CLEFT LIP AND PALATE

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					                              CLEFT LIP AND PALATE

 INCIDENCE/EPIDEMIOLOGY

Clefting in

     1.   U.S. 1:750 births
     2.   Asians 2.1:1000
     3.   Caucasians 1:1000
     4.   Blacks 0.41:1000
     5.   Males: Females 2:1
     6.   Isolated clefts 1:2000 without racial influence
     7.   Isolated palates
     8.   Female:Male 2:1

Site of clefting

   1. Left: Right: Bilateral 6:3:1
   2. Most common on left

ETIOLOGY

   1. Environmental
   2. Genetic

Environmental Factors

   1.    Facial mesenchyme
   2.    Facial width
   3.   Persistent high tongue position during development
   4.   Inadequate shelf force
   5.   Infections (rubella, toxoplasmosis)
   6.   Growth hormone deficiency
   7.   Drugs




   Drugs causing clefting

   1.   Steroids
   2.   Diazepam
   3.   Aminopterin
   4.   Anticonvulsants ( incidence 10X)


                                                             1
      5. Smokers (2X incidence)

Genetics

33% Show family history

EMBRYOLOGY

At 6 weeks

             o  Maxillary process
             o Lateral nasal process
             o Median nasal process
         These three processes join and fuse to form the primary palate
         Current thought is cleft will result with no mesodermal migration across site




7 weeks

             o   Median nasal process and maxillary process have fused creating upper
                 lip and anterior maxillary alveolus




8 weeks

             o   Complex totally fused and mesodermal migration completed
             o   Tongue, which has been postured superiorly between lateral palatal
                 shelves of maxilla, moves inferiorly allowing palatal processes to grow
                 toward midline and fuse, form nasopalatine foramen to uvula




11 weeks

             o   Total palatal closure

TIMING

Lip

Millard "rule of ten’s"



                                                                                       2
                           o    Ten weeks old
                           o    Ten grams Hgb
                            o Ten pounds weight
         Randall advocated repair in first ten days of life,
         as soon as health permitted
         Avoid week 2-6 weeks of life due to ¯ Hgb during this period
         Delay of closure allows parents to fully appreciate defect and the results of
          surgery
         Landmarks more visible as age increases

      Palate

                       Children speak at approximately 18 months
                       Early 1 ° surgery before age 1 yr.
                       Late surgery after age 1-2 yr.
                       Delayed complete palatal repair 12-24 months.
                       Late complete palate repair age 2-5 yr.
                       Early complete palate repair age 3-9 mo.
                       Early soft palate repair 3-9 mo.: then delayed hard palate
                        repair 6-15 mo.
                       Early vomer flap 3-6 mo.; then soft palate repair

      Kaplan:

         3-6 mo. ideal time for repair.
         Data shows that the earlier the palate is repaired
         the sooner the soft palate can begin normal function and
         speech can begin development without abnormal patterns.




      Most important factor in achieving good speech is

         reconstruction of muscular levator mechanism,
         regardless of technique or sequence.
         Most centers in U.S. repair palates by 12-18 mo.

ANATOMY

Lip

         Width of cleft is impressive, but vertical height between cleft and non-cleft
          side is most important.


                                                                                      3
        Principle is to lengthen cleft side of lip to equal vertical height on non-cleft
         side
        Key elements: midpoint cupid’s bow and peaks of cupid’s bow

        Obicularis oris muscle bundles parallel cleft margins and insert abnormally
         into skin and mucosa

        Nasal septum deviated to the side of cleft and base is on no-cleft side

        Alar base widely flared




Palate 4 muscle groups

            1.   Levator veli palatini (LVP)
            2.   Tensor veli palatini (TVP)
            3.   Uvulus
            4.   Palatopharyngeus

Tensor veli palatini (TVP)

   1.    origin = Sphenoid bone and membranous wall of eustachian tube
   2.    Slings around hamulus and
   3.    fuses with contralateral TVP in midline aponeurosis
   4.    Function-stiffens soft palate and opens eustachian tube
   5.    Innervation-Cranial nerve V3

Levator veli palatini (LVP)

   1.    origin = Temporal bone and eustachian tube
   2.    inserts into soft palate posterior to TVP
   3.    Function-elevates soft palate in speech and swallowing
   4.    Innervation-Cranial nerve IX and X

Uvulus

   1.    Arises from palatal aponeurosis and
   2.    posterior nasal spine with vertical fibers to the tip
   3.    Function-elevates uvula
   4.    Innervation-Cranial nerve IX and X

Palatopharyngeus



                                                                                            4
      1.   Two origins on soft palate
      2.   Anterior inferior joining uvulus
      3.   Superior posterior attaching to mucosa on nasal side of palate
      4.   Forms arch of soft palate
      5.   Inserts in pharyngeal wall and posterior rim of thyroid cartilage
      6.   Function-narrow and seal nasal pharynx
      7.   Innervation-Cranial nerve IX and X

CLASSIFICATION

Clefts of lip usually classified as

      1.   narrow,
      2.   wide (>10mm),
      3.   unilateral,
      4.   bilateral

Veau, first to classify clefts

              o   Veau I (A) Cleft soft palate only
              o   Veau II(B) Cleft from uvula to incisive foramen
              o   Veau III (c) Unilateral cleft through soft palate, hard palate,
                  including alveolus
              o   Veau IV(D) Bilateral cleft of alveolus, hard and soft palate, vomer is
                  free




Kernahan "Y"

                  With Millard modification (see diagram)




TECHNIQUES

Lip

4 Fundamental approaches used in U.S.

              1. Lip adhesion




                                                                                           5
           o   Most common in bilateral wide clefts with protrusive premaxilla and
               inadequate tissue for primary repair
           o   Wait six months for remodeling of maxilla secondary to lip tension
               then secondary repair




           1. Straight line repair

           o   Reserve for incomplete clefts and revision of lip scar




           1. Triangular flap (Tennison-Randall)

           o   Requires exact measurements and mathematical design
           o   Can achieve reproducible results in inexperienced hands




Markings

                         o   Base of columella non-cleft side (point 4)
                         o   Crest of cupid’s bow non-cleft side (point 2)
                         o   Midpoint depth of cupid’s bow cleft side (point1)
                         o   Crest of cupid’s bow cleft side (point 3)
                         o   **Distances should be equal (2 to 1and 1 to 3)**
                         o   Base of columella cleft side (point 5)
                         o   Inner aspect of ala cleft side (point 6)




                         o   Depth of philtrum (point 7)
                         o   Point 3 and 7 should be at right angles to
                             mucocutaneous junction
                         o   Distance from 5 to 3 plus 7 to 3 must equal length of
                             opposite lip 4 to 2
                         o   Point 9 is established so 6 to 9 equal 5 to 13
                         o   Point 12 is established so 9 to 12 equals 8 to 12
                         o   Points 10 and 11 are midpoints of transverse line from
                             point 7 to 13 and 9 to 12


                                                                                      6
The only tissue sacrificed is from cleft side at nostril floor




Relaxing incision made in vestibule without disturbing perisoteum

Repair of muscle vital to avoid bulging of tissue lateral to repair




Lengthening of lip corresponds to length of base of triangle (point 8 to 9)




Lengthening of medial lip is achieved by backcut (point 3-7)

           1. Rotation advancement flap (Millard)




                      "Cut as you go"




                      Difficult to master




                      Unsurpassed for incomplete/narrow clefts




                      Markings




                         o   Crest of cupid’s bow non-cleft side (point 1)
                         o   Depth of cupid’s bow cleft side (point 2)
                         o   Crest of cupid’s bow cleft side (point 3)
                         o   Point 1 to 2 equals 2 to 3
                         o   Base of columella non-cleft side (point 6)



                                                                              7
                        o   Base of columella cleft side (point 5)
                        o   Inside base of ala on cleft side (point 7)
                        o   End white roll on lateral side of cleft (point 9)
                        o   Midpoint base of columella (point 4)
                        o   Point 3 to 4 equals 7 to 9
                        o   Development of flap "P" used to reconstruct nasal floor
                            and ala

Palate

Von Langenbeck




          o   Two parallel incisions along cleft to expose a nasal mucosal layer and
              oral mucosal layer and access to muscles and bony edges
          o   Two incisions parallel to alveolus from lateral incisor region to distal
              of tuberosity; further release may be necessary into mucosa crossing
              pterygomandibular raphe
          o   Subperiosteal release of both sides to allow for medial movement of
              flaps
          o   Greater palatine neurovascular bundle is tethered from canal
          o   Nasal mucosa and oral mucosa are sutured in two-layered closure
          o   May incise vomer mucosa and suture nasal mucosa to vomer




Wardill-Kilner Technique (V-Y Pushback)




          o   Modification to von Langenbeck procedure to release palatal length
              for improved function
          o   Release at anterior aspect of von Langenbeck incision
          o   Care should be taken to preserve greater palatine vessels. Osteotomy
              of foramen may be necessary to increase mobility




Furlow Technique (Double-reversing Z-plasty)




                                                                                     8
         o   Procedure for soft palate only
         o   Two Z-plasties: one on oral mucosa and one on nasal mucosa *reverse
             orientation
         o   Reorients muscle fibers of palate
         o   May be combined with von Langenbeck or V-Y Pushback




Kriens

         o   Both von Langenbeck and V-Y pushback muscles are not oriented
         o   Kriens modification with submucosal dissection of muscles on nasal
             side performing three layered closure




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posted:8/23/2011
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