Primary Cleft Lip Repair

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CME Primary Repair of Bilateral Cleft Lip and Nasal Deformity   John B. Mulliken, M.D. Boston, Mass.

Learning Objectives: After studying this article, the participant should be able to: 1. List five principles that guide synchronous repair of bilateral complete cleft lip and nasal deformity. 2. Explain how different growth rates for the  principal nasolabial features are applied during primary repair. 3. Describe two approaches for positioning the alar cartilages to form the columella. 4. Discuss the influences on referral patterns for a newborn with bilateral cleft lip.

Traditional repair of bilateral cleft lip focused on labial closure but accentuated the nasal deformities, which were addressed later. By the end of the past century, singlestaged labial closure had replaced the old multistaged procedu proc edures res and the tec techni hnical cal emp emphas hasis is had beg begun un to shi shift  ft  from secondary to primary nasal correction. Now, presurgical maxillary orthopedics sets the bony foundation for synchronous nasolabial repair and for closure of the al veolar clefts. The study of normal nasolabial growth and the typical stigmata of the conventional methods provides the nece necessary ssary forek foreknowle nowledge dge to guide surgic surgical al sculpt sculpture ure in three dimensions and to anticipate the fourth dimension. The convergence convergence of seve several ral force forcess are chang changing ing refer referral ral lines for children born with bilateral cleft lip. These include affirmation of centers of excellence, surgeons’ selfregulation, prenatal diagnosis, economics of health-care delivery, deliv ery, and incre increasing asing parental sophi sophistica stication tion.. Thes These e pressures are not necessarily in conflict. Care by a subspecia spe cializ lized ed pla plasti sticc sur surgeo geon n and exp experi erienc enced ed tea team m is in the besst in be inte tere rest stss of th the e ch chil ild d an and d th the e thi hird rd-p -par arty  ty  payyer pa er.. (Plast. Reconstr. Surg.  108: 181, 2001.)

These branded children beckoned surgeons to change their traditional operative strategies. Over Ov er th the e pas pastt dec decade ade,, tw two o imp import ortant ant ad vances have been made in the repair of bilateral cleft lip and nasal deformity: (1) evolution to singlesingle-stage stage nasolabial closure with positioning of the alar cartilages and sculpting of the soft tissues to shape the columella and nasal lobule, and (2) improved techniques for presurgical maxillary alignment to permit closure of the alveolar clefts and facilitate primary nasolabial repair. Although the principles for single-stage repair are established, craftsmanship continues to evolve. Now, over a half century  after Barrett Brown’s discomfortable observation, it can be said that the outcome for the infant born with bilateral cleft lip is equal to 2 and surpass thatgiven of itsthe unilateral counterpart.can Furthermore, preoperative ad vantage of nasolabial symmetry, these children require very few revisions. Before undertaking primary repair of double cleft lip, there are some lessons to review.

 James Barrett Brown and colleagues introduced their 1947 article with the pithy statement that a bilateral cleft lip is twice as difficult  to repair as a unilateral cleft and the results are only half as good.1 Indeed, techniques for correction of bilateral cleft lip have lagged behind those for unilateral cleft lip. The infant with bilateral cleft lip has been subjected to multiple procedures only to endure sundry revisions throughout childhood. Despite the surgeon’s best efforts, the child’s diagnosis was painfully  obvious to all—even at a distance—and these

LESSONS FROM   SURGICAL   HISTORY 

Many com Many comple plex x malf malforma ormatio tions, ns, onc once e onl only  y  repara rep arable ble by st stag aged ed op opera erati tion ons, s, ca can n be co corrrected, rect ed, usu usually ally more suc succes cessfu sfully, lly, in a sin single gle procedure. This lesson is illustrated in the annals of the bilateral cleft lip and nasal deformity. Emphasis had focused on labial closure

stigmat sti gmataa were not easi easily ly eras erased ed by revi revision sions. s.

and ignored nasal distortion. Surgical text rec-

From the Division of Plastic Surgery and Craniofacial Centre, Children’s Hospital, Harvard Medical School. Received for publication August  4, 2000; revised October 17, 2000.

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ommended staged repair, one side of the cleft  and then the other. There was a misconception that the diminutive prolabium lacks the potential for growth. Techniques for repairing the doubledou ble-labi labial al cle cleft ft were adap adapted ted fro from m tho those se used for the more common unilateral form, and they typically involved the introduction of  a rectangular or triangular flap from the lateral

producess a mid produce midlin line e nas nasolab olabial ial cre crease ase tha that  t  deepens deepe ns with smili smiling. ng. The circ circumfe umferenti rential al philtral scar produces a bulge rather than a dimple. The best prolabial scars follow repair in infancy, done under minimal tension. Recruiting tines of a forked flap from the central lip in a child can cause thickened and permanently wide philtral scars. Even in the best of 

labial to left augment prolabial height. These elements procedures geometric (often asymmetric) labial scars and usually resulted in a long-l lon g-lip ip def deform ormit ityy and a tig tight ht upp upper er lip lip.. Straight-line repair minimized vertical elongation of the central lip but produced an abnormally wide and shield-shaped philtrum. There  was longstanding controversy over whether or not to preserve the prolabia prolabiall vermilion, leave it  as a tiny strip, or excise it completely. Apposition of the orbicularis oris muscle was usually  not mentioned. Some surgeons thought muscular cul ar clo closur sure e wou would ld inh inhibi ibitt pre premax maxill illary  ary  3 growth. Nevert Nevertheless, heless, with increas increasing ing attention to muscular closure in the unilateral deformity, reports began to underscore the importan por tance ce of orb orbicu icular lar rep repair air in bil bilate ateral ral 4–8 clefts. Surgeons conceded to the complexity of the bilateral cleft nasal deformity and deferred correction.9–11 The conventional teaching was that  the col columel umella la is ina inadequ dequate ate,, and num numerou erouss “secondary” procedures were devised to elongate the “short columella.” There are two ma jor strategies.12 The first, popularized by Cronin, involves rotating bipedicled straps of tissue from the nostril sills.13 This method gives modest columellar length. A second method, the forked-flap procedure of Millard, involves re-

hands, the staged forked-flap(1) procedure results in an unusual appearance: a rectangular columella (with a broad base and without a  waist); (2) a sharp columellar-labial angle; 3) abnormally elongated/enlarged nostrils; (4) a tendency to columellar over-elongation with a disproportionate ratio of nostril length-to-nasal tip; and, sometimes, (5) a downward drift of  the columellar base.16–18 Furthe Furthermore, rmore, the medial cru crura ra beco become me unn unnatu aturall rallyy pos positi itioned oned in the tip, resulting in a break at the columellarlobula lob ularr con connec necti tion. on. Bec Becaus ause e of th these ese pro probblems, some surgeons began to wonder whether labi la bial al sk skin in be belo long ngss in th the e co colu lume mell lla—or a—or if  more tissue is needed at all.  Whereas delayed nasal repair was customary  for the repair of bilateral cleft lip, the simultaneous correction of the unilateral left lip and nasal deformity became accepted practice. Further th ermor more, e, th there ere was no ev evide idenc nce e th that at ea early  rly  manipulation of the alar cartilage impairs the development of the nasal tip. LESSONS FROM   BILATERAL   CLEFT   STIGMATA 

Every child with a repaired bilateral cleft lip has a characteristic appearance whose origins are both intrinsic to the malformation and iatroge tr ogeni nic. c. Th The e ph philt iltrum rum is bo bowed wed,, wi wide, de, un un-dimpled dimp led,, ove overly rly long long,, oft often en asym asymmet metric, ric, and

5,9,14

cru cruiti iting ng labial ialpermutations tissue tis sue to th the e this colume col umella lla.. In There arelab two of method. infants with a wide prolabium, the tines of the forked flap are banked at the time of labial closu cl osure re an and d tr tran ansp spose osed d to th the e co colu lume mella lla in earlyy chi earl childh ldhood. ood. More oft often, en, Mil Millard lard pref prefers ers three-stage columellar lengthening: (1) bilateral labial adhesions to stretch the prolabium; (2) elevation, rotation, and banking the tines  while  whi le nar narro rowin wing g the phi philtr ltrum um (at ag age e 18 months); and, finally, (3) retrieval of the prolabial prongs and elevation, along with the medial crura, to augment the columella (at age 2  years).15 The forked-flap method, like all secondary  procedure proc edures, s, caus causes es pecu peculiar liar tert tertiary iary dist distoror16 tions. Most techniques introduce a nexus of  scars across the columellar-labial junction. This

lacking a white ridge. If the vermilion-mucosa is preserved, theprolabial free margins of  the th e lat latera erall lab labial ial ele eleme ment ntss han hang g lik like e swa swags gs,, flanking a thin median tubercle that is covered by insufficient vermilion and chapped mucosa (“whistling lip deformity”).19 In profile, the upper lip is flat or convex, whereas the lower lip everts (“cleft lip lower lip deformity”).20 The child struggles to obtain bilabial closure over a protrusi prot rusive, ve, retr retrocl oclined ined,, and vert vertical ically ly elon elon-gated premaxilla. The accompa accompanying nying nasal deformities are primarily deformational but are also postsurgical. The tip is broad, the medial crura are pulled inferior-posteriorly, the nostrils are slumped, the alar domes are buckled and splayed, and the alae nasi are flared, sometimes likened to “cat’s knees.” 19 Often the alar lobules are hypoplastic (a primary deformity).

 

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The caudal margin of the alar cartilage protrudes into the lateral vestibule, producing an oblique ridge or web.21 Without amends for the  vertically long lateral labial elements and normal muscular attachment of the alar bases, an unnatural elevation of the alae nasi will occur and become more pronounced whenever the child smiles.22 But of all the nasolabial distor-

splayed alar cartilages. The nasal tip was narrowed by V-Y plasty. Bilateral adhesions were done, followed by definitive labial repair at a second sec ond sta stage. ge. The col colume umella llae e loo looked ked qui quite te 25 normal in McComb’s assessment at 4 years. Mulliken also focused on early positioning of  the alar cartilages. This was initially done at a second sec ond stag stage e in con conjunc junctio tion n with int intrana ranasal sal

tions, the short columella is most obvious. Principles that guide the surgical repair of  bilate bil ateral ral cle cleft ft lip and nas nasal al def deform ormity ity hav have e been bee n in indu duce ced d fr from om th the e st stig igmat mataa of co conv nven en-tional techniques and by study of the literature19: (1)   Symmetry . This is foremost. Staged repair portends asymmetry. Even the smallest  nasolabial difference on the two sides will magnify with growth. (2)   Primary muscular continu-  ity . Orbicularis oris muscular bundles must be comple com pletel telyy mobi mobilize lized d from the lat lateral eral labi labial al elements and apposed throughout the vertical extent of the upper lip. (3)  Proper philtral size  and sha shape  pe . The con constr struct ucted ed phi philtr ltrum um wid widens ens remarkably (in the upper portion more so than inferio inf eriorly) rly) and dis display playss con conside siderabl rable e vert vertica icall growth.. (4) Formation of median tubercle from lat-  growth eral labial elements.   There is no prolabial white roll in the complete deformity, and both central vermilion and mucosa are deficient. (5) Primary positioning of alar cartilages to construct the  nasal nas al tip and col colum umell ella. a.   Techniques based on this principle have dramatically changed the faces of children born with bilateral cleft lip.

transposition the banked tinesabandoned, of a forked flap.19 By l987,ofbanking had been and primary columellar lengthening and nasaltip projection were achieved solely by apposition and elevation of the alar domes and by  sculpt scu lpting ing the nas nasal al sof softt tis tissue sues. s.22 Oth Other er surgeons were on the same track to primary nasal corre co rrect ctio ion. n. In 19 1991 91,, Tr Trot ottt an and d Mo Mohan han wer were e  working in Malaysia, where socioeconomic factorss mad tor made e mul multis tistag taged ed rep repairs airs imp imprac ractic tical. al. They devised a single-stage nasolabial repair, based base d on open rhinoplast rhinoplastic ic exp exposu osure re of the 26 dislocated disloca ted alars. Cut Cuttin ting g and asso associa ciates tes described another variation on the open-tip approach proa ch and adde added d pres presurgi urgical cal str stretc etchin hing g or elongation of the columella.27 “The columella is in the nose” became the shibboleth shibbole th of surgeon surgeonss who advocated primary  16,25 nasal repair. The old, non-anatomic techniques that involved secondary recruitment of  prolabial skin into the columella were wrong. Instead, the alar cartilages should be placed in the proper position at the time of labial repair, followed by trimming and redraping the soft  tissues of the nasal tip. No longer would the columella columel la includ include e labial (often hair-bea hair-bearing) ring) skin, the nasolabial junction be transgressed by  scars sc ars,, or th the e ph philt iltrum rum be en enci circl rcled ed by sc scar ar tissue.

THE   K EY  EY : PRIMARY   R EPAIR E PAIR OF DEFORMITY 

THE

 N ASAL

Nasal dissection of stillborn infants with bilatera lat erall lab labial ial cle clefti fting ng rev reveal ealss tha thatt the ala alarr domes crura are splayed, caudally  23 rotatedand like middle a “bucket handle,” and sublux subluxed ed from their normal anatomic position overlying the upper lateral cartilages. cartilages.19,23 Broadben Broadbentt and 24  Woolf  de desc scrib ribed ed a cas case e of pri primar maryy med media iall advancement of the alar domes combined with excision of skin from the broad tip. However, it   was McComb who led the vanguard for primary  columel colu mellar lar elo elonga ngation tion.. He ini initia tially lly tri tried ed pri21 mary elevation of a forked flap and published his follow-up analysis 10 years later, including measurements measurem ents of columel columellar lar growth growth..23 By then he had become disenchanted with this strategy  and presented a revised technique for primary  nasal repair in 1990—without a forked flap. 19 In the first stage, McComb used an external incision (“flying bird”) to open the nasal tip and allow the apposition and suspension of the

LESS ESSONS ONS FRO FROM M

THE

  FOURTH   DIMENSION

Unlike the sculptor who works in stone, the surgeon must work with a patient who grows and who whose se nas nasola olabia biall pro propor portio tions ns cha change nge.. Thus, Thu s, th the e su surge rgeon on mu must st co conc ncept eptual ualize ize th the e child’s appearance as a young adult. To do so, the surgeon must have a thorough understanding of thr three-d ee-dime imensio nsional nal form and fou fourth rth-dimensional alterations that occur with normal growth. Thankfully, Farkas and colleagues documented the changes in the important nasolabial features, between 1 and 18 years, in 1593 North American Caucasians.28 Nasal height (nsn) and width (al-al) develop early, reaching a mean mea n of 76 76.9 .9 an and d 87 pe perc rcen entt of adu adult lt si size, ze, respectively, by age 5 years. In contrast, nasaltip protrusion (sn-prn) and columellar length

 

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(sn-c (sn-c') ') develop slowly, attaining a mean of only  two-thirds two-t hirds of adult size by 5 years of age. All the labia la biall lan landma dmarks rks gro grow w rap rapidl idly, y, rea reach chin ing g approximately 90 percent of adult proportions by  age 5 years. The cutaneous upper lip attains adult height by 3 years in girls and by 6 years in boys.30  A working fourth-dimensional hypothesis for

ferior to the basilar premaxilla, causing it to lingually incline and causing the vomer to bow. The most commonly used active presurgical devic dev ice e is bas based ed on th the e pr prot otot otyp ypic ic de desig sign n of  Georgiade Georgiad e and associat associates, es,30,31 refined and popularized by Latham while working in collaboration rat ion wi with th Mi Milla llard rd (F (Fig ig.. 1) 1)..32,33 The acry acrylic lic plates plat es of the cus custom tom-ma -made de app applian liance ce are

the of bilateral cleft is programmed to craft on a smallrepair scale those features thatlip are for rapid growth, compared with normal, agematched infants. The corollary premise is that  slow-g slo w-growi rowing ng feat features ures can be con constru structe cted d of  norm no rmal al siz size e or sl slig ight htly ly la large rgerr th than an no norma rmall 16 size. This accounting for temporal changes mustt also inc mus include lude the naso nasolabi labial al dis distort tortions ions that are particular to children with repaired bilate bil ateral ral cle cleft ft lip lip.. Su Such ch kn knowl owledg edge e can be gained only by observation of older patients, one’s own and those of predecessors and colleagues. The fast-growing features grow even faster in the child with bilateral cleft lip, with the exception exception of the medi median an tub tubercl ercle. e. Con Con- versely, the slow-growing features, specifically  nasal-t nas al-tip ip proj project ection ion and colu columell mella, a, seem to grow more slowly in the child with bilateral cleft lip.29

pinned the maxillary shelves.the A looped is passedtotransversely through neck ofwire the premaxilla, just behind the premaxillary alveoluss an lu and d we well ll an ante teri rior or to th the e pr prem emax axil illa lary ry- vomerine suture. The maxillary segments are expanded by a ratcheted screw in the midplane of th the e de devi vice ce,, wh whic ich h is tu turn rned ed da dail ilyy by th the e child’s parents. Elastic chains on each side are connected to the trans-vomerine wire, looped around a pulley in the posterior section of the appliance, and attached to a cleat on the most  anterior point of the maxillary acrylic plates. Tension on the elastic chain retracts the premaxilla; tension may be periodically adjusted. Typically it takes about 6 weeks to align the premaxilla with the expanded palatal segments to effect closure of the alveolar clefts (gingivoperiosteoplasty). Latham’s device is most successful in correcting the premaxillary anteroposterior position; however, the movement is more retr retrocli oclinat nation ion tha than n retr retropos opositi ition. on. The device is somewhat less successful in amending rotation and is least successful in preventing  vertical elongation.

PRESURGICAL   PREMAXILLARY –M –M AXILLARY  M ANIPULATION

Synchron Synch ronous ous na nasol solabi abial al rep repair air can be accomplished only after proper alignment of the three maxillary segments. Most bilateral cleft  lips are complete, but sometimes there is a tiny  band on one side that causes rotation of the premaxilla. The protrusive premaxilla must be retracted and centralized, whereas the lateral maxillary require expansion. There aresegments two basicusually strategies for presurgical premaxillary orthopedic manipulation, active and passive. The latter method is favored by  those who are concerned about the potential deleterious effects of forcing the segments into position. The passive molding plate is retained by und underc ercuts uts;; thi thiss mai mainta ntains ins the tra transv nsvers erse e  width of the maxillary maxil lary segments. Because B ecause there is no expansion, space is often inadequate for the th e pre premax maxil illa. la. Ext Extern ernal al fo forc rce e is ne need eded ed to retract ret ract the prem premaxi axilla, lla, usi using ng eit either her adhe adhesiv sive e tape, an elastic band attached to a headcap, or bilateral labial adhesion. Because there is no muscle in the prolabial element of a bilateral complete cleft lip, preliminary labial adhesions are pron prone e to deh dehisc isce. e. Fur Furthe thermor rmore, e, ext externa ernall traction techniques tend to focus pressure in-

FIG. 1. The Latham pin-retained pin-retained presurgic presurgical al orthopedic orthopedic appliance. Tightening the elastic chain retrudes the premaxilla; turning the screw expands the palatal shelves.

 

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Some orthodontists think there are no advantages to premaxillary orthopedics in the management of bilateral cleft lip.34 However, proponent proponentss of active versus passive techniques constitute the two maj major or sid sides es of th the e on ongoi going ng deb debate ate.. Ear Early  ly  longitudinal studies of children managed with a Latham device show no serious deleterious effects on occlusion and growth.33,35 However, critics of-term active premaxillary orthopedics document  long-te long rm evid evidenc ence e for mino minor r midf midfacia acial l retr retruu36,37 sion.  What  Whatever ever the outc outcome ome in term termss of midfacial fac ial posi position tion,, thre three e advan advantage tagess of pres presurgi urgical cal align ali gnmen mentt of th the e max maxill illary ary seg segmen ments ts mus mustt be under un dersc score ored: d: (1 (1)) it pe permi rmits ts phi philtr ltral al des desig ign n of  proper proportions, (2) it facilitates primary nasal correction, and (3) it allows closure of the alveolar gaps, thus preventing fistulas and (possibly) permitting bony ingrowth and stabilization of th the e ar arch ch.. Fu Furt rthe herm rmor ore, e, if th ther ere e is a ne neararnormal maxillary foundation, there could be less postoperative prolabial distortion and interalar  widening  wide ning.. If the chi child ld exh exhibit ibitss midf midfacia aciall retr retruusion, maxillary advancement is a predictably successf ce ssful ul pro proced cedure ure whe when n don done e af after ter co compl mplet etion ion of  growth. In the near future, it is likely that maxillary distraction with an entirely internal device  will be availab available le for patient patientss who might might benefit benefit in childhood. PRIMARY  R EPAIR E PAIR OF

THE

 

DEFORM DEF ORMITY  ITY 

  PRIMARY   P ALATE

Markings 

The infant is typically 4 to 5 months old at  the time of synchronous repair. The philtral flap is designed with slightly biconcave sides and an d a da dart rt-s -sha hape ped d ti tip. p. Th The e si size ze of th the e fl flap ap depends on the raceofand of the For infant and on the appearance theage parents. a Caucasian infant 4 to 6 months of age, suggested dime di mens nsio ions ns ar are e 6 to 8 mm fo forr ph phil iltr tral al fl flap ap length: 3 to 4 mm wide between the peaks of  Cupid’s bow and 2 mm wide at the columellarlabial junction. A strip of skin is drawn on each side of the philtral flap; these will be deepithelialized to simulate the philtral ridges. The proposed Cupid’s bow peak-points are sited on the lateral labial elements so there will be sufficient  central centr al white roll for the handle of the Cupid’s bow and enou enough gh verm vermilio ilion n to con constr struct uct the median tubercle. tubercle. The alar base flaps are drawn at th their eir ju junc ncti tion on wi with th th the e lat later eral al lab labial ial ele ele-ments.. The vermilion-mucosal ments vermilion-mucosal line is tattoo tattooed, ed, as are the other important anatomic points to be preserved during repair (Fig. 2,  above, left ). ).

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 Dissection 

The philtral flap is incised, the flanking tabs are deepithelialized, and the remaining prolabial skin is discarded. The lateral white line vermilion-mucosal flaps are incised, and the alar base flaps are elevated. Orbicular muscular bundles bund les are diss dissect ected ed from the lat lateral eral labi labial al elements (Fig. 2, above, center ). ). The splayed alar cartilages cartila ges are exp exposed osed through through the rim inc inciisions; it is helpfu helpfull to support the cartilages cartilages with a cotton-tipped swab (Fig. 2,  above, right ). ). Labial Closur Closure  e 

Mucosal flaps are elevated from the lateral and medial sides of the cleft defects to construct the nasal floors. Gingivomucoperiosteal flaps are apposed, closing the alveolar clefts. The premaxillary vermilion-mucosa is trimmed to shorten the anterior wall of the gingivolabial sulcus (Fig. 2,  second row, left ). ). The remaining premaxillary mucosa is sutured to the periosteum to form the posterior wall of the anterior gingivolabial sulcus. The lateral labial elements are advanced medially as the buccal sulci are closed. The lateral mucosal flaps form the anterior wall of the central sulcus. The orbicular muscles are apposed apposed,, inferio inferiorly-torly-to-superio superiorly, rly, throughout the vertical height of the lip. The uppermost suture suspends pars peripheralis to the periosteum of the anterior nasal spine (Fig. 2,  second row, right, left panel ). ). The redundant tips of the lateral labial flaps are trimmed to form the median tubercle (Fig. 2,  second row, right, right panel ). ). The distal end of the philtral flap is inset. The philtral flap is secured to the muscular layer. This helps to depress the philtral flap and to raise the lateral labial lab ial fl flaps aps in an ef effo fort rt to si simul mulat ate e ph philt iltral ral ridges. Yet, a realistic philtral dimple and flanking columns seem just beyond the surgeon’s craft. The cephalic margin of the cutaneous flaps must be trimmed to correct for lateral labial height; rarely is adjustment necessary at  the medial edges. These final steps in cutaneous cl closu osure re sh shoul ould d be don done e af afte terr na nasal sal correction. Nasal Repair 

 A midline nasal-tip incision is not necessary, for with experience, it is possible to fully visualize the dislocated alar cartilages through bilateral rim incisions.29  An interdomal mattress suture is placed to appose the middle crura and genua. One or two mattress sutures sus-

 

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FIG. 2. The Mul Mullik liken en met method hod of sing singlele-stag stage e naso nasolab labialrepai ialrepair: r: the sem semi-o i-open pen app approa roach ch to nasa nasal-t l-tip ip car cartil tilage agess through bilateral rim incisions.

pend each lateral genu (and lateral crus) to the ipsilateral upper lateral cartilage (Fig. 2, third row, left ). ). A cinch suture is placed through each alar base and is tightened until the interalar distance is less than 25 mm (Fig. 2,   third  row ,   right ). ) . The tips of the alar base flaps are trimmed to form the nasal sills. A suture placed through the dermis of each alar base to the underlying muscle serves to (1) prevent alar elevation with smiling and normal action of the depressor alae nasi muscles, and (2) form the

normal cymal shape of the lateral sill (Fig. 2, third row, right panel ). ). Once the alar cartilages are in proper position, extra skin in the soft triangles becomes obvious and should be excised, including the skin of the lateral columella (Fig. 2,  below, left, left panel ). ). This resection narrows the tip, defines fin es the col colume umella llar-lo r-lobul bular ar jun juncti ction, on, elon elon-gates the nostrils, and narrows the columellar  waist. There is also redundancy in the vestibular lining that becomes apparent after position-

 

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FIG. 3 . (Above, left ) Newborn with bilateral complete cleft lip/palate. The cutaneous band on the left is incised to allow  placement of the transverse pin of the Latham device. ( Above, center ) Intraoperative view of patient at age 4.5 months, after maxillary orthopedics and premaxillary centralization. (Above, right ) Intraoperative view after single-stage nasolabioalveolar repair. (Below, left, center, right ) Appearance of patient at age 1 year (6 months postoperatively).

ing the alar cartilages. Lenticular excision of  this extra mucosa helps to obliterate the lateral  vestibular web and supports the lateral crura (Fig. 2,  below, left, inset ). ). The completed repair is illustrated in Figure 2,   below, right . Clinical examples are show in Figures 3 and 4. TECHNICAL   MODIFIC ODIFICATIONS ATIONS FOR   A NATOMIC NATOMIC   V   ARIANTS

 Although most bilateral cleft lips are complete, there are various incomplete incomplete forms, symme metr tric ic an and asymme asy mmetr tric ic,,ridges with wi th and a sp spec ectru trum m of  clefting ofdthe alveolar secondary  palate.38

Symmetric Bilateral Incomplete 

Certainly Certai nly,, the sym symmet metric ric bila bilater teral al inc incomomplete variant is the easiest to repair. Usually, the alveolar ridge is intact or there is minor notching. The steps are the same as described for the bilateral complete deformity, with some technical considerations. The most important  decision is whether or not to build the median tuberc tub ercle le fro from m the lat lateral eral labi labial al ele elemen ments ts (afores (af oresaid aid prin princip ciple le 4). If the there re is suf suffic ficien ient  t  prolabial prol abial muscle, white roll roll,, and verm vermilio ilion, n, thes th ese e ca can n be pre prese serve rved d and apposed apposed to th the e corresponding lateral elements in a bilateral butt joint. However, more often than not, the cent ce ntral ral li lip p sh shou ould ld be co cons nstr truc ucte ted d as in th the e

 

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FIG. 4 . (Above, left ) Newborn with bilateral cleft lip, right cutaneous band, and intact right  alveolus alveo lus and secon secondary dary palate. ( Above, right; below ) Appearance of the patient at age 5.5 years.

complete deformity. Careful attention should be given to the width of the philtral flap because it has the same tendency to overgrow  transversely, as in the complete deformity. Positio Posi tionin ning g the alar car cartil tilages ages may not be necessary unless the columella measures short  and the genua are slumped. Another caveat is to sufficiently narrow the interalar dimension, for it will widen with time. Asymmetric (Complete/Incomplete) 

There is a range of severity in the asymmetric complete/i compl ete/incomp ncomplete lete bilat bilateral eral clef cleftt lip, de-

pending on the extent of soft-tissue bridging and underlying alveolopalatal disjunction. If  a tiny cutaneomucosal band on the incomplete side pulls over the premaxilla, premaxilla, it is usually best to divide it to allow presurgical centralization of the premaxilla and symmetric repair. If one side is only partially cleft, the complete side should be addressed first. Unilateral dentof den tofaci acial al ort orthop hopedi edics, cs, fol follow lowed ed by a lip lip-nasal adhesion on the complete side, levels the surgical field before simultaneous bilateral nasolabial (and unilateral alveolar) clo-

 

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sure. During the second stage, the surgeon should sho uld emp emphas hasize ize (ov (overc ercorre orrect) ct) repa repair ir on the th e mor more e sev severe erely ly in invol volved ved sid side. e. Fu Furth rtherermore, it is easier to match the “good” side to the “bad” side than vice versa. Complete Bilateral with Intact Secondary Palate 

The rare complete bilateral cleft lip with an in inta tact ct se seco conda ndary palate pal ate astic parti par ticu cular lar chalallenge. len ge. Neith Ne ither erryext extern ernal al isela elasti c tra tract ction ion ch nor dentofacial orthopedics is possible because the premaxi pre maxilla lla is rigi rigidly dly proc procumbe umbent. nt. The There re are two alternatives: alternatives: (1) try to accompl accomplish ish bilateral nasolabial repair over the protruding premaxilla; ill a; or (2) perf perform orm a prem premaxil axillary lary-vom -vomerin erine e ostectomy and positioning and bilateral gingi voperiosteoplasty, in conjunction with nasolabial repair. A word of caution: the risk of premaxil ma xillar laryy ne necro crosis sis is rea real. l. Th The e in inci cisio sions ns fo forr premaxillary ostectomy/positioning and alveolar closure impair the premaxillary blood supplyy an pl and d lim limit it ven venou ouss dra drain inag age e to th the e se sept ptal al mucosa and preserved vomerine mucosa. Primary premaxillary positioning in an infant with bilateral complete cleft lip can cause midfacial retrusion. 39,40 Ho Howe weve ver, r, th this is is un unli like kely ly in a child with an intact secondary palate. Other Technical Variations on the Theme of Single-  Stage Repair 

Open rhinoplasty is another way to access and primarily position the subluxed alar cartilages, as first described by Trott and Mohan in 1993.26 Their dissection plane is anterior to the medial crura. The prolabial-columellar unit is pedicled on the dorsal nasal skin and based on the paired columellar arteries.41–43 Their philtral flap is design designed ed to corresp correspond ond to the width of the columellar base. Because the lateral philtral inc incisi isions ons ext extend end acro across ss the col columel umellar lar base, this tissue cannot be used to construct the medial sills; the sills are formed almost entirely  entirely  from the alar flaps. Redundant Redundant skin is removed from fro m th the e lat latera erall la labia biall el eleme ement nts. s. Th The e in inte terrdomal dom al fa fatt is ele elevat vated ed,, th the e mid middl dle e cr crura ura are apposed and secured to the septum, and the soft tissues are gathered to enhance tip projection (Fig. 5). Their postope postoperative rative photographs photographs show normally proportioned columellar length and nasal-tip protrusion. protrusion. The philtra appear to be wi wide de.. Pe Perh rhaps aps th this is is ac acce cept ptab able le in As Asian ian children, who have a slightly broader Cupid’s bow than Caucasian children.44 Distal ischemia is a potential problem if the philtral flap were to be designed smaller using Trott’s method.

FIG. 5. The Trott method method of open approach approach to nasal-tip nasal-tip cartilages.26 The philtral-columel philtral-columellar lar flap (with rim extensions) is turned upward to expose the anterior surface of the dislocated dislo cated middle crura and genua.

Furthermore, this technique requires sutural reconstitution of the columellar labial angle, and this could also impede philtral circulation. circulation. Nakajima and coworkers introduced presurgical and postsurgical molding to minimize the bilateral cleft nasal deformity.45,46 Cutting and associates extended this strategy to preoperative tiv e stre stretch tching ing of the col colume umella. lla. The Theyy fabr fabriicated an acrylic, double outrigger and prolabial bi al ba band nd,, at atta tach ched ed it to a pa pass ssiv ive e pa pala lata tall molding plate, and secured it to the cheeks  with tape.27,47 Their labial markings are similar to those of Trott and Mohan.26 However However,, their open-tip open-t ip approach differs in that the prolabia prolabiallcolumellar flap is elevated at a deeper plane (membranous septum). This dissection is less likely to compromise the vascularity of the distal prolabium. Their placement of interdomal sutures is done from the underside of the alar cartilages (Fig. 6). Cutting and associates underscore that preoperative expansion of nasal lining is as important as columellar elongation because it lessens tension on the interdomal apposition and mimimizes widening of the na48

sal tip. tip They Th emphas emp hasize ize rly that th at the th e mol moldin g prongs pron gs. mus must teypus push h ant anterio eriorly because bec ause ofding the tendency to produce a “turned up” nasal tip. Neit Ne ithe herr th the e Tr Trot ottt no norr th the e Cu Cutt ttin ing g me meth thod odss inclu in clude de cu cuta taneo neous us exc excisi ision on of th the e dom domalalcolumel col umellar lar rims or res resect ection ion of ves vestibu tibular lar lining. R EVISIONS EVISIONS

Symmetry is the major preoperative advantage of a bilateral cleft lip over a unilateral cleft. In part for this reason, the number of  revisions for a double cleft lip should be less than for a single cleft lip. The most common secondary problem is extra mucosa (festooning) in the lateral labial elements. Often this is associated with minor vertical deficiency of the median tubercle. It is best to wait until after the

 

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FIG. 6. The Cutti Cutting ng method method29 of open approach to nasalnasal-tip tip cartil cartilages. ages. The philtr philtral-me al-medial dial crural-columella complex (incised through the membranous septum with extensions into the intercartilaginous ridges) is reflected by retrograde dissection to display the underside of the splayed middle crura and genua.

permanent central incisors have erupted and, if possible, after the premaxilla is in the correct  sagittal position and angulation before adjusting the free labial margin to give proper dental show. There is a litany of procedures for the

abnormallyy wide nose abnormall nose,, requi requiring ring readv readvance ance-ment of the alar bases. Sometimes Sometimes domal divergence must be addressed. The alar cartilages can be readjusted through rim incisions without the need for open rhinoplastic exposure.

“whistling “whistl ing lip” def deformi ormity ty (e.g (e.g., ., V-Y adva advance nce-ments,, double/ ments double/single single Z-plasty, mucosal grafting). Particularly effective is the technique of  deepithe deep itheliali lialized, zed, medi medially ally based subm submucos ucosal al flaps, tunnelled across the midline, to augment  the th e ce cent ntral ral red lip lip..49 If the there re is ins insuff uffici icient  ent  lateral submucosa, a thick dermal graft serves to plump the median tubercle. Any excess mucosa can be trimmed to give a normal contour of the free margin in relation to the central incisors.  An uncommon, annoying problem is prolapse of the posterior wall of the anterior gingivolabi givo labial al sul sulcus cus.. Res Resusp uspens ension ion of the sul sulcal cal mucosa to the premaxillary periosteum is easily  accomplished. Nasal Nas al rev revisi isions ons in chi childh ldhood ood are rare rarely  ly  needed. The most common is correction of an

Final nasal adjustments are done after completion of growth and maxillary advancement (if  necessary) (e.g., tip reduction, nasomaxillary  narrowing, and septal resection). OBLIGAT BLIGATION ION

TO

  PERIODIC  A SSESSMENT SSESSMENT

Not only must surgeons prefigure the rate of  growth of nasolabial features, compounded by  the distortion of the deformity, but they also are obli obligat gated ed to peri periodi odical cally ly asse assess ss the these se changes to learn whether or not the predictions are accurat accurate. e. Photography is the minimal docume doc ument ntati ation on nee needed ded.. Fro Fronta ntall and lat latera erall  views (with the head in a neutral position) are not enough; there must also be a basal view. For the latter, Pigott recommends that the nasal dome be placed well above a line drawn between the medial canthi.50 Lehman suggests

 

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that McComb’s rule be followed in publishing photographs in support of a new method of  cleft lip repair, i.e., show 10 consecutive patien ti ents ts wit with h fo foll llow ow-u -up p of 10 or mor more e ye years ars..51  Although admirable, this rule is unrealistic, particularly if applied to the repair of bilateral cleft lip. Less than 10 percent of cleft lips are bilateral, bilate ral, so even a high-v high-volume olume surgeon might 

the older children. A short cutaneous upper lip is attractive, provided there is sufficient height  of the cen centra trall red lip lip.. Alt Althou hough gh ver vermil milion ion-mucosal height was made full, in one-half of  the older children this dimension was just below the mean. Nev Nevert erthel heless ess,, the total uppe upperr labial height was either normal or slightly more than normal. Admittedly, there are problems

see only a few suchprofessional new patients each Thus, in an average career of year. 25 to 30 years, a surgeon will follow a relatively small number of these children to adulthood.  What is needed is a convenient, objective, and rapid way to evaluate nasolabial symmetry  and proportions throughout the growing years.  Although clumsy, a panel can be convened to assess the photographs, using a rating scale.52 Other methodologies are direct anthropometry and computer-aided photogrammetry (indirect anthropometry). There is great potential for laser scanning or a similar advanced technology to assess results.53,54 This technology will likely incorporate the soft-tissue landmarks used in medica medicall anthropometry, anthropometry, so until it bec becomes omes avai availabl lable, e, ant anthrop hropomet ometry ry can be done the old-fashioned way, by handheld vernier caliper.16,55,56 Intraoperative anthropometry provides baseline values for subsequent documentation of  changes in the nasolabial dimensions. This was done don e for 45 con consec secuti utive ve inf infant antss und underg ergoing oing single-stage repair of bilateral complete cleft  lip and nas nasal al def deform ormity ity..29 FastFast-growin growing g features, and nasal length and nasal width, were set 88 perc percent ent and 96 perc percent ent,, res respec pectiv tively, ely, shorter than those of age-matched control infants. Slow-growing features, nasal protrusion

 with thisthrough study. First, author’s technique evolved three the technical phases in the study period, from two-stage to single-stage repair—but the principles did not change. Second, the study was serial and retrospective, so the measurements in the three phases cannot  be compared at the same point in time. Third, only on ly 12 of 32 ch child ildren ren ha had d un under dergo gone ne th the e one-sta onestage ge repa repair ir des descri cribed bed her herein ein,, and the their ir mean age at evaluation was 2.5 years.  A proviso for any anthropometric study is that normal scalar measurements do not necessarily equate with normal appearance. Nevertheless, it is the author’s strong impression that a child with measurements within 1 SD of  normal looks better than a child with abnormal  values. Furthermore, inclination of the upper lip influences influences appearan appearance; ce; i.e., a vertical upper lip looks longer than a protrusive lip. Thus, if  the th e pre premax maxill illaa is lin lingua gually lly in incli cline ned, d, whi which ch many are, this causes the upper lip to appear longer than it may be by mensuration. It is dif diffic ficult ult to com compar pare e one sur surgic gical al method with another without a standard way to asses ass esss an anat atomi omicc ou outc tcome ome.. Koh Kohou outt an and d col col-leagues used photogrammetric analysis of two surgeo sur geons’ ns’ res result ults; s; one use used d the Mul Mullik liken en method (group I) and the other used the Trott  method (group II).57 The authors attempted to

and colu columell mellar ar leng length, th, were cons construc tructed ted longer than normal (130 percent and 167 percent, respectively). Because all labial features grow rapidly, these were downsized, with the exception of central vermilion-mucosal height  that was deliberately made full. Follow-up anthropometry required first measurin su ring g a co coho hort rt of no norma rmall ch chil ildr dren en;; th this is 16 showed no differences from the results of the larger samples determined by Farkas and colleagues.28 Thirty-two children with repaired bilateral complete cleft lip were assessed from age 1 to 12 years. 16 Nasal-tip protrusion and columellar height were at the mean or longer. Interalar distance was about 2 SD above the mean.. Cut mean Cutane aneous ous upp upper er lab labial ial hei height ght remained short in children younger than age 5  years but tended to scatter around the mean in

distingui distin guish sh res result ultss att attrib ributa utable ble to met method hod-ologic design from those produced by execution of the particular method. Nasal-tip Nasal-tip projection was above normal with both methods but  more so in group I. Interalar dimension was abnormally wide in both groups. Columellar length, as a proportion of tip projection, approached normal in group I but was short in group II. Philtral width (in proportion to nasal  width) was normal in group I but abnormally  high in group II. An overly wide Cupid’s bow in group II was attributed to design. Photogrammetry Photog rammetry also permits the determ determiination of nasolabial angle and nasal-tip angle, provided the head is not rotated. Kohout and associates found that the nasolabial angle was obtuse in early childhood but narrowed in late childho chi ldhood od and adol adolesc escenc ence, e, pres presumab umably ly be-

 

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cause of an increase in upper labial and columellar mel lar obli obliqui quity. ty. Nas Nasal-t al-tip ip ang angle le was blun blunted ted after aft er bot both h the Mull Mullike iken n and Trot Trottt met method hods; s; there were no long-term measures of possible change in the slow-growing lobule.57

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Older children with bilateral cleft lip con-

Boorman goes further by raising the question of whether the rarity of the bilateral deformity  and its att attend endant ant prob problems lems might argu argue e for 62 further control of referral. Such sweeping regulations would be unlikely  in America’s decentralized health-care system,  where change occurs slowly and, usually, voluntarily. Parameters for cleft care have been

tinue walkergone through they doors of cleft centers having hav ingtound underg one man many procedu proc edures res and requiring more. They are unhappy because of  their appearance and functional problems, but  the patterns of cleft care are changing. Fewer general plastic surgeons take on primary cleft  lip repair. Two possible reasons for this trend are (1) est establi ablishe shed d pos postgr tgradu aduate ate fel fellows lowship hip training in pediatric plastic surgery, and (2) increas inc reased ed num number ber of cra craniof niofacia aciall team teamss and opportun oppo rtunitie itiess for pedi pediatri atricc plas plastic tic surg surgeons eons.. Remarkably, this increased focus on care by a few is occurring despite diminished reimbursement for cleft work. Outcome analyses in other pediatric surgical specialities specia lities underscore the value of subspe subspecialcialization and high-volume operators. For example, there are lower inpatient costs and fewer complication compli cationss if uretero ureteroneocyst neocystostomy ostomy is done by fello fellowship wship-train -trained ed pedia pediatric tric urolog urologists ists as 58 compared with general urologists. For pediatric cardiac surgeons, there is an inverse relationship tionsh ip between the annual number of procedures (or a surgeon’s case load) and inpatient  mortality.59,60 In these examples, the determinant of outcome is indisputable; the results of  cleft treatment are more difficult to assess. Surely every infant born with cleft lip/palate, particularly a complete deformity, deserves the

est establ ablish ished ed Association by th the e Ame Americ rican an Cl Cleft atee63 eft Pal Craniofacial (ACPA). ThisPalat organization also surveyed North American teams and dete determine rmined d stan standards dards-of-of-care care deli delivery. very.64 Of 220 responding teams, the mean number of  new cleft lip cases seen per year was 17. However, 37 percent of U.S. and Canadian centers reported repo rted perf performi orming ng few fewer er tha than n 10 prim primary  ary  labial repairs each year. These numbers would be even lower with more than one plastic surgeon on a team. The ACPA evaluation did not  address the level of activity necessary to maintain tai n com compet petenc encyy or pro provid vide e opt optima imall car care. e. These issues are on the agenda of the Craniofacial Outcomes Registry, which is supported by a grant from The National Institute of Dental and Craniofacial Research. This program is being bei ng con condu duct cted ed wit with h th the e coo coope perat ration ion of   ACPA and its members. Prospective data, collected, and centralized by the Craniofacial Outcomes Registry, will permit inter-team inter-team comparison is onss of ou outc tcom ome, e, th the e pu purp rpos ose e be bein ing g to promote higher-quality care in the future. In the mea meant ntime ime,, th the e tra tradit dition ional al ref referr erral al lines for a newborn with cleft lip/palate are changing at the grassroots level. Rather than a pediatrician, pediatr ician, the prenat prenatal al ultrason ultrasonographer ographer or perinatologist is increasingly the first to guide parents to a plastic surgeon. Savvy parents of-

care of an experienced team. Scandinavia has the longest tradition of centralized cleft care, beginning in 1933. There is only one center in Denmark, two in Finland, two in Norway, and six in Sweden. The six-center Euro Cleft Study  showed that standa standardizati rdization, on, centra centralizati lization, on, and part partici icipat pation ion of hig high-v h-volu olume me ope operat rators ors  were  wer e ass associa ociated ted with goo good d outc outcome omess (and 61 fewer revisions). In the United Kingdom, the “threshold “thresh old volume” for primary cleft repair has recently been mandated, the result of an outcome assessment of the country’s cleft centers under the auspices of the National Health Ser vice. The investigating committee concluded that the number of cleft units should be reduced drastically and that children should be sent only to teams composed of two surgeons, each   seeing 40 to 50 new patients annually. 62

ten seek info informat rmation ion (alb (albeit eit non– non–peer peer-re-re viewed) in medical cyberspace, and parental network net workss advi advise se the these se “In “Intern ternaut auts” s” wher where e to searc sea rch. h. Hav Havin ing g co comp mple lete ted d a cr cram am cou cours rse e in cleft care, parents are likely to demand to take their infant to a cleft specialist outside their insurance network. Countercurrent to this rising ti tide de of par paren ental tal sel selff-ref referr erral al sta stand nd th the e health heal th main mainten tenanc ance e orga organiz nizati ations ons tha thatt ins insist  ist  that all children stay within the network and accept acc ept ass assign ignmen mentt to the their ir gen general eral pla plasti sticc surgeon. Rather than take up arms against third-party  payers, why not join them? Consider collaborative outcome studies. One hypothesis would be that primary repair of a cleft lip in a restrictive insura ins uranc nce e set setti ting ng is pen pennyny-wis wise e and pou poundndfoolish. Perhaps if initial closure were done by 

EPILOGUE

 

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sub subspe specia cialis lists, ts, the there re wou would ld be few fewer er pro procecedures and revisions, and decreased costs to all parties. It is easy to document efficacy in terms of speech, dentition, and facial growth. Standardized evaluation of aesthetic results should soon be possible using advanced technology, such as laser scanning. A child’s happiness and paren pa renta tall ac acce cept ptanc ance e co could uld be as asses sesse sed d by a 65

quality-of-life  Who shouldoutcome care for study. an infant with cleft lip?  At a societal level, this question exemplifies the larger issue of what we want our health-care syst sy stem em to be— be—an and d fo forr wh whom. om. Bu Butt th the e sa same me question also could be asked by the surgeon called to see a newborn with cleft lip. Remember the golden rule of pediatric care:  Do for the  child what you would want done for your own .  John B. Mulliken, M.D.  Division of Plastic Surgery  Children’s Hospital  300 Longwood Avenue  Boston, Mass. 02115  [email protected] 

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15. Millard, Millard, D. R., Cassisi, Cassisi, A., and Wheele Wheeler, r, J. J. Desig Designs ns for correction and camouflage of bilateral clefts of the lip and palate.  Plast. Reconstr. Surg.  105: 1609, 2000. 16. Mulliken Mulliken,, J. B. Bil Bilate ateral ral comple complete te cleft lip and nasal deformity: An anthropometric analysis of staged to synchronous repair.  Plast. Reconstr. Surg.  96: 9, 1995. 17. Pigott Pigott,, R. W. Aesthe Aesthetic tic conside consideration rationss related related to to repair repair of bilateral cleft lip nasal deformity. deformity.  Br. J. Plast. Surg. 41: 593, 1988. 18. McComb, McComb, H. Pri Primar maryy repair of the bilater bilateral al cleft lip 19. 20. 21. 22. 23. 24. 25.

nose:: A 15 nose 15-y -yea earr re revi view ew anda ne new w tr trea eatm tmen entt pl plan an.. Plast. Reconstr. Surg.  86: 882, 1990. Mullike Mull iken, n, J. B. Pri Princi nciple pless and tec techni hnique quess of bil bilate ateral ral com com-plete cleft lip repair.  Plast. Reconstr. Surg.  75: 477, 1985. Pensler, Pensle r, J. M., and Mulli Mulliken, ken, J. J. B. The cleft cleft lip lowerlower-lip lip deformity.  Plast. Reconstr. Surg.  82: 602, 1988. McComb, H. Pri McComb, Primar maryy repair of the bilater bilateral al cleft lip nose.  Br. J. Plast. Surg.  28: 262, 1975. Mulliken, Mulli ken, J. J. B. Corr Correctio ection n of the the bilatera bilaterall cleft cleft lip nasal deformity: Evolution of a surgical concept.  Cleft Palate  Craniofac. J.  29: 540, 1992. McComb, McCo mb, H. H. Prim Primary ary repair repair of of the bilate bilateral ral cleft cleft lip lip nose: nose:  A 10-ye 10-year ar revi review. ew. Plast. Reconstr. Surg.  77: 701, 1986. Broadb Bro adbent ent,, T. R., and Woolf, Woolf, R. M. Cle Cleft ft lip nasal nasal dedeformity.  Ann. Plast. Surg.  12: 216, 1984. McComb, H. Pri McComb, Primar maryy repair of the bilater bilateral al cleft lip nose: nos e: A 4-ye 4-year ar rev review iew.. Plast. Reconstr. Surg. 94 94:: 37 37,, 19 1994 94..

1. Brown Brown,, J. B., McDowell McDowell,, F., and Byars, Byars, L. T. Dou Double ble clefts of the lip.  Surg. Gynecol. Obstet.  85: 20, 1947. 2. Mulli Mulliken, ken, J. J. B. Repai Repairr of bilater bilateral al complete complete cleft lip and and nasal deformity: State of the art.  Cleft Palate Craniofac.  J.  37: 342, 2000. 3. Manch Mancheste ester, r, W. M. The repair repair of double double cleft cleft lip lip as part  part  of an integrated program.  Plast. Reconstr. Surg.  45: 207, 1970. 4. Glo Glover ver,, D. M., and Newcom Newcomb, b, M. R. Bil Bilate ateral ral cleft cleft lip lip repair rep air and the flo floatin ating g pre premax maxill illa. a. Plas Plast. t. Recon Reconstr. str. Surg Surg.. 28: 365, 1961. 5. Milla Millard, rd, D. R., Jr. Closu Closure re of of bilateral bilateral cleft lip and and elonelongation gat ion of col colume umella lla by two ope operat ration ionss in inf infanc ancy. y. Plast. Reconstr. Surg.  47: 324, 1971. 6. Duffy, M. M. Restor Restoration ation of orbicu orbicularis laris oris muscl muscle e concontinu ti nuit ityy in therepa therepair ir of thebila thebilate tera rall cl clef eftt li lip. p. Br. J. Plast. Surg.  24: 48, 1971. 7. Randa Randall, ll, P., P., Whitaker Whitaker,, L. A., A., and LaRos LaRossa, sa, D. The impor impor-tance of muscle reconstruction in primary and secondary cleft lip repair.  Plast. Reconstr. Surg.  54: 316, 1974. 8. Rehrma Rehrmann nn,, A. Co Const nstru ruct ctio ion n of the upper upper li lip, p, co colu lu-mella, mel la, and orb orbicu icular laris is mus musclein clein bil bilate ateral ral cle clefts. fts. J. Max-  illofac. Surg.  3: 2, 1975. 9. Millard, D. R., Jr.   Cleft Craft: The Evolution Evolution of Its Surgery,  Vol. 2. Boston: Little, Brown, 1977. 10. Black, P. W., and Scheflan Scheflan,, M. Bilate Bilateral ral cleft cleft lip lip repair: repair: Putting it all together.  Ann. Plast. Surg . 12: 118, 1984. 11. Noord Noordhof hoff, f, M. S. Bil Bilate ateral ral cle cleft ft liprecon lipreconstr struct uction ion.. Plast. Reconstr. Surg.  78: 45, 1986. 12. Croni Cronin, n, T. D., and and Upton, Upton, J. Length Lengthening ening of the short  columella associated with bilateral cleft lip.  Ann. Plast. Surg.  1: 75, 1978.

26.. Trot 26 Trott, t, J. A.,and Moha Mo han, n, N. Aat prel pr elim imin inar aryyofre repo port rt on on one stage open tip rhinoplasty the time lip repair ine bilateral cleft lip and palate: The Alor Setar experience.  Br. J. Plast. Surg.  46: 215, 1993. 27. Cutting Cutting,, C., Grayson, B., Brecht, L., Santiago, Santiago, P., Wood, R., and Kwon, Kwon, S. Presu Presurgical rgical colume columellar llar elongati elongation on and primary retro retrograde grade nasal recon reconstructi struction on in onestage bilateral cleft lip and nose repair.  Plast. Reconstr. Surg.  101: 630, 1998. 28. Far Farkas kas,, L. G., Pos Posnic nick, k, J. C., Hreczko Hreczko,, T. M., and Pro Pron, n, G. E. Grow Growth th patter patterns ns of of the nasol nasolabial abial regi region: on: A mormorphometric study.  Cleft Palate Craniofac. J.  29: 318, 1992. 29. Mul Mullik liken, en, J. B., B., Burvin, Burvin, R., R., and Farkas, Farkas, L. G. Rep Repair air of  bilateral complete cleft lip: Intraoperative nasolabial anthropometry.  Plast. Reconstr. Surg . 107: 307, 2001. 30. Georgia Georgiade, de, N. G., and Latham, Latham, R. A. Max Maxill illary ary arch arch alignment in bilateral cleft lip and palate infant, using pinned coaxial screw appliance.   Plast. Reconstr. Surg. 56: 52, 1975. 31. Geo Georgi rgiade ade,, N. G., Mason, Mason, R., Riefko Riefkohl, hl, R., et al. Pre Preop op-erative positioning of the protruding premaxilla in the bilatera bila terall cleftlip patie patient. nt. Plast. 1989. 9. Plast.Reconstr.Surg. Reconstr.Surg. 83:32, 198 32. Millar Millard, d, D. R., Jr., Jr., and and Latham, Latham, R. A. Impro Improved ved prima primary  ry  surgical and dental treatment of clefts.  Plast. Reconstr. Surg.  86: 856, 1990. 33. Mil Millar lard, d, D. R., Latham, Latham, R., Huifen, Huifen, X., X., et al. Cle Cleft ft lip and palate treate treated d by presu presurgica rgicall orthop orthopedics edics,, gingi gingi- voperiosteoplasty, and lip adhesions (POPLA) compared with previous lip adhesion method: A preliminary study of serial dental casts.  Plast. Reconstr. Surg. 103: 1630, l999. 34.. Ross 34 Ross,, R. B. B.,, an and d Ma MacN cNam amer era, a, M. C. Ef Effe fect ct of pr pres esur urgi gica call infant orthopedics on facial esthetics in complete bilateral cleft lip and palate.  Cleft Palate Craniofac. J.  31: 68, 1994.

13. Cronin, Cronin, T. D. Leng Lengtheni thening ng colume columella lla by by use of skin skin from nasal floor and alae.  Plast. Reconstr. Surg.  21: 417, 1958. 14. Mil Millar lard, d, D. R., Jr. Col Colume umella lla length lengtheni ening ng by a for forked ked flap.  Plast. Reconstr. Surg.  22: 454, l958.

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Self-Assessment Examination follows on  page 195.

 

Self-Assessment Examination Primary Repair of Bilateral Cleft Lip and Nasal Deformity  by John B. Mulliken, M.D. 1. ALL THE FOLLOWING ARE COMMON STIGMATA STIGMATA AFTER TRADITIONAL REPAIR OF BILATERAL BILATERAL CLEFT LIP,   EXCEPT:  A) Slumped genu B) Elevated alae nasi C) Bowed philtru philtrum m D) Proclined premaxilla premaxilla E) Thin median median tubercle tubercle 2. THE FOLLOWING FOLLOWING ARE CRITI CRITICISM CISMS S OF THE FORK FORKED ED FLAP PROCE PROCEDURE DURE FOR COLUMELLAR  COLUMELLAR  ELONGATION,   EXCEPT:  A) Scars at columellar-labial angle B) Inade Inadequate quate columellar columellar length C) Wide philtral philtral scars scars D) Abnor Abnormal mal columellar columellar shape E) Medial crura at nostril-lobular nostril-lobular junction 3. PRINCIPLES PRINCIPLES FOR PRIMARY REPAIR REPAIR OF BILAT BILATERAL ERAL COMPLETE COMPLETE CLEFT LIP AND NOSE INCLUDE INCLUDE THE FOLLOWING,  EXCEPT:  A) Maintain symmetry  B) Secur Securee prima primary ry muscular continuity  continuity  C) Desig Design n prope properr prola prolabial bial size and configuration configuration D) Construct median tubercle from prolabial vermilion-mucosa vermilion-mucosa E) Form columella columella and nasal tip by positioning positioning alar cartilages cartilages 4. ALL OF THE FOLLOWING ARE POTENTIAL ADVANTAGES OF PRESURGICAL PRESURGICAL ORTHOPEDICS,  EXCEPT:  A) Permit gingivoperiosteopl gingivoperiosteoplasty  asty  B) Correct protrusive basal premaxilla premaxilla C) Facilitate primary nasal nasal correction D) Stabi Stabilize lize dental arch arch E) Allo Allow w proper design design of philt philtrum rum 5. ALL OF THE FOLLOWING ARE FAST-GROWING NASOLABIAL NASOLABIAL FEATURES,  EXCEPT:  A) Interalar width B) Colume Columellar llar length length C) Cutane Cutaneous ous upper lip height height D) Cupid Cupid’s ’s bow width E) Medi Median an tubercle tubercle 6. WHICH WHICH OF THE FOLLO FOLLOWING WING NASOLABIAL NASOLABIAL FEATURES FEATURES IN A CHILD WITH REPAIRED REPAIRED BILATERAL BILATERAL COMPLETE CLEFT LIP IS LEAST LIKELY TO APPROXIMATE THE AGE-MATCHED NORMAL MEASUREMENT?  A) Interalar width B) Nasal protrusi protrusion on C) Colume Columellar llar length length D) Width of Cupid’s Cupid’s bow  E) Total upper upper lip height height

 

CONSTRUCTED CONTRARY TO ITS PREDICTED 7. WHICH OF THESE NASOLABIAL FEATURES SHOULD BE CONSTRUCTED GROWTH RATE?  A) Nasal-tip protrusion B) Colume Columellar llar length length C) Inter Interalar alar width width D) Cupid Cupid’s ’s bow  E) Medi Median an tubercle tubercle 8. WHICH WHICH IS THE EASIE EASIEST ST AND MOST AVAILABLE AVAILABLE METHODOLOGY METHODOLOGY TO ASSES ASSESS S NASO NASOLABIA LABIAL L FEATU FEATURES RES  AFTER REPAIR OF BILATERAL CLEFT LIP?  A) Anthropometry  B) Computer-aided photogrammetry (indirect (indirect anthropometry) C) Laser surface surface scanning scanning D) Panel evaluatio evaluation n E) Digital three-dimensional three-dimensional photography  9. WHICH WHICH (OR WHO) IS LEAST LIKELY TO GUIDE PARENTS PARENTS OF AN INFANT WITH A BILATERAL COMPLETE COMPLETE CLEFT LIP TO A SUBSPE SUBSPECIALI CIALIST ST PLASTIC SURGEON?  A) American Cleft Palate-Craniofacial Association B) Health maintenance organization C) Gener General al plastic surgeon surgeon D) Inter Internet net E) Anoth Another er parent parent

To complete the examination for CME credit, turn to page 283 for instructions and the response form.

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