Cleft Palate
by Edward A. Luce, MD
A 4-month old cleft palate patient who has failed to gain weight and has intermittent episodes of “turning
blue.” He has a normal cardiac work-up.
Clinical Examination
The clinical picture is consistent with the Pierre Robin syndrome of retrognathia, glossotosis, and upper airway
obstruction as well as a cleft palate, which is not a part of the original description of the syndrome. Other
possibilities exist in a differential diagnosis such as infantile tracheomalacia, which must be ruled out, but for the
purposes for this discussion a diagnosis of Pierre Robin syndrome will be assumed. The pertinent history would
further detail the failure to thrive as well as the particular details of the cyanotic episodes. Physical examination
would focus on other congenital anomalies of the head and neck. Stickler is a common syndrome in association
and others include velocardiofacial and Nager syndromes. The cleft of the palate is usually quite wide and ushaped which can create some difficulties in operative repair.
Pathologic Anatomy
In normal embryologic palate formation, the tongue is positioned between the 2 vertically oriented palatal
shelves. As the mandible grows, the tongue is permitted to move anteriorly, which in turn will permit the palatal
shelves to swing into a horizontal position and fuse in the midline. The conventional hypothesis in Pierre Robin
syndrome is that a retrognathic mandible interferes with tongue descent, which in turn does not mechanically
permit horizontal orientation of the palatal shelves.1 The functional pathophysiology is that the infant is unable
to maintain a patent upper airway because of the glossoposis. A spectrum exists from the severe presentation
of obstruction at birth through clinical pictures such as the one given, namely a failure to thrive later in infancy
with intermittent episodes of obstruction.
Appropriate Treatment Options
In this baby, a stepwise approach of treatment measures of an increasing interventive nature will help determine
the optimal treatment. Conservative means such as prone positioning of the baby with oxygen supplementation
as is needed may suffice, although unlikely in this scenario. Pulse oximetry monitoring can also be used during
sleep.
Historical methods of treatment have been of a tongue lip adhesion or tracheostomy. The contemporary
management is mandibular distraction.2
The treatment plan has been successful and the child is now 1-year of age and ready for palate repair.
Anatomy Of The Velopharynx
The 6 muscles that are attached to or contained within the palate and the velopharyngeal mechanism are the
levator and tensor veli palatini, superior pharyngeal constrictor, palatopharyngeus, which are paired structures
and the midline uvulus muscle.
The tensor veli palatini originates on the lateral wall of the pharynx and wraps around the pterygoid hamulus and
inserts into the palatal aponeurosis at the posterior border of the hard palate. The tensor aerates the middle ear
by opening the eustachian tube orifice. The tensor veli palatini may play a role in production of tenseness of the
soft palate prior to contraction of the levator sling. Dysfunction of the tensor palatini produced by the abnormal
course and insertion will result in failure of the eustachian tube orifice to open and the middle ear is not properly
aerated. This dysfunction leads to serous otitis media followed by purulent otitis media and ultimately hearing
loss. The treatment of choice is equilibration or pressure equalization tubes placed through the tympanic
membrane. In cleft palates, the levator veli palatini is not in the normal anatomical configuration, namely
horizontal, but rather is abnormally anteriorly inserted on the posterior and medial border of the hard palate as
well as the palatal aponeurosis.
The levator muscle is principally distributed in the middle half of the velum or soft palate as measured from the
posterior nasal spine to the tip of the uvula. The levator functions to elevate the soft palate and move the soft
palate posteriorly and superiorly to appose to the posterior pharyngeal wall during speech. The
palatopharyngeus lies within the posterior tonsilar pillar and splits into 2 heads within the lateral aspect of the
velum and the levator courses between those 2 heads. Laterally, the palatopharyngeus blends with the superior
pharyngeal constrictor on the lateral pharyngeal wall. The superior pharyngeal constrictor appears to be the
principal muscle for lateral pharyngeal wall movement, although the palatopharyngeus contributes in a hemisphincteric mechanism.
The 3 principal operative procedures employed for cleft palate repair are the Von Langenbeck, the V-Y, and
Furlow double-Zplasty. The more common procedures are V-Y and Furlow palatoplasty. The dilemma about
the appropriate timing of cleft palate repair is that the palate needs to be repaired so that an intact
velopharyngeal mechanism is available for the development of correct speech, probably no later than the age of
9-12 months. The repair of the palate at a very early age creates raw areas that must heal by secondary
intention and scarring and may have higher probability of late maxillary deformities.
The essential or major steps in palate repair in both the Von Langenbeck and V-Y are the following:
 In both repairs (the V-Y repair is often referred to as the Wardill-Kilner) through lateral palatal incisions
undermining of the mucoperiosteal flaps as well as freshening of the edges of the cleft is
accomplished to allow for a 2 layer repair.
The V-Y repair differs in that the incisions are joined anteriorly to create 2 posteriorly based mucoperiosteal
flaps. In this repair the flaps are dissected from anterior to posterior to expose the palatine vessels at their
emergence from the greater palatine foramina.
Palatal repairs also share the following:
 Dissection of the anterior abnormal insertion of the levator muscle off the aponeurosis at the posterior
border of the hard palate and performance of an intravelar veloplasty, namely a horizontal and
posterior repair and orientation of the levator muscle fibers.
 Many surgeons also employ vomer flaps. The vomer flaps are incised in the midline from anterior to
posterior and superiorly based flaps are raised bilaterally off the vomer. These flaps are sutured end
to end to the adjacent medial border of the nasal mucosa on the hard palate and as far posteriorly as
possible on the anterior portion of the soft palate. The pros are the availability of a second layer of
closure beneath the oral suture line in the hard palate. The cons are a possible disturbance of vomerdriven facial growth.
The Furlow is a double opposing Z-plasty repair. For example, on the left side of the patient the oral flap is a
mucoso-periosteal muscular flap and on the right side the oral flap is mucosa alone. For the nasal flaps, on the
left side, the flap is nasal mucosa alone, and the right side is a musculo-mucosal flap incorporating nasal
mucosa. The result should be the muscles are aligned in a transverse orientation across the soft palate. The Zplasty also lengthens the entire soft palate.
Management
Babies with Pierre Robin sequence or syndrome have a much higher incidence of postoperative respiratory
obstruction after cleft palate repair. Because of this observation, many centers prophylactically place a nasal
trumpet in the operating room at the completion of the procedure. If not, the tongue suture can be used to pull
the tongue anteriorly and a nasal trumpet inserted. In most instances endotracheal intubation will not be
necessary.
References
1. Avery J K, Chiego DJ. Essentials of Oral Histology and Embryology: A Clinical Approach. 3rd ed. New
York, NY: Elsevier Mosby; 2004 .
2. Denny A, Kalantarian B. Mandibular distraction in neonates: a strategy to avoid tracheostomy. Plast
Reconstr Surg. 2002;109:896.
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