
artery
Greater palatine
artery
Maxillary artery
Ascending
pharyngeal artery
Ascending
palatine artery
Facial artery
External carotid
artery
Figure 65-4 Reproduced with permission rom Miloro M, Ghali GE, Larsen PE, Waite PD, et al. Peterson’s Principles o Oral and
Maxillo acial Surgery. 2nd ed. Vol. 2. Hamilton: BC Decker Inc.; 2004.

What is the blood supply o the maxilla ollowing osteotomy?
Ascending pharyngeal artery and the ascending palatine branch o the acial artery, and the descending palatine
branch o the maxillary artery (Fig. 65-4).

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Plastic & Reconstructive Surgery Board Review

What are the complications o genioplasty?
1. Dehiscence o the incision
2. Mental nerve paresthesia
3. Ptosis o mentalis muscle
4. Damage to root apices
5. In ection

At what age is it appropriate to per orm orthognathic surgery?
Once skeletal maturity is reached. In emales, this occurs at ages 14 to 16 years old. In males, this occurs at ages
16 to 18 years old. T is can be veri ed with serial lateral cephalometric and wrist radiographs. T e patient during
this time will have undergone presurgical orthodontics, which includes alignment and positioning o the dentition
over basal bone and decompensation o the dentition.

What are the key eatures and surgical correction or a cle t lip and palate?
Cle ts are characterized by maxillary de ciency in the transverse, anteroposterior, and vertical dimensions.
Examination reveals anterior and posterior dental crossbites, an Angle Class III malocclusion, mid ace sagittal
de ciency, and vertical overclosure. T e incidence o maxillary hypoplasia has been reported to be approximately
21%. T is is an iatrogenic cause as a result rom scar tissue ormation a ter primary lip repair, ollowed by the
palatoplasty. In cle t patients without repair, the skeletal and dental growth patterns are relatively normal.
Surgical correction is a Le Fort I maxillary advancement osteotomy.

What is the surgical approach to the cle t maxilla or a Le Fort I osteotomy?
In unilateral cle t palates and those without severe scarring, a traditional circumvestibular incision, at least 5 mm
apical to the mucogingival junction and extending to the zygomaticomaxillary buttresses, can be per ormed.
For a bilateral cle t palate, severe palatal scarring, or a previous island palatal repair, an anterior buccal pedicle
with a small vertical incision to allow or tunneling, should be le t intact to allow or adequate per usion o the
premaxilla.
When per orming the osteotomy and mobilizing the maxilla, the greatest point o resistance is usually in the
vertical portion o the palatine bone near the descending palatine vessels.

An 18-year-old emale with mandibular hypoplasia undergoes a bilateral sagittal split osteotomy. An
elevator is inserted along the in erior border o the mandible to elevate the masseteric sling, when all o a
sudden, pro use bleeding occurs. Which artery is most likely involved?
T e acial artery, which courses along the submandibular gland and passes lateral and superior to mandible at the
antegonial notch.
Other vessels that can be injured during a mandibular osteotomy are the masseteric, in erior alveolar, medial
pterygoid, and retromandibular. Most injuries are due to indirect trauma rom a retractor placement, an
osteotome, or bur.

A 17-year-old male undergoes a bilateral sagittal split osteotomy. Upon separation o the proximal and distal
segments, a racture occurred in the distal segment posterior to the last erupted tooth. What is the next step?
T e split must be completed through the planned osteotomy. T e ree segment can then be xated with bicortical
screws to the proximal segment, which is then xated to the distal segment, usually with a plate.
Un avorable mandibular ractures can occur 1.9% to 3.9% during bilateral sagittal split osteotomies. T ere is a
slightly higher incidence when third molars are present, and it is recommended that third molars be removed at
least 9 to 12 months prior to the osteotomy.

CHAPTER 65 Orthognathic Surgery
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What are nasal complications a ter a Le Fort I osteotomy?
a. Septal deviation
b. Inadequate turbinate reduction
c. Alar base widening
d. Nasal tip over-rotation
e. Dorsal de ormities

A 20-year-old male with vertical maxillary excess undergoes a Le Fort I maxillary impaction. A ter xating
the maxilla, and immediately ollowing the release o maxillomandibular xation, the patient developed an
anterior open bite. What is the cause?
T ere was inadequate reduction o the posterior maxilla, creating inter erences that displaced the condyles
rom the ossa during xation o the maxilla. Upon release o the maxillomandibular xation, the condyles are
repositioned correctly into the ossa, which causes the apertognathia.

A 19-year-old emale with mandibular hyperplasia, a Class III Angle molar and cuspid malocclusion, and
bilateral TMJ arthralgia and anterior disc displacement. What is an appropriate surgical procedure?
An intraoral vertical ramus osteotomy will allow or a mandibular setback to correct the malocclusion, and results
in an anterior and in erior displacement o the condyle. T is increases the joint space and can promote disc
reduction.

A 16-year-old emale with the diagnosis o bilateral idiopathic condylar resorption presents to the of ce with
severe retrognathia, a high mandibular plan angle, loss o chin projection, and an anterior open bite. What
is an appropriate surgical procedure or correction o this patient’s de ormity?
Bilateral total temporomandibular joint arthroplasties and a Le Fort I osteotomy with a counterclockwise rotation
o the maxillomandibular complex. Idiopathic condylar resorption, rheumatoid arthritis, trauma, and condylar
hypoplasia which can present as a shortened ramus height and steep mandibular plane angle. T ese can be
corrected with an alloplastic MJ replacement in conjunction with a Le Fort I osteotomy.
REFERENCES
Bell WH, Prof t WR, White RP. Surgical Correction o Dento acial De ormities. Vols. 1 & 2. Philadelphia, PA: WB Saunders; 1980.
Booth PW, Schendel SA, Hausamen JE. Maxillo acial Surgery. 2nd ed. Vol. 2. St Louis, MO: Churchill Livingstone; 2007.
Ferraro JW. Fundamentals o Maxillo acial Surgery. New York, NY: Springer-Verlag; 1997.
Fonseca RJ, Marciani R, urvey , et al. Oral and Maxillo acial Surgery. Vol. 3. 2nd ed. St Louis, MO: WB Saunders; 2009.
James JN, Costello BJ, Ruiz RL. Management o cle t lip and palate and cle t orthognathic considerations. Oral and Maxillo acial
Surgery Clinics. 2014;26:4.
Miloro M, Ghali GE, Larsen PE, Waite, PD, et al. Peterson’s Principles o Oral and Maxillo acial Surgery. Vol. 2. 2nd ed. Hamilton: BC
Decker; 2004.
Nale JC. Orthognathic surgery and the temporomandibular joint patient. Oral and Maxillo acial Surgery Clinics. 2014;26:4.
Piecuch JF. OMS Knowledge Update Sel -Study Program. Vol. 2. Rosemont: American Association o Oral and Maxillo acial Surgeons;
1998.
Prof t WR, urvey A, Phillips C. T e hierarchy o stability and predictability in orthognathic surgery with rigid xation: an update
and extension. Head Face Med. 2007;3:21.
Robl M , Farrell BB, ucker MR. Complication in orthognathic surgery, a report o 1000 cases. Oral and Maxillo acial Surgery Clinics.
2014;26:4.

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635
Jonathan Bank, MD,
Donald B. Johnson, MD, and
Bruce S. Bauer, MD, FACS, FAAP

What structures develop rom the hillocks o the rst branchial arch?
T e tragus, the helical crus, and helical root (lymphatic drainage to parotid nodes).

What structures develop rom the three hillocks o the second branchial arch?
T e helix, scapha, concha, antihelix, antitragus, and lobule (lymphatic drainage to posterior auricular nodes).
T e most common auricular sinus lies at the junction between the helical root and the superior helix (rises at the
boundary between the rst and second arch contributions).

What embryologic structure gives rise to the external auditory meatus?
T e rst branchial groove (lymphatic drainage to both parotid and posterior auricular nodes).

What is the normal size o the ear?
5.5 to 6.5 cm or the normal adult ear height and 3 to 4.5 cm or the normal adult ear width. T e width varies
rom 66% o the height in children to 55% o the height in adults. For children 4 years o age, a subnormal ear
height (i.e., < 2 SD) is below 4.5 cm.

Describe normal ear growth.
85% o ear development occurs by 3 years o age with ull development achieved between 6 and 15 years o age.

What is the normal position o the ear?
1. One ear length posterior to the lateral orbital rim.
2. Mean inclination rom vertical o 20-degree posteriorly.

What is the normal scalp-to-helix distance or each third o the ear? (Upper, middle, and lobule)
1. Upper: 1.0 to 1.2 cm
2. Middle: 1.6 to 1.8 cm
3. Lobule: 2.0 to 2.2 cm
Congenital Ear
Deformities
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How is the concha subdivided?
T e concha cavum and concha cymba, which are separated by the helical root (also re erred to as the root o the
helical crus).

T e superior crus o the antihelix is bordered by what structures?
T e scapha and the triangular ossa.

T e in erior crus o the antihelix is bordered by what structures?
T e triangular ossa and the concha cymba.

What is the triangular ossa?
T e concave area between the superior crus and the in erior crus.

Describe the arterial blood supply to the ear.
Super cial temporal artery supplies the anterior sur ace. Posterior auricular artery supplies the posterior sur ace o
the ear, lobule, and retroauricular skin. T ere are numerous per orating vessels interconnecting the two territories.

Describe the venous drainage o the ear.
Anterior ear is drained by the super cial temporal and retromandibular veins. Posterior ear is drained by posterior
auricular veins draining into the external jugular vein.

Describe the innervation o the ear (Fig. 66-1).
Sensory supply o the anterior sur ace o the ear is via the auriculotemporal (V3) and great auricular (CN II–III)
nerves. T e posterior sur ace is innervated by the great auricular nerve, and the mastoid branches o the lesser
occipital nerve. T e region o the meatus (and medial concha) is supplied by the nerve o Arnold rom the Vagus
nerve (oropharyngeal cancer may present with re erred pain to the ear via vagal nerve bers common to the
oropharynx and concha).
Figure 66-1 Innervation o the ear.
*Illustrations by Matthew Gould.

CHAPTER 66 Congenital Ear De ormities
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Microtia is seen in what syndromes (Fig. 66-2)?
1. Hemi acial microsomia ( rst and second branchial arch syndrome).
2. Goldenhar syndrome (oculoauriculovertebral dysplasia; characteristically has bilateral ear de ormities).
3. reacher Collins syndrome (bilateral de ormities).
Figure 66-2 Microtia.

What is the proposed pathogenesis o microtia?
Obliteration o the stapedial artery. T e bilateral de ormity in reacher Collins syndrome has been demonstrated
to arise rom a di erent event occurring early in gastrulation (Poswillo) and has also been associated with
thalidomide.

For the microtia patient, at what age is it usually recommended or the child to undergo total ear
reconstruction?
raditional recommendations advocated reconstruction by age 6 or 7 be ore starting school. However, optimal
reconstruction requires costal cartilage o su cient size and shape to carve the key ramework details and maintain
the strength to display these details through the overlying skin envelope. Better results can be obtained at age 9 to
10 years or later, with a lower rate o revision. One must balance potential psychological issues o dealing with the
de ormity versus the bene ts o delaying surgery.

How many stages are usually required or total ear reconstruction?
wo or three, depending on the particular technique.

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What is usually involved in the rst stage?
Harvest o a costal cartilage gra t and creation o a cartilage ramework, elevation o a f ap or coverage o the
ramework and insertion o the ramework in the pocket. T e majority o surgeons today rotate the lobule in the
rst stage and design the ramework to include the tragus.

What is usually involved in the second and third stages?
Elevation o the reconstructed ear, placement o a banked cartilage gra t or additional ramework elevation, and
coverage with ascia f ap and skin gra t. In cases where lobule was not rotated in the rst stage and the tragus not
reconstructed, these procedures are per ormed in the second and third stages along with elevation o the ear.

Which ribs are usually harvested or the cartilage ramework?
Portions o costal cartilages 6 through 9, ipsilateral or contralateral (surgeon pre erence).

What morbidities are associated with the harvest o costal cartilage?
Harvesting costal cartilage may lead to chest wall de ormity, scarring, and substantial pain; o these, distortion o
the chest wall is the greatest concern. Delaying reconstruction until 10 years o age, preserving the perichondrial
margin during harvest, and reconstituting the donor-site de ect with cartilage ragments may signi cantly reduce
the potential or de ormity.

What regional ap can be utilized when the standard skin aps are unsuitable or ear reconstruction?
T e most readily available is the temporoparietal ascia f ap based on the super cial temporal artery with split
thickness skin gra t.

What are the advantages/disadvantages o using polyethylene implants (Medpor®) in ear reconstruction?
Porous polyethylene implants o er excellent de nition and contralateral ear matching, reconstruction at a younger
age, a shorter duration o treatment (typically 4 months), and are not associated with autologous reconstruction
chest morbidities. Conversely, allopathic materials have a higher rate o in ection and extrusion, and implant
exposure commonly results in loss o the temporoparietal ascia used or implant coverage.

In microtia patients, what must be present or canal and middle ear reconstruction?
Jahrsdoer er developed a scoring system to predict outcomes o middle ear reconstruction. T e two absolute
criteria or a patient to quali y or surgery are good cochlear unction and no imaging evidence o a mal ormed
inner ear; surgery is de erred i the middle ear and mastoid have ailed to aerate. Patients should understand
that tympanoplasty/canaloplasty may not improve hearing substantially and complications place the auricular
construct at risk.

When should bone-anchored hearing aids (BAHA) be placed in patients who are undergoing ear
reconstruction?
It has been recommended that BAHAs should be placed only a ter ear reconstruction has been completed to avoid
compromising the mastoid-region skin used to cover the autologous ramework. However, coordinated treatment
by otolaryngologist and plastic surgeons, and the development o transcutaneous bone conduction implants
(tBCI) make placement possible at any stage during reconstruction—synchronized care also reduces the number
o scars.

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What are the characteristics o a prominent ear de ormity (Fig. 66-3)?
Prominent ear arises rom two main actors: hypertrophy o the concha—recognized as the most signi cant actor,
and e acement o the antihelical old. Prominence is urther inf uenced by the skull base upon which the ear sits
(as most easily demonstrated by the di erent ear position and prominence in positional plagiocephaly).
Figure 66-3 Prominent ear de ormity.

What is the role o ear taping and splinting in the management o the prominent ear de ormity?
Up to 6 months o age, molding the ear with taping and splinting may improve ear appearance and prevent the
need or surgical correction. However, the younger the patient, the more e ective the treatment and the shorter
the time required or correction.

Why is this limited to the rst 3 to 6 months o li e?
Circulating maternal estrogen increases hyaluronic acid in cartilage rendering it more de ormable. T is probably
acilitates passage through the birth canal during delivery but can be exploited to reshape de ormities o the ear.
While maternal estrogen levels drop within hours o partum, the e ect on the cartilage remains longer. Beyond the
neonatal period the change rom so t to rmer cartilage is less dramatic and splinting will be required or a longer
period. ypically beyond 3 months most in ants will not tolerate the necessary 24-hour splinting.

What are the primary maneuvers used to better de ne the ef aced antihelical old and what are the associated
complications?
Procedures can be divided into those that score or thin the antihelix (anterior or posterior sur ace) and those that
accentuate it with sutures. Scoring or abrading the cartilage (particularly the anterior sur ace) can be associated
with sharp and irregular olding. Sutures alone can be associated with suture disruption and loss o correction.

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What maneuvers can af ect conchal hypertrophy and what are the associated complications?
T e maneuvers or conchal reduction vary rom cartilage excision to setback o the conchal bowl. Some authors
will also reduce conchal height by lowering the position at which they create the antihelical old. Conchal setback
can be associated with loss o xation sutures. As in the previous question, e orts to overaccentuate the antihelix
to a ect the height o the concha can be associated with irregular olds.

What is the ef ect o conchomastoid sutures?
Reduction o conchal projection. Conchal reduction can also be achieved by direct excision through an anterior or
a posterior incision.

What is the ef ect o Mustardè sutures?
Permanent conchoscaphal sutures are placed to recreate the antihelical old.

Conchomastoid sutures are more commonly associated with what complication?
Meatus distortion.

What is the most common complication ollowing otoplasty surgery?
Recurrent de ormity with 10% to 25% undergoing revision otoplasty.

What are common un avorable results o otoplasty?
T ese can be divided into undercorrection and overcorrection. Undercorrection is most o ten secondary to
either an insu cient number or inaccurate placement o permanent sutures, or loss o support by resorption o
absorbable sutures. Nontechnical causes include unrecognizing underlying skeletal asymmetry, or ailure to treat
conchal hypertrophy. Overcorrection, mainly in the orm o the telephone ear deformity results rom relative over-
resection o skin o the middle third o the ear, resulting in an apparent prominence o the upper and lower thirds.
It is accentuated when lobular hypertrophy is not recognized and incompletely treated.

A post otoplasty “pinned back” appearance is attributed to what technical shortcomings?
A pinned back appearance is due to inadequate resection o the hypertrophic conchal bowl and over accentuation
o the antihelix, resulting in lost visibility o the helical rim.

What is cryptotia and how is it treated?
“Hidden ear” de ormity is characterized by the absence o the superior auriculocephalic sulcus and results rom
overaccentuation o the superior or in erior crus, tipping the upper pole o the ear beneath the adjacent scalp.
Correction requires release o abnormal cartilage adhesions on both posterior and anterior sur aces o the ear and
coverage o the de ect with both auricular and postauricular f aps.

What are the eatures o the constricted ear ( ormerly termed both lop ear and cup ear)?
T e our undamental aspects o the constricted ear include lidding, protrusion, decreased ear size, and low ear
position. T e majority o constricted ears have decreased radius o curve o the helical rim and some degree o skin
de ciency.

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What is Stahl’s ear?
Stahl’s ear, commonly re erred to as “Spock ear,” is characterized by either a third crus or abnormal angulation o
the superior crus, projecting the helical rim upward or outward. While di cult to correct surgically, this de ormity
is perhaps the easiest o all ear de ormities to correct with neonatal splinting.
REFERENCES
Bauer BS. Congenital and acquired de ormities o the ear. In: Bentz ML, Bauer BS, Zuker RM, eds. Principles and Practice of Pediatric
Plastic Surgery. St Louis, MO: Quality Medical Publishing; 2008:801–850.
Bauer BS. Reconstruction o microtia. Plast Reconst Surg. 2009;124(Suppl 1):14E–26E.
Brent B. Microtia repair with rib cartilage gra ts: a review o personal experience with 1000 cases. Clin Plast Surg. 2002;29:257–271.
Brent B. T e acquired auricular de ormity. A systematic approach to its analysis and reconstruction. Plast Reconstr Surg. 1977;59:
475–485.
Constantine KK, Gilmore J, Lee K, Leach J Jr. Comparison o microtia reconstruction outcomes using rib cartilage vs porous polyethylene
implant. JAMA Facial Plast Surg. 2014;16:240–244.
Eriksson E, Vogt PM. Ear reconstruction. Clin Plast Surg. 1992;19:637–643.
Lentz AK, Plikaitis CM, Bauer BS. Understanding the un avorable result a ter otoplasty: an integrated approach to correction. Plast
Reconstr Surg. 2011;128:536–544.
Poswillo D. T e pathogenesis o the rst and second branchial arch syndrome. Oral Surg Oral Med Oral Pathol. 1973;35(3):302–328.
Weerda H. Surgery of the Auricle: Tumors, Trauma, Defects, Abnormalities. New York, NY: T ieme; 2007.

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Stephen Colbert, MD, FACS
CONGENITAL NEVI

De ne “congenital.”
Existing at birth.

Does “congenital” imply “hereditary”?
No.

What is a nevus?
A chronic benign lesion o the skin or mucosa, commonly known as “mole” or “birthmark”. Nevi may be either
present at birth (congenital) or appear a ter birth (acquired).

What is a giant nevus?
Predicted to be greater than 20 cm in largest diameter in adulthood, correlating in an in ant to 6 cm on the head
and 9 cm on the body; greater than 1% body sur ace area on the head and neck, or 2% on the rest o the body; or,
o a size that cannot be excised in a single procedure.

What is the incidence o congenital nevi?
1% to 2%.

What is the incidence o giant nevi?
For lesions greater than or equal to 9.9 cm, approximately 1 in 20,000.

Nevi results rom a proli eration o what cells?
Melanocytes, o neuroectodermal origin.
Congenital Nevi/Tissue
Expansion
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In what layer(s) o the skin do nevi occur?
Dermis and epidermis.

For what cancer are nevi generally considered a risk actor?
Malignant melanoma.

What is the risk o melanoma in one who has a giant congenital nevus?
5% to 7% by the age o 60 is the most o ten reported range; however, reported incidence in the literature ranges
rom 0% to 42%.

What is an acquired nevus?
T e more common “mole,” the occurrence o which begins in childhood and increases rapidly in adolescence and
young adulthood.

How are nevi classi ed by location and histologic pattern?
Compound, intradermal, and junctional.

What is a compound nevus?
A nevus with ully ormed nests o cells in the epidermis and newly orming cells in the dermis.

What is an intradermal nevus?
A nevus with nests o cells located exclusively within the dermis; clinically indistinguishable rom a compound
nevus.

What is a junctional nevus?
A nevus with nests o cells con ned to the dermoepidermal junction; usually presenting as a small, discrete, at, or
slightly raised macule.

How are small congenital nevi managed medically?
Baseline photography and regular ollow-up.

What eatures o small nevi warrant biopsy?
T ose eatures suggestive o melanoma: asymmetry, border irregularity, color variegation, and diameter larger than
6 mm; in addition, any nevus that has changed appearance.

At what location(s) are congenital nevi associated with leptomeningeal melanoma?
Congenital nevi o the head, neck, and trunk, particularly over the spine.

What is a nevus o Ota?
Pigmented nevus in the distribution o the ophthalmic (V1) and maxillary (V2) division o the trigeminal nerve;
usually unilateral, may af ect mucosa, associated with development o glaucoma and rare malignant degeneration.

CHAPTER 67 Congenital Nevi/ issue Expansion
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In what demographic segment are nevi o Ota seen?
Most common in emales and Asian populations; occurring in early in ancy, approximately 50% congenital, and
in early adolescence.

What is the recommended treatment o large congenital nevi?
Giant nevi should be removed surgically as soon as possible, as there is risk o melanoma even in the rst 3 to
5 years o li e; nonsurgical or ablative methods are not recommended; surgical excision is staged or involves
tissue expansion.

How are small- and medium-sized congenital nevi treated?
reatment remains controversial with recommendations ranging rom early excision o all nevi to li etime
observation with removal only or speci c changes.
TISSUE EXPANSION

What is the main advantage o using tissue expansion in reconstruction?
Replacing missing or pathologic tissue with normal-appearing tissue.

What are other advantages?
Expanded tissue is typically sensate and well vascularized, and nal donor de ects are minimized.

In what type o situations should tissue expanders be avoided?
Immediate traumatic or in ected elds, open wounds, proximity to malignant tumor, nonexpandable or high-risk
tissue such as a skin gra t or irradiated tissue.

What are the common disadvantages o tissue expansion?
Requires two operations, creates an aesthetic de ormity during expansion, and may cause transient discom ort
during expansion.

What is “creep”?
Viscoelastic de ormation occurring immediately with expansion.

Besides creep, what actors increase dimensions o expanded tissue in the short term?
Loss o interstitial uid by pressure, and recruitment o adjacent mobile tissues.

Does stretch o tissue have any e ect on cellular proli eration?
Yes, tension has been shown to induce DNA synthesis and cellular mitosis.

Does expansion a ect blood f ow in expanding tissue?
Yes, blood ow increases in expanding tissue; in addition, the placement o an expander unctions as a delay or
the overlying ap.

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What happens to the epidermis during expansion?
T e epidermis thickens.

What happens to the dermis during expansion?
T e dermis thins.

Are these changes in the epidermis and dermis permanent?
No, such changes have been shown to undergo reversal ollowing expander removal.

What are the risks to at, muscle, and nerves during expansion?
Compression could cause atrophy o all o these tissues; in addition, compression o sensory nerves may lead to
paresthesias or discom ort.

What tissues are particularly amenable to expansion?
T e scalp and breast; in general, the arther rom these tissues, the less success ul expansion becomes.

What may happen i an expander is placed beneath an incision?
Dehiscence and expander exposure.

How should exposure o a tissue expander be treated?
In general, exposure o an expander is best treated by removal, pocket drainage, and expander replacement at a
later date; in selected situations, removal, sterilization, and immediate replacement may be appropriate; in other
selected situations, some may consider completion o expansion in the setting o exposure appropriate.

Does tissue expansion require placement o an expandable implant?
No, techniques such as presuturing, external expanders, and lacing devices allow tissue expansion and ultimate
wound closure without placement o internal expanders.

Is placement o incisions or expander insertion important?
Yes, one o the most critical actors af ecting outcome is the placement o the nal scar, which is usually af ected by
placement o the initial incision; in addition, placement o the incision in tissue that is to be expanded may lead to
a widened scar or even dehiscence and expander exposure.

What technical error committed during expander placement will likely lead to expansion ailure?
Inadequate development o the pocket where the expander will be placed.

What complications are associated with tissue expanders?
Seroma ormation, transient alopecia during scalp expansion, contour de ormities such as dog-ears postoperatively,
widened scars, hematoma, in ection, expander exposure, expander extrusion, and tissue necrosis.

What is the bene t o a textured sur ace on a tissue expander?
Relative immobility due to tissue adherence, maintenance o proper expander orientation, increased capsular
compliance, and reduction in capsular contracture.

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REFERENCES
Congenital Nevi
Bittencourt FV, Marghoob AA, Kop AW, Koenig KL, Bart RS. Large congenital melanocytic nevi and the risk or development o
malignant melanoma and neurocutaneous melanocytosis. Pediatrics. 2000;106:736–741.
Newman WA. Dorland’s Illustrated Medical Dictionary. 28th ed. Philadelphia, PA: WB Saunders; 1994;1864–1956.
Fitzpatrick B, Johnson RA, Wolf K, Suurmond D. Color Atlas and Synopsis of Clinical Dermatology: Common and Serious Diseases.
4th ed. New York, NY: McGraw-Hill; 2001.
Gosain AK, Santoro D, Larson DL, Gingrass RP. Giant congenital nevi: a 20-year experience and an algorithm or their management.
Plast Reconstr Surg. 2001;108(3):622–631.
Marghoob AA. Congenital melanocytic nevi evaluation and management. Dermatol Clin. 2002;20:607–616.
Marghoob AA, Schoenbach SP, Kop AW, Orlow SJ, Nossa R, Bart RS. Large congenital melanocytic nevi and the risk or the
development o malignant melanoma: a prospective study. Arch Dermatol. 1996;132:170–175.
Watt AJ, Kotsis SV, Chung KC. Risk o melanoma arising in large congenital melanocytic nevi: a systematic review. Plast Reconstr Surg.
2004;113(7):1968–1974.
Tissue Expansion
Concannon MJ, Puckett CL. Wound coverage using modi ed tissue expansion. Plast Reconstr Surg. 1998;102(2):377–384.
De Filippo RE, Atala A. Stretch and growth: the molecular and physiologic in uences o tissue expansion. Plast Reconstr Surg.
2002;109(7):2450–2462.
Manders EK. Reconstruction using so t tissue expansion. In: Cohen M, ed. Mastery of Plastic and Reconstructive Surgery. Boston, MA:
Little, Brown and Co; 1994:201–213.
Pasyk K, Argenta L, Austad ED. Histopathology o human expanded tissue. Clin Plast Surg. 1987;14:435–445.

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Samita Goyal, MD

Describe breast development.
During the sixth week o gestation, ectodermal cells orm a primordium along the primitive mammary ridges
(“milk lines” that extend rom the axilla to the groin). By the 10th week o gestation, the upper and lower aspects
o these ridges atrophy; the middle portion o the ridges, located at the level o the ourth interspace, persist and
eventually develop into breast tissue. In the th month o gestation, the areola develops, and shortly a ter birth,
the nipple appears.
A ter birth, residual circulating maternal estrogens may cause neonatal gynecomastia which typically involutes as
these hormones are metabolized. T elarche precedes puberty by approximately 1 year and marks the beginning o
breast growth. At puberty, estrogens cause ductal and stromal tissue proli eration and progesterones cause alveolar
budding and lobular growth. Breast growth is generally completed by 16 to 18 years o age.

Describe chest wall development.
During the ourth week o gestation, ectodermal cells rom the neural crest and somatic cells rom the paraxial and
lateral plate join and di erentiate into orty somites caudal to the head region. T ese urther di erentiate into the
dermomyotome (dorsal) and sclerotome (ventral), which become the trunk musculature and skeleton, respectively.
T e sclerotome also di erentiates into the vertebral bodies. T e sternum develops during the sixth week o
gestation separately rom the ribs, arising rom longitudinal pairings o mesenchymal tissues. Failure o usion leads
to a cle t sternum.

How can congenital breast disorders be generally classi ed?
Hypoplastic (athelia, amazia, amastia, unilateral/bilateral hypoplasia, tuberous breast, and Poland syndrome).
Hyperplastic (gynecomastia, hyperplasia, symmastia, polythelia, polymastia, and giant broadenoma).

What organ system should always be included in the initial preoperative evaluation o patients with
gynecomastia?
Genitalia may reveal an underlying cause such as testicular tumors, nonpalpable and/or undescended testes. T ese
ndings should prompt genetic and/or endocrine evaluation prior to surgery.
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What pharmaceutical agents are linked to the development o gynecomastia?
Cimetidine, digitalis, minocycline, spironolactone, anabolic steroids, haloperidol, opiates, marijuana,
phenothiazines, tricyclic antidepressants, progestins, amphetamines, isoniazid, methyldopa, estrogens, diazepam,
reserpine, and theophylline.

How is gynecomastia classi ed?
T ere are three grades:
Grade I: small enlargement, no skin redundancy.
Grade IIA: moderate enlargement, no skin redundancy.
Grade IIB: moderate enlargement with skin redundancy.
Grade III: marked enlargement with skin redundancy.

What is the modi cation o gynecomastia classi cation or surgical planning?
Simpli y to the three grades:
Grade I: Localized button o tissue localized to areola. Chest not atty, no skin excess. Simple excision.
Grade II: Di use gynecomastia on atty chest, indistinct tissue edges. Suction lipectomy adjunct to excision.
Grade III: Di use gynecomastia with excessive skin, requires skin excision outside areola and/or nipple
repositioning.

What are the options or surgical management o gynecomastia?
Suction-assisted lipectomy or ultrasound-assisted lipectomy only.
Glandular resection through periareolar incision + /− suction-assisted lipectomy.
Skin and glandular resection.
Concentric circle resection.
Pedicled relocation o nipple with skin resection.
Breast amputation with ree nipple gra ting.
Wise-pattern mastopexy should NO be per ormed in gynecomastia.

What is the role o liposuction in gynecomastia?
Suction-assisted lipectomy is primarily used to improve chest contour by lateral eathering as an adjunct to
surgical excision. Although, it can be used as a primary treatment modality in breast tissue that is primarily
atty. Ultrasound-assisted liposuction (UAL) can be used as a primary modality in most patients and is primarily
applicable to dense, brous tissue. It o ers the advantage o minimal external scarring. With the use o UAL, the
use o excisional techniques may be reserved or patients with severe gynecomastia with signi cant skin excess
AF ER attempted UAL.

What is the timing o surgical intervention in patients with gynecomastia?
Surgery should not be o ered to patients with gynecomastia or duration o less than 1 year. I gynecomastia persists
or more than 1 year, the likelihood o spontaneous resolution is low and surgery may be o ered anytime a ter 1 year.
Correctable medical causes such as endocrine imbalance, medications, or tumors preclude surgical intervention.

What is the incidence o gynecomastia in males at puberty?
Approximately 65% to 75%.

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What is the typical time length until spontaneous resolution o gynecomastia in adolescent boys?
16 to 18 months. Best to wait as long as 2 years prior to intervention. I psychosocial disturbance, may intervene
earlier.

What percentage o males with gynecomastia present at puberty have residual gynecomastia at age 17?
8%.

In which medical condition is gynecomastia associated with an increased incidence o breast cancer?
Kline elter syndrome (at least 20 times general population). Breast biopsy is indicated in such cases.

What is the most common complication a ter correction o gynecomastia?
Hematoma.

What is the management o gynecomastia in the neonate?
Requires no therapy, typically resolves in several weeks.

What are the common causes o gynecomastia?
Increase in estrogens, decrease in androgens, or de ciency in androgen receptors.

What are the multiple disease states in which gynecomastia can be seen?
Primary testicular ailure (Kline elter, XXY syndrome), secondary testicular ailure (orchitis, mumps), endocrine
disorders (hyper- or hypothyroidism, adrenocorticohyperplasia), liver disease (most common alcoholic cirrhosis),
tumors (lung cancer, testicular cancer, adrenal tumors, pituitary tumors, colon or prostate cancer), congenital
syndromes, debilitated disease states (e.g., severe burn).

What tests are generally required by insurance companies prior to approval o gynecomastia surgery?
Endocrine workup.

How is juvenile breast hypertrophy classi ed?
emporal classi cation:
Prepubertal hypertrophy: develops be ore puberty, usually bilateral. Virginal hypertrophy: develops a ter puberty,
either unilateral or bilateral.
Classi cations o asymmetry:
Unilateral hyperplasia, bilateral symmetrical hyperplasia, combination o hyperplasia and hypoplasia.

What is the treatment o choice in juvenile breast hypertrophy?
Reduction mammoplasty. High recurrence rates may require urther surgery.

When should surgery be per ormed in juvenile breast hypertrophy?
Surgery should be delayed until a ter puberty, a ter breast growth has ended.

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What is the management o breast enlargement be ore puberty?
Rare condition requires search or chromosomal abnormalities, drug, and endocrine workups.

Describe the course o giant broadenomas.
T ey present during puberty as unilateral, rapidly growing masses. T ese lesions are benign and discrete, and
are thought to occur as a hypersensitivity to physiological circulating levels o gonadal hormones. Diagnosis is
con rmed by breast tissue biopsy. I growth is very rapid, surgery may be indicated prior to the completion o the
growth due to its large discrepancy in size.

What is the incidence o polythelia?
Polythelia is the most common congenital breast anomaly that a icts both boys and girls, with an incidence o 5%.

When should supernumerary nipples be excised?
In young girls, prior to puberty be ore accessory breast tissue can orm.

Where are accessory mammary structures ound most requently in patients with polymastia?
T e axilla ollowed by the in ramammary region.

Which areas are commonly af ected in ectopic polymastia?
Ectopic polymastia: Breast tissue in areas outside the milk line. Reported in midline, ace, ear, neck, back, buttock,
posterior or dorsal thigh, scalp, shoulder, and epigastrium.

What associated organ systems are af ected in polymastia?
Renal and thoracic. T e incidence o polymastia is lower than polythelia.

What are the incidence and most common location or polythelia (supernumerary nipple)?
5% and in ramammary region (commonly misdiagnosed as nevi).

What is the dif erence between amastia, amazia, and athelia?
Amastia: Congenital absence o the breast (nipple–areola complex and glandular tissue).
Amazia: Absence o glandular tissue only.
Athelia: Absence o nipple alone.

What is the primary eature o Poland syndrome?
Unilateral aplasia or hypoplasia o the pectoralis major muscle (absence o sternal head) and breast hypoplasia.

What is the incidence o Poland syndrome?
1 in 20,000 to 30,000. Most cases are sporadic and 75% occur on the right side. Some reports o amilial cases.
Male to emale ratio is 3:1.

What is the suggested primary de ect in Poland syndrome?
Subclavian artery disruption sequence resulting in diminished blood supply to a ected limb yielding partial loss o
tissue in the a ected regions.

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What are the other associated de ormities o Poland syndrome?
Absence o ribs 2 to 4, hypoplasia o the muscles o the shoulder girdle including the latissimus dorsi,
brachysyndactyly, shortening o orearm bones, de ciency o subcutaneous at, absence o axillary hair, aplasia or
hypoplasia o nipple–areola complex.

What study is a use ul adjunct in the preoperative workup o patients with Poland syndrome?
High-resolution C scan to con rm status o latissimus dorsi and de ne associated skeletal anomalies.

What are the three aspects o chest wall reconstruction in Poland syndrome?
Stabilization o chest wall, creation o a so t tissue “ ll,” breast reconstruction.

What is the surgical management o the breast de ormity in male patient with Poland syndrome?
Latissimus dorsi neuromuscular island f ap reconstruction. As early as 5 to 6 years o age. Free latissimus trans er
may be considered i the ipsilateral latissimus is not available. Custom-made silicone implant is another alternative.

What is the surgical management o the breast de ormity in the emale patient with Poland syndrome?
At age 12 to 13, temporary expander or symmetry during breast development, exchange or permanent implant
at the end o breast development. Latissimus dorsi used in cases o inadequate subcutaneous tissue. In adult
patients, pedicled RAM f ap may be used, not used in adolescent patients or those considering childbirth.

What percentage o patients with syndactyly o the hand have Poland syndrome?
10%.

What are the options or skeletal chest wall de ormity reconstruction in patients with Poland syndrome?
No contraindication to single-stage repair. Customized silicone prosthesis placed subcutaneously or beneath
latissimus dorsi muscle f ap or split-rib gra ts; i so t tissue lacking, cover gra ts with prosthetic patch or latissimus
dorsi muscle f ap (pre erred), which serves as base or mammary prosthesis. Some cases may require contralateral
mastopexy. Free tissue trans er may be required.

What are the components o the tuberous breast de ormity?
Breast hypoplasia (de ciency o base diameter), breast tissue herniation into the areola (leading to a large areola
diameter), de ciency o skin envelope, and elevation o the in ramammary old.

What is the classi cation o the tuberous breast de ormity?
ype I—lower medial quadrant hypoplasia. ype II—lower medial and lateral quadrant hypoplasia. ype III—
severe breast constriction.

What is the surgical management o the tuberous breast de ormity?
In most cases, augmentation with a periareolar mastopexy. In severe breast ptosis, augmentation with wise-pattern
mastopexy. In severely de cient skin envelope, two-stage reconstruction with tissue expansion.

What are the causes o unilateral hypoplasia?
Congenital condition or acquired secondary to iatrogenic injury to breast bud.

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What is the surgical management o unilateral hypoplasia?
Delay reconstruction until 13 years i possible. At age 13, a temporary expander is placed or symmetry during
breast development, exchanging this or a permanent implant at the end o development. Submusculo ascial
position is pre erred. Nipple–areola reconstruction at completion o expansion.

What is the other anatomic consideration at the time o implant placement in the hypoplastic breast?
Breast constriction with a shortened and high in ramammary old. Must be addressed at the time o implant
placement.

Are congenital breast cases covered by insurance?
It depends on the individual insurance policies. With appropriate documentation, some cases may be covered by
insurance. Some policies consider all congenital breast cases “cosmetic” and do not cover them.

What is pectus excavatum and how is it treated?
Pectus excavatum is a congenital chest wall de ormity leading to a concave curvature o the chest wall, that is,
a caved-in appearance. It is usually diagnosed within the rst year o li e (90% o cases) and typically worsens
into adolescence. T e most popular surgical intervention was initially described by Ravitch and involves a
submammary or vertical midline approach to elevate the pectoralis major muscles rom the chest wall and
subsequently resect the de ormed cartilage. T e sternum is then elevated a ter cutting it transversely, ollowed by
reapproximation o the pectoralis muscles. T is is secured in place with a temporary metal strut (Adkins strut),
which is removed approximately 6 months later. T e Nuss procedure involves placing a retrosternal bar that
pushes the sternum anteriorly without any resection o sternal bone. Care is taken to avoid injury to intrathoracic
structures during placement, and the bar is le t in place or 2 years.

What is pectus carinatum and how is it treated?
Pectus carinatum is a congenital chest wall de ormity that leads to a convex curvature o the chest wall, that is, a
bowed-out appearance. It occurs 10% as o ten as pectus excavatum. It is treated similarly to the Ravitch approach
or pectus excavatum. A ter resection o the de ormed sternum, the perichondrial bundles are f attened with
ree ng sutures. A newer, less invasive approach involving an external prosthesis to f atten the chest wall has been
described, but long-term ollow-up is yet to be determined.
REFERENCES
Albanese C , Rowe MI. Congenital thoracic de ormities. In: Benz ML, ed. Pediatric Plastic Surgery. 1st ed. Stam ord: Appleton &
Lange; 1998:730–733.
Borschel GH, Costantino DA, Cederna PS. Individualized implant-based reconstruction o Poland syndrome breast and so t tissue
de ormities. Ann Plast Surg. 2007;59(5):507–514.
Bostwick J. Plastic and Reconstructive Surgery of the Breast. St Louis, MO: Quality Medical Publishing; 1990:478.
Caouette-Laberge L, Borsuk D. Congenital anomalies o the breast. Semin Plast Surg. 2013;27:36–41.
Georgiade NG, Georgiade GS, Rie kohl R. Esthetic breast surgery. In: McCarthy JG, ed. Plastic Surgery. Vol. 6. Philadelphia, PA: WB
Saunders; 1990:3839–3840.
Hoehn JG, Georgiade GS. Congenital and developmental de ormities o the breast and breast asymmetries. In: Georgiade GS, Rie kohl
R, Levin LS, eds. Plastic, Maxillofacial and Reconstructive Surgery. 3rd ed. Baltimore, MD: Williams & Wilkins; 1997:715–729.
Jones KL. Smith’s Recognizable Patterns of Human Malformation. 5th ed. Philadelphia, PA: WB Saunders; 1997:302–303.
Karp NS. Gynecomastia. In: Grabb and Smith’s Plastic Surgery. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007:616–620.

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May N, Vasconez LO, Jurkiewicz MJ. reatment o macromastia in the actively enlarging breast. Plast Reconstr Surg. 1977;59:575–578.
Mc Kinney P. Gynecomastia. In: Aston SJ, Beasley RW, T orne CHM, eds. Grabb and Smith’s Plastic Surgery. 5th ed. Philadelphia, PA:
Lippincott-Raven; 1997:753–757.
Neuman JF. Evaluation and treatment o gynecomastia. Am Fam Physician. 1997;55:1835–1844, 1849–1850.
Rie kohl R, Zavitsanos GP, Courtiss EH. Gynecomastia. In: Georgiade GS, Rie kohl R, Levin LS, eds. extbook of Plastic, Maxillofacial
and Reconstructive Surgery. Baltimore, MD: Williams & Wilkins; 1997:820–828.
Rohrich RJ, Ha RY, Kenkel JM, Adams WP Jr. Classi cation and management o gynecomastia: de ning the role o ultrasound-assisted
liposuction. Plast Reconstr Surg. 2003;111:909–923; discussion 924–925.
Sadove AM, van Aalst JA. Congenital and acquired pediatric breast anomalies: a review o 20 years’ experience. Plast Reconstr Surg.
2005;115:1039–1050.
Simon BE, Ho man S, Kahn S. Classi cation and surgical correction o gynecomastia. Plast Reconstr Surg. 1973;51(1):48–52.
Skandalakis JE, Gray SW, Ricketts R, et al. T e anterior body wall. In: Skandalakis JE, Gray SW, eds. Embryology for Surgeons: T e
Embryologic Basis for the reatment of Congenital Anomalies. 2nd ed. Baltimore, MD: Williams & Wilkins; 1994:559–563.
uerk M. Medications that cause gynecomastia. Plast Reconstr Surg. 1993;92:1411.
van Aalst JA, Phillips JD, Sadove AM. Pediatric chest wall and breast de ormities. Plast Reconstr Surg. 2009;124:38e–49e.
Wood RJ, Bostwick J. Congenital breast de ormities. In: Benz ML, ed. Pediatric Plastic Surgery. 1st ed. Stam ord: Appleton & Lange;
1998:739–746.

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Amir H. Taghinia, MD and
Joseph Upton III, MD

What is the OMT classi cation system?
T e Oberg, Manske, onkin classi cation system is a new system or classi cation o congenital hand di erences.
T is system was proposed in 2010 and has been recommended to replace the previous IFFSH classi cation
system. T e new system re ects deeper clinical and genetic understanding o these conditions. It divides congenital
hand di erences broadly into three categories: mal ormations, de ormations, and dysplasias.

What is the most common congenital hand anomaly?
Polydactyly is the most common congenital hand anomaly and it a ects approximately 4 to 12 per 1,000 live
births.

What are the three types o polydactyly?
1. Radial polydactyly: Also known as preaxial polydactyly or thumb duplication. Most commonly seen in
Caucasian or Asian populations.
2. Central polydactyly: Unusual, comprises only 10% o polydactyly cases. Most o ten associated with syndactyly
(synpolydactyly).
3. Ulnar polydactyly: Also known as postaxial polydactyly. Most commonly seen in A rican-American or Native
American populations.
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What is the most common type o radial polydactyly (thumb duplication)?
ype IV; polydactyly occurs at the metacarpophalangeal joint level.

What are the di erent types o ulnar polydactyly? Which o these occurs most commonly?
T ere are three types o ulnar polydactyly. ype I is a so t tissue nubbin with a skin bridge. T ere is a small
neurovascular bundle within this bridge. ype II has skeletal connections with the polydactyly typically occurring
at the th metacarpophalangeal joint level. ype III is duplication o the entire ray. T e most common type is
type I.

Which thumb partner is typically ablated in reconstruction o radial polydactyly? Why?
Usually, the ulnar thumb is preserved and the radial partner is ablated. T e main reason to keep the ulnar partner
is because it preserves the ulnar collateral ligament o the metacarpophalangeal joint. In practice, the best-looking
and most unctional thumb is preserved.

In type IV radial polydactyly, i the ulnar partner is preserved, reattachment o what structure(s) is
important to ensure good long-term unction?
T e radial collateral ligament and the insertion o the thenar intrinsic muscles. Preservation o the ulnar partner
implies ablation o the radial partners. When the radial partner is removed, the radial collateral ligament and
the insertion o the thenar intrinsic muscles onto the radial side should be preserved. Once the radial partner
is discarded, these structures need to be reattached to the ulnar partner to restore balance and centralize the
metacarpophalangeal joint.
Figure 69-1 Wassel classi cation or radial polydactyly.

What classi cation system is most commonly used or radial polydactyly? What are the types o radial
polydactyly?
T e classi cation system most commonly used is the Wassel classi cation (Fig. 69-1). Radial polydactyly is
classi ed into six categories based on the level o the polydactyly. T e odd numbers indicate polydactyly at the
phalanx/metacarpal level, whereas the even numbers indicate polydactyly at the joint level. ype I polydactyly
occurs at the distal phalanx, type III at the proximal phalanx, and type V at the metacarpal level. ype II
polydactyly occurs at the interphalangeal joint, type IV at the metacarpophalangeal joint level, and type VI at the
carpometacarpal (CMC) joint level.

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What are the long-term un avorable outcomes a ter reconstruction o radial polydactyly?
Reconstructed thumbs are usually smaller and sti er than their normal counterparts. Bulges are possible i the
bi d metacarpal or phalangeal heads are not shaved. Deviations are possible i the intrinsic muscle tendons are not
rebalanced well.

What is syndactyly? What are the di erent types o syndactyly?
Syndactyly occurs when the ngers are used together—“webbed” digits. It is the second most common congenital
hand anomaly. Simple syndactyly indicates usion o the skin only. Complex syndactyly indicates usion o the
bone and skin. Complete syndactyly extends to the ngertips, whereas incomplete syndactyly terminates more
proximally (see Fig. 69-2).
Figure 69-2 ypes o syndactyly.

Which webspace is most commonly involved in syndactyly?
T e third webspace.

What is the embryological timing o digital separation?
T e upper limb bud rst orms around 4 weeks o gestation. Bones start to appear in the hand around 5 weeks
o gestation. Digital rays start to orm around 6 weeks o gestational age. At 7 weeks, loose mesenchyme between
digital rays starts to break down and notches appear between the rays. By the end o the eighth week, digital
separation is complete.

What is the typical age or syndactyly release? What are the indications or releasing the syndactyly earlier?
T ere is controversy about the optimal timing or syndactyly release. T e typical age or syndactyly release is
between 1 and 2 years, and most authors advocate release around 12 to 14 months o age. Release is per ormed
earlier i the syndactyly is causing abnormal deviation or angulation o the involved digits. A typical scenario is
syndactyly o the ourth webspace with bony usion at the distal phalanx. Ring nger growth is restricted distally
by a shorter small nger—thus causing ulnar deviation and possible PIP exion contracture o the ring nger.

What are the major principles o syndactyly release?
1. Full-thickness aps or reconstruction o the commissure—hourglass dorsal ap.
2. Zigzag incisions on the palmar sur ace to prevent contractures across exion creases.
3. Full-thickness skin gra ts or resur acing the resultant de ects—non–hair-bearing skin rom the lower abdomen
is advocated.
4. Adequate postoperative immobilization—well-padded long-arm cast.
5. Staged release—do not release both ulnar and radial sides during the same procedure.

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What is the proximal limit o syndactyly release (when do you stop releasing the webspace)?
T e bi urcation o the common digital artery is the proximal limit. I the nerve bi urcation is more distal, then the
nerve can be internally neurolysed to separate the digital nerves more proximally.

In a complex syndactyly, how can one be sure there will be adequate blood supply to each digit once the
syndactyly is released?
I there is any doubt about vascularity, one can obtain an angiogram preoperatively. T e other option is to
release the syndactyly in stages. In the rst stage, the syndactyly is released partially (i.e., convert to incomplete
syndactyly), and in the second stage release is completed.

What is the most common reason or skin gra t loss a ter syndactyly release?
Inadequate postoperative immobilization. Children are quite active and should be immobilized in a long-arm cast
to assure good skin gra t take a ter syndactyly release.

What is “web-creep”?
T is is mild to severe recurrence o the webbing a ter syndactyly release. T is can happen in the long term and is
related to a combination o growth and scar contracture at the base o the webspace. Minor procedures may be
required (z-plasty) to release the webspace.

What is the best method or surgical release o the rst webspace? Other than the skin, what other
structure(s) need to be released? What are two commonly used f aps or reconstruction o tight rst
webspaces?
T e best method or surgical release o the rst webspace is a our- ap z-plasty. When releasing the rst webspace,
it is important to release the tight investing ascia o the rst dorsal interosseous and adductor pollicis muscles.
T e two most commonly used aps or reconstruction o a tight rst webspace are the reverse pedicled radial
orearm ap and the groin ap. Other options include the reverse pedicled posterior interosseous ap (which is
less reliable) or ree asciocutaneous aps.

What anatomic eature distinguishes constriction ring syndrome rom symbrachydactyly?
T e hand mani estations o constriction ring syndrome and symbrachydactyly can look very similar. Both involve
shortened, used, and/or webbed digits. Symbrachydactyly, ormerly called atypical cle t hand, typically a ects
the hand in the shape o a U—with the central digits/hand being more a ected. T e major anatomic eature that
distinguishes these two entities is that in constriction ring syndrome, the anatomy proximal to the level o the
de ormation is completely normal. T is allows more reliable unctional return a ter reconstruction surgery such as
toe trans ers.

How is constriction ring o the digits treated?
Excision o the cutaneous ring and at/ ascia advancement aps with or without z-plasties.

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Outline the classi cation system or constriction ring syndrome.
T e classi cation system or constriction ring is shown below:
ype I (mild)—Shallow indentation o skin and so t tissue without distal lymphedema.
ype II (moderate)—Distal lymphedema, acrosyndactyly, and even discontinuous neurovascular or
musculotendinous structures, but without vascular compromise.
ype III (severe)—Progressive lymphaticovenous or arterial compromise.
ype IV—Intrauterine amputation.
A type IIIB was introduced by Weinzweig (Plastic and Reconstructive Surgery, 1995). T is is a dynamic sub-type
which shows progressive swelling along with evolving vascular compromise. Limb salvage can be per ormed with
emergent surgery despite a severe band, thereby, reducing the likelihood o limb loss. T ese limbs are o ten le t
with long-term growth retardation and neurologic de cits.

What are treatment options or short digits (e.g., in constriction ring syndrome or symbrachydactyly)?
T e treatment options are (1) vascularized toe trans ers, (2) distraction lengthening, and (3) nonvascularized toe
phalanx trans ers. Vascularized toe trans ers are technically demanding but can provide excellent unction and
appearance. Distraction lengthening is dif cult in the phalanges and prone to complications. Nonvascularized
toe phalanx trans ers require supple, elastic so t tissues to accommodate the trans erred phalanges. None o these
options should be entertained i the child has good or excellent unction.

What is symphalangism?
Fused phalanges. T e digits are usually short and sti . T e joints are not present or do not bend. T ere are no
exion/extension creases.

What is clinodactyly?
Deviated digit (see Fig. 69-3). T e deviation is in a radioulnar direction. T e most commonly a ected digit is the
th digit where deviation occurs toward a radial direction at the level o the middle phalanx. A “delta” phalanx
may cause clinodactyly. A delta phalanx is a condition where the growth plate on one side o the digit does not
grow as well as the other side, causing deviation in a radioulnar direction.
Figure 69-3 Clinodactyly.

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What is camptodactyly?
Bent digit (see Fig. 69-4). T is re ers to a exion de ormity o a digit in the AP plane. It usually involves the PIP
joint o the th digit.
Figure 69-4 Camptodactyly.

What are the possible anatomic anomalies that cause camptodactyly?
T ese could be abnormal insertions o intrinsic and extrinsic tendons that cause an imbalance o exion/extension.
T e most common variations are abnormal insertions o the intrinsic muscles or exor digitorum super cialis
within the digit. Joint contractures and tight collateral ligaments are usually secondary and not the primary
de orming orce.

When is it appropriate to release camptodactyly?
In general, most patients with this condition should be treated with stretching and splinting. Surgical intervention
may be appropriate or those with > 70 degrees o exion contracture. However, the results are not satis actory in
the long term and recurrence is common.

What is a congenital trigger thumb?
Congenital trigger thumb is a condition where the IP joint o the thumb is held in exion and cannot be passively
extended. Focal overgrowth o the exor tendon is a possible cause. T e exor gets stuck proximal to the A1
pulley causing exion de ormity. A palpable nodule called Notta’s node is typically elt at the A1 pulley level. T e
treatment is surgical release o the A1 pulley. In children younger than 18 months, spontaneous resolution may
be seen within 6 months. I the child is older than 2 years, one should entertain release. Steroid injections do not
work in children. T e most common complication o surgical release is injury to the digital nerves.

What is macrodactyly? When does one amputate versus debulk?
Large digit. Debulking can be entertained or mild orms o macrodactyly. However, i the nger is very large,
has poor range o motion, inhibits hand unction, and is insensate (or has poor sensation), then it should be
amputated.

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What is the classi cation system or thumb hypoplasia? Describe the ve types o thumb hypoplasia?
T e classi cation system is the Blauth system.
ype I: small thumb with normal joints, hypoplastic intrinsic muscles, and normal unction.
ype II: smaller thumb, joints normal with possible ligament instability, more hypoplasia o the intrinsic muscles,
possible rst webspace de ciency.
ype IIIa: small thumb with laxity o the MP joint collateral ligaments, thenar intrinsic muscles are small and
weak, tight rst webspace but unctional CMC joint—child holds objects against MP head o the thumb.
ype IIIb: similar to type IIIa except metacarpal is incomplete and the CMC joint is not unctional—child cannot
hold objects with thumb.
ype IV: oating thumb (“pouce ottant”)—skin bridge connection to the hand only.
ype V: complete absence o thumb.

What are the treatment options or the di erent types o thumb hypoplasia?
ype I: no treatment.
ype II: based on de ciency—possible webspace release with or without ligament reconstruction or
opponensplasty.
ype IIIa: webspace release, opponensplasty, and MP joint ligament reconstruction.
ype IIIb, IV, and V: pollicization.

What is the best age in which to per orm a pollicization procedure?
Index nger pollicization remains the ideal restorative procedure or a missing or extremely hypoplastic thumb.
While there is no clearly established age or doing this procedure, the surgeon has to balance early intervention
or better cortical integration with delayed intervention to allow a technically easier operation due to size o the
anatomic structures. Considering these issues, most surgeons agree that the “best” age or pollicization is between
1 and 2 years o age.

What are the options or treatment o a Blauth type IIIb thumb?
Most surgeons agree that the best reconstructive option or a type IIIb thumb is ablation o the native hypoplastic
thumb and index nger pollicization. In addition to severe hypoplasia o the intrinsic and extrinsic muscles, a type
IIIb thumb lacks a unctional CMC joint. No ligament reconstruction or tendon trans er known can make up
or this de ciency. A ew authors have reconstructed the CMC joint with a ree metatarsophalangeal joint trans er
rom the toes. Results or this operation have not shown superiority to index nger pollicization.

What are the options or opponensplasty in congenital cases?
ypically, the exor digitorum super cialis to the ourth digit is trans erred. It is common to use this or type II
or IIIa thumbs, because the distal end o the tendon can be used to reconstruct o the radial and ulnar collateral
ligaments. In other cases, the abductor digiti minimi can be trans erred (Huber trans er).

What is radial club hand?
Best termed radial longitudinal de ciency, this condition occurs when the radius is shorter than the ulna. T ere
are our types, ranging rom mild longitudinal de ciency to complete absence o the radius. T e patients have
short orearms with radially deviated wrists. T umb hypoplasia or absence is a common associated nding.

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Plastic & Reconstructive Surgery Board Review

What are other conditions associated with radial or thumb de ciency?
T ese mani est as hematologic and/or cardiac anomalies.
1. Holt–Oram syndrome: Autosomal dominant condition with cardiac anomalies (septal de ects, conduction
de cits such as atrial brillation).
2. Fanconi anemia: Bone marrow ailure around age 6 to 7.
3. T rombocytopenia absent radius: Low platelet levels, thumb is usually una ected.

What are the common hand mani estations o Poland syndrome?
Hand hypoplasia and brachysyndactyly (short, webbed digits).

What is Apert syndrome?
Acrocephalosyndactyly. Cranio acial anomalies (mid ace hypoplasia) and symphalangism with syndactyly o the
hands and eet.
REFERENCE
onkin M, olerton S, Quick , et al. Classi cation o congenital anomalies o the hand and upper limb: development and assessment
o a new system. J Hand Surg. 2013;38:1845–1853.

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Javier A. Couto, BS and
Arin K. Greene, MD, MMSc

How are vascular anomalies classi ed?
Lesions are divided into tumors and mal ormations.
Vascular Anomalies
CHAPTER 70
Tum ors

Malform ations
Infantile hemangioma

Capillary
Congenital hemangioma

Lymphatic
Kaposiform hemangioendothelioma Venous
Pyogenic granuloma

Arteriovenous
Overgrowth syndromes (e.g., Klippel–Trenaunay)

What is in antile hemangioma?
A benign tumor o endothelial cells.

How common is in antile hemangioma?
It is the most common tumor o in ancy; 4% to 5% o Caucasians are a ected.

What does in antile hemangioma look like?
Red; but deeper lesions under the skin may appear bluish.

What tissues are af ected by in antile hemangioma?
Skin and/or subcutaneous tissue o the head and neck (60%), trunk (25%), or extremity (15%). T e liver is the
most common visceral location.