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Bilateral Cleft Lip

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					                                 CHAPTER 12


                                 Bilateral Cleft Lip
                                 Joseph E. Losee




1. The patient in question appears to have a cleft of the lip                often with the use of a specialized bottle, while they go
   with or without extension into the palate on both sides                   through the motions of suckling. Infants, with isolated
   of the face. The general management of patients with a                    clefts of the lip with intact palates, are often able to
   bilateral cleft lip is similar to that of a unilateral cleft lip.         generate enough negative pressure to feed normally.
   Many families come to the surgeon and cleft–craniofacial
   team for prenatal consultation and greatly benefit from
                                                                          • Is the infant having respiratory or breathing diffi-
                                                                             culties? More frequently associated with isolated cleft
   this antenatal meeting. Patients are seen soon after birth;               palate, breathing difficulties can occur in children with
   and depending upon the severity of the deformity, may                     micro-retrognathia and Pierre Robin sequence. Infants
   require intervention prior to definitive lip repair. During                with cleft lip (with or without cleft palate) usually do
   the initial consultation with the infant and family, several              not have associated breathing difficulties.
   questions are paramount.
                                                                       2. A complete physical examination should be performed
   • Is there a family history of clefting or other congeni-              to determine whether associated congenital anomalies
      tal anomalies? A thorough history should be obtained
                                                                          (i.e., heart disease) exist. Particular attention should be
      with particular attention paid to any relatives with clef-
                                                                          paid to the craniofacial examination; and the full extent
      ting or congenital anomalies, as well as any prenatal
                                                                          of the cleft and its involvement of the lip, alveolus, and
      conditions that might be related to orofacial clefting.
                                                                          palate determined. Clefts of the lip can be classified as
      The general incidence of clefting found in Caucasians
                                                                          complete (affecting the entire lip), incomplete (affecting
      (1 in 500) and Asians (1 in 400) is higher than that of
                                                                          a portion of the lip with an intact Simonart’s band in the
      African Americans (1 in 1300). The incidence of cleft
                                                                          nostril sill), or forme fruste (affecting the orbicularis oris
      lip (with or without cleft palate) is higher in males;
                                                                          with intact skin).
      while the incidence of an isolated cleft palate is higher
      in females. The increased risk of having a second child             • Is it a complete cleft? If a complete bilateral cleft of
      with a cleft is 4%; and this risk increases to 9% when                 the lip exists, the lateral alveolar segments are often
      there are two affected children in the family. If a par-               widely separated, and the premaxilla is anteriorly dis-
      ent and single sibling have a cleft, the risk for another              placed resulting in a “fly away” premaxilla. A “fly-away
      child with orofacial clefting increases to 17%.                        premaxilla” exists when the midline prolabium (lip tis-
                                                                             sue anterior to the bony premaxilla) and premaxilla
   • Are there associated congenital anomalies? Infants                      protrude anteriorly on a stem of the vomer, as they
      with cleft lip (with or without cleft palate) have asso-
      ciated medical conditions up to 30% of the time.                       have no connections to the more posterior lateral pala-
      Cleft lip (with or without cleft palate) is less likely to             tal shelves. Primary surgical setback of the premax-
      present as part of a larger syndrome, when compared                    illa, practiced historically, is no longer advocated since
      to isolated clefts of the palate. Infants with cleft palate            it has been shown to affect midface development. In
      alone have nearly a 10% risk of carrying the 22q.                      addition, a complete cleft of the lip and nose results in
      11 deletion of Velocardiofacial syndrome, as well as an                a flat nose lacking projection, a short or nonexisting
      increased risk for Pierre Robin sequence and Stickler                  columella, and flared alar bases tethered to widely dis-
      syndrome.                                                              placed pyriform rims. Within the nasal tip, the lower
                                                                             lateral cartilages are also widely displaced. Generally,
   • Is   the infant eating and gaining weight? Children
                                                                             complete clefts of the lip, nose, and palate require pre-
      with orofacial clefts may have initial feeding diffi-
                                                                             surgical infant orthopedics (i.e., nasoalveolar molding)
      culties, requiring extra time and attention; however,
                                                                             prior to definitive repair. Nasoalveolar molding realigns
      it is unusual for them to not ultimately do well. Those
                                                                             the premaxilla and alveolar segments, as well as elon-
      infants with complete clefts of the lip and palate are
                                                                             gating the columella and approximating the lip seg-
      unable to generate a seal and create negative pressure
                                                                             ments, facilitating the definitive lip and nose repair. If
      necessary for successful sucking. Infants with clefts
                                                                             presurgical infant orthopedics is not available or able to
      need to have liquid “dispensed” into their mouths,
                                                                             be obtained, a preliminary surgical “lip adhesion” may

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     be performed, converting the wide complete cleft into             specialist. Maneuvers to improve swallowing include
     an incomplete cleft.                                              upright feeding and use of a wider-bore nipple to
                                                                       improve the flow of milk or formula. Fluids are “dis-
   • Is it an incomplete cleft? If a child is born with an             pensed” in the infant’s mouth as they make the suck-
     incomplete bilateral cleft of the lip and/or palate, the
                                                                       ling motions. It is exceedingly rare for a child with a
     deformity is less severe. This deformity can be repaired
                                                                       cleft to require prolonged nasoenteric feeds or place-
     primarily at approximately 3 months of age without
                                                                       ment of a gastrostomy tube.
     the need for presurgical infant orthopedics.
   • Is the nose distorted? A bilateral complete cleft of the        • For those infants with complete clefts, consultation
     lip and palate will almost always cause distortion of the         with the cleft team’s pediatric dentist or orthodontist
     nose since the premaxilla onto which the columella                should occur as soon as possible. The pediatric dentist
     and septum rest is anteriorly displaced.                          or the orthodontist will evaluate the feasibility of pre-
                                                                       surgical infant orthopaedics (i.e., nasoalveolar mold-
   • Are the dental arches well aligned? A bilateral com-              ing) prior to cleft lip repair. Nasoalveolar molding
     plete cleft of the lip and palate will also usually result        repositions the “fly away” premaxilla, aligns the max-
     in malalignment of the dental arches. In these cases,             illary segments, and molds the nasal cartilages while
     an appliance may be created to mold the segments and              elongating the columella. This will greatly facilitate the
     within the first weeks as a means of facilitating the              definitive cleft lip and nose repair. If presurgical infant
     ultimate repair.                                                  orthopaedics is not available or possible, consideration
3. No particular studies are required prior to surgery, pro-           is given to a preliminary surgical “lip adhesion,” con-
   vided the complete physical examination has not raised              verting the complete cleft into an incomplete cleft,
   any concern about any comorbidity (i.e., heart disease).            prior to definitive cleft lip and nose reconstruction.
   • A thorough examination by a pediatrician must “clear”           • If the history or physical examination identifies any
     the infant for surgery. Prior to the elective lip and nose        associated anomalies, a consultation with the cleft–
     repair, the child should be eating well, gaining weight,          craniofacial team’s medical geneticist is necessary.
     and growing normally. If an associated syndrome is                Further evaluation and testing may be appropriate
     suspected, further studies are often required (i.e.,              (i.e., ophthalmological assessment for suspected Stick-
     renal ultrasound, echocardiogram, ophthalmologic                  ler syndrome in a child with Pierre Robin sequence,
     evaluation). Most cleft surgeons perform the definitive            and routine screening for the 22q.11 deletion of Velo-
     primary cleft lip and nose repair around 3 to 6 months            cardiofacial syndrome in all isolated cleft palates).
     of age (for the full-term infant) when the risk of anes-
                                                                  5. The concept of a “reconstructive ladder” has little impli-
     thetic complications diminishes. However, the time
                                                                     cation in cleft surgery, and the goals of cleft lip and nose
     of surgery may be delayed, particularly if the infant is
                                                                     repair are quite specific. Cleft lip and nose repair can
     undergoing presurgical infant orthopedics. The “rule
                                                                     be conceptualized as “philtral subunit reconstruction,”
     of 10’s” has been offered as a guide or prerequisite
                                                                     reconstructing a symmetric Cupid’s bow. The deformities
     for surgery: 10 kg, 10 g of hemoglobin, and 10 weeks
                                                                     unique to complete bilateral clefts include: (1) splayed
     of age.
                                                                     lower lateral cartilages, (2) a deficient columella and
4. The current standard of care dictates that infants born           poor tip projection, (3) splayed alar bases tethered to
   with orofacial clefts benefit from evaluation in the setting       wide pyriform rims, (4) loss of a philtrum, (5) anteri-
   of a cleft and/or craniofacial team. The input of the team’s      orly displaced premaxilla and displaced lateral maxillary
   professionals, with experience in managing the complex            segments, and (6) orbicularis oris muscle discontinuity.
   care of these unique patients, is important.                      Therefore, the goals specific to bilateral cleft lip and nose
   • Often, infants born with complete clefts experience             reconstruction include: (1) nasal tip reconstruction, (2)
     initial problems with feeding, and these issues may be          creation of a columella from nasal tissue, (3) release
     addressed by the cleft team’s speech and swallowing             and repositioning (overcorrection) of the alar bases,

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                                                                           mined where the lateral lip vermilion “begin to lose
                                                                           their fullness.” The white roll of the new Cupid’s bow
                                                                           is obtained from the lateral lip elements, 2 mm on
                                                                           either side. Extra mucosa is harvested from the lat-
                                                                           eral lip elements to create a “pouting tubercle” for the
                                                                           central lip. The lateral lip advancement flaps are cre-
                                                                           ated 1 to 2 mm longer than the length of the philtral
                                                                           “tie flap.” The lateral lip elements are cut and buc-
                                                                           cal sulcus incisions allow for radical mobilization and
                                                                           advancement to the midline.
                                                                       •   Muscle reconstruction. The orbicularis oris muscle is
                                                                           freed from the overlying skin and underlying mucosa
                                                                           bilaterally. Following repair of the mucosa, the muscle
                                                                           is approximated in the middle, on top of the premax-
                                                                           illa and beneath the neophiltrum. It is sutured to the
                                                                           nasal spine.
Figure 12-1 Schematic drawing highlighting reconstruc-                 • Skin repair. The lateral lip elements are advanced to
tion of a bilateral cleft lip.                                             the new philtrum and deep subcutaneous and cuticu-
                                                                           lar sutures are placed. Great care is taken in placing
     (4) creation of a philtrum from prolabial tissue, placing             these buried sutures so that the skin edges are per-
     the scars along the normal philtral ridges, (5) creation of           fectly coapted. This obviates the need for external
     a Cupid’s bow, and central lip tubercle from lateral lip tis-         cutaneous sutures.
     sue, and (6) reestablishment of a muscular sphincter.           6. The cleft surgeon must have a clear understanding of the
     • Nasal reconstruction. The alar bases, which are                  potential postoperative complications of cleft lip, nose,
        stretched across a wide pyriform rim, need to be com-           and palate repair.
        pletely released and radically mobilized from the facial       • Early postoperative complications include bleeding
        skeleton. They are overcorrected with a narrowing                  and airway compromise from tongue swelling and/or
        cinch suture using permanent material such as nylon                narrowing of the nasal airways. Persistent or signifi-
        or Prolene. Bilateral rim incisions allow for dissection           cant postoperative bleeding should be explored in the
        of the lower lateral cartilages, removal of the fibro-              operating room. Following cleft lip nasal reconstruc-
        fatty tissue interposed between them, and intradomal               tion, swelling may temporarily decrease the nasal air-
        sutures bringing the alar domes together. The soft tri-            ways and affect the obligate nasal breather. Swelling
        angles are judiciously excised and columellar shaping              affecting the tongue or nasal airways can be treated
        stitches are placed.                                               with racemic epinephrine, steroids, and close observ-
     • Philtral reconstruction. The new “tie shaped”                       ation. Early wound complications (i.e., wound dehis-
        philtrum is designed from the prolabial tissues. The               cence or acute infection) are rare events. The treatment
        lateral long edges of the philtrum bow inward slightly.            of minor wound separation is usually conservative.
        Postoperatively, the philtrum will stretch in both the             Significant breakdown can be treated conservatively
        horizontal and vertical dimensions, and is thus created            or with immediate “re-repair” depending upon the
        narrow with future growth in mind. The philtrum is                 etiology (i.e., trauma or infection). Stitch abscesses
        marked 2- to 3-mm wide at the columellar-philtrum                  should be addressed immediately with suture removal
        junction superiorly. The philtrum is made 6 to 8 mm                to prevent a granulating “pimple” that will likely com-
        in height. The low point of Cupid’s bow is marked                  promise the ultimate scar.
        inferiorly, and the height of Cupid’s bow marked 2 mm          • Late     postoperative complications include hyper-
        on either side, for a total width of Cupid’s bow being             trophic scarring which can be managed with frequent
        4 mm on the new philtrum. The remaining skin of the                massage and application of local steroids. Should sig-
        prolabium may be discarded.                                        nificant scarring occur, linear scar contraction might
     • Lateral lip elements. The corresponding high points                 result in secondary deformities (i.e., whistle deformity)
        of Cupid’s bow, on the lateral lip elements, are deter-            requiring revisional surgery.




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                                                    Congenital
                                                      defect
                                                     in the lip


                                                                    Family
                                     Associated
                                                                   history
                                    malformations
                                                                  of clefting




                         Incomplete                                         Complete
                           cleft lip                                         cleft lip



                                                                               Wide
                                                                              palatal
                             Lip
                                                                            arches, fly-
                           repair
                                                                               away
                                                                            premaxilla


                                                                                           Yes


                                                                                                  Presurgical
                                                                                                 nasoalveolar
                                                                                                   molding


                                                                                           No


                                                                                                  Lip repair
                                                                                                     (∼3
                                                                                                  months of
                                                                                                     age)

Algorithm 12-1 Algorithm for management of patients with bilateral clefts of the lip and/or palate.




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                                                                   References
     P RA C T I C A L P E A R LS                                   1. Mulliken JB, Wu JK, Padwa BL. Repair of bilateral cleft
                                                                      lip: Review, revision, and reflections. J Craniofac Surg.
     1. Presurgical infant orthopaedics (nasoalveolar mold-
                                                                      2003;14(5):609-620.
        ing) repositions the “fly away” premaxilla, aligns
        the neonatal maxillary segments, approximates the          2. Mulliken JB. Primary repair of bilaterel cleft lip and nasal
        lateral lip segments, and stretches the columella,            deformity. Plast Reconstr Surg. 2001;108(1):181-194.
        setting the stage for definitive lip and nose repair.
     2. If presurgical infant orthopaedics are unable to be
        performed, a preliminary “lip adhesion” will convert
        the complete cleft into an incomplete cleft prior to
        definitive repair.
     3. The bilateral cleft lip nasal deformity is reconstructed
        with: (1) radical release of the widely splayed alar
        bases and overcorrection with alar base cinch stitch,
        (2) infracartilaginous or rim incisions to dissect the
        fibro-fatty tissue from between the splayed lower
        lateral cartilages, (3) intradomal tip suture recon-
        struction, (4) soft triangle excision and columellar
        shaping sutures.
     4. A narrow philtrum should be created from the cen-
        tral prolabial tissue cognizant that it will grow with
        time.
     5. A natural-appearing Cupid’s bow should be created
        from the white roll of the lateral lip elements.
     6. A “pouting” central lip tubercle should be created
        from the lateral lip element vermilion.
     7. Care should be directed toward accurate recon-
        struction of the orbicularis oris muscle beneath the
        neophiltrum




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