Clefts of the lip, alveolus and palate are the most common congenital malformations of the head and neck and are the second most common congenital malformation of the entire body, trailing only clubfoot in incidence (3).   Great advances have been made in the last 30 years in treatment, but multiple surgical procedures and a lifetime of clinic visits are still required, leading to  emotional and physical stress for the patient and their families. 

        The ideal treatment of the cleft lip, alveolus and palate involves a multidisciplinary team approach (1-5, 12, 15).   Members include a pediatrician who oversees the development of the child;  the palatal surgeon (usually a general plastic surgeon, sometimes an otolaryngologist); the oral maxillofacial surgeon for the development of the dentitition and the palate;  the otolaryngologist for diseases of the ear; the psychologist for both patient family; the speech and language therapist; and the clinical coordinating nurse. 

Epidemiology and Genetics

        Clefts of the palate, alveolus and lip (CLAP) are syndromic or non-syndromic.  Syndromic types are by definition associated with other malformations, and include the Pierre-Robin Sequence, Treacher-Collins Malformation, Trisomies 13 and 18, Apert’s Syndrome, Stickler’s Syndrome and Waardenburg’s Syndrome.  At last count, more than 300 syndromes were associated with CLAP (3,4, 12).  Syndromic etiologies include single gene transmission such as trisomies; teratogenic causes such as fetal alcohol syndrome; or environmental causes such as amniotic band syndrome or maternal diabetes mellitus (3, 12). 

         Non-syndromic CLAP is a diagnosis of exclusion, and is considered to be of multifactorial inheritance with known predicted rates of recurrence(6).      

        An isolated cleft palate (CP) is genetically distinct from an isolated cleft lip (CL) or a combined CLAP.  Cleft lip with or without cleft palate has an incidence of 1:1000 births in the United States and approximately 1:600 births in the United Kingdom, but is different among ethnic groups (3,4).  American Indians have the highest incidence, at 3.6:1000 births, followed by Asians, whites and blacks (0.3:1000).   In contradistinction, isolated cleft palate is the same among all racial groups, with an incidence of approximately 1:2000 births.  The male:female ratio for CL or CLAP is 2:1, whereas for isolated CP it is 1:2 (3).   

Embryology

        The palate is divided by the incisive foramen into the primary and secondary palates, with the primary palate being anterior to the foramen and the secondary palate posterior to the foramen. The primary palate develops between 4 and 5 weeks gestation followed by the secondary palate between 8 and 9 weeks gestation.  The primary palate, lip and alveolus develop as a mesodermal and ectodermal proliferation of the frontonasal and maxillary processes.  A primary cleft is a failure of proliferation, not a failure to meet in the midline.  At no time in the development of the normal primary palate is there a separation (7).   Although there may be an isolated cleft lip without a cleft alveolus, a cleft alveolus is always associated with a cleft lip (2,4). 

        The secondary palate develops as a medial ingrowth of the lateral maxillae with fusion in the midline.  In normal development, therefore, a natural midline cleft exists, which is in contrast to the normal development of the primary palate.   Deformities which prevent midline fusion, such as micrognathia and/or macroglossia, may result in a failure of midline fusion and therefore a secondary cleft palate.

        Clefts of the palate and lip are also classified as complete or incomplete.  Complete clefts of the lip and alveolus involve extension into the anterior nose.  Complete clefts of the secondary palate involve both the hard and soft palates with extension into the nose and exposure of the vomer.  As will be discussed, attachment of the vomer may play a crucial role in the success of palatal repair (2).   An incomplete cleft has a midline attachment, ranging from a mucosal covering to one with musculature attachment to the midline raphe.  The submucous cleft of the soft palate is an extension of the cleft of the secondary palate where mucosa meets in the midline of the soft palate but the velar musculature (levator veli palatini, tensor veli palatini) does not.  The classic findings of a submucous cleft include a bifid uvula, absence of musculature attachment to the midline raphe of the soft palate, and a hard palate notch.

Normal Anatomy

        The upper lip is longer than the lower lip, and is shaped liked a flattened ‘M’.   The lower lip is shaped like a flattened ‘W’.  Cupid’s bow defines the central portion of the upper lip and the apices of the bow join the philtrum.  The nadir bisects the apices.  The lip extends laterally to approximately the lateral limbus of the eye.   Surrounding the lips is the orbicularis oris, the sphincter of the mouth.   The maxilla  has several distinct anatomical areas.  The nasal spine is the anterior projection of the maxilla and alveolus.  The alveolar process of the maxilla surrounds the palate and houses the teeth.  The incisive canal is located posterior to the incisors, and transmits the lesser palatine artery, one of the distal branches of the internal maxillary artery.  Posteriorly and laterally along the palate is the greater palatine foramina, which transmit the greater palatatine artery, a branch of the internal maxillary artery.  The palate itself is formed from the maxilla, the horizontal process of the palatine bone and the pterygoid plates.  The soft palate attaches to the posterior portion of the hard palate and interdigitates with the lateral pharyngeal wall via several muscular attachments.   From the nasopharyngeal to the oral cavity surface, the muscles of the soft palate consist of the palatopharyngeus, the salpingopharyngeus, the levator and tensor palatini, the muscular uvula, the palatoglossus and the superior constrictor muscle. 

        The superior constrictor muscle is the primary sphincter of the pharyngeal phase of swallowing and is responsible for preventing regurgitation into the nasopharynx (velopharyngeal insufficiency, VPI) .  The tensor veli palatini connects from the eustachian tube to the scaphoid fossa of the sphenoid bone and then to the lateral soft palate.  It tenses the palate, but is not believed to play a major role in palatal elevation.   Tubal dilitation from the tensor palatini is probably minimal (13,14).  The levator veli palatini orignates from the bony cartilaginous junction of the eustachian tube and wraps around the hamulus before connecting to the soft palate.  The levataor is responsible for palatal elevation and perhaps tubal dilitation (13).  The salpingopharyngeus is a consistently small muscle with probable minimal effects upon palatal and tubal function (13).      

Cleft Anatomy

        The Unilateral Cleft Lip.  The CLAP can be divided into defects of the lip, alveolus and palate.  The cleft lip is a failure of mesodermal proliferation resulting in complete or incomplete defects.   The complete unilateral cleft lip includes the orbicularis oris muscle, where the medial portion of the muscle attaches to the columella and the lateral portion to the nasal ala cartilage.  The medial vermillion border is usually thin (called the white roll).  An incomplete cleft lip ranges from a mucosal covering to a slight defect in the bulk of the orbicularis muscle which is barely detectable.

        The nasal defect of a unilateral cleft lip is fairly constant.  The ipsilateral lower lateral cartilage is usually flattened and rotated laterally and inferiorly, resulting in horizontal, short appearance.  The columella is short and often bends and dislocates the septum.  The overall appearance is a flattened dome with a wide, horizontal ipsilateral nostril.

        The Bilateral Cleft Lip.  The bilateral cleft lip is similar to the unilateral defect with the exception of a complete absence of orbicularis muscle on the medial aspect (the premaxilla or prolabium).   The prolabium is usually extruded to a varying degree.  The nasal deformity is essentially a duplication of the unilateral defect, with a bilaterally flattened dome, short columella and bilateral horizontal nostrils.  

        Clefts of the Primary Palate and Alveolus.  The primary palate is that portion anterior to the incisive foramen.  A cleft of the primary palate results in a gap from the incisive foramen through the alveolus.  Clefts of this type are always associated with clefts of the lip. 

        Clefts of the Secondary Palate.  Clefts of the secondary palate are a failure of medial growth of the palatal shelves.  Fusion begins at the incisive foramen and progresses posteriorly.  The vomer is midline, with various attachments to the remnant palate.  The defect of the soft palate is failure of midline fusion.  The palatal musculature attaches to the posterior hard palate.   

        There are a wide clinical range of clefts of the secondary palate, from the submucous cleft  to a complete cleft of the hard and soft palate.  The submucous cleft palate is as a midline diasthasis of the velar musculature, a bifid uvula and a notch in the posterior hard palate. 

Classification

        There is no universally accepted classification of clefts, although the most commonly used is the Veau classification, which was described in 1931 (2-4, 7).  Veau Class I is an isolated soft palate cleft;  Class II is a hard/soft cleft palate;  Class III is unilateral cleft lip and palate;  Class IV is a bilateral cleft of the lip and palate.   Most surgeons describe the defect rather than using the Veau system.  For example, a Veau Class III would be described as a unilateral complete cleft of the lip, alveolus, primary and secondary palates.

General Management:  The Team Approach

        Most literature discusses the timing and type of surgical repair of CLAP, and this leads to the perception that CLAP is a process which can be surgically “fixed”.  In reality, it is a lifelong process requiring numerous interventions by a concerted, dedicated team (1-5, 7, 10-12, 15).  The timetable of care is best divided into various life stages.

        The Neonatal Period.  The immediate response to a child born with CLAP is usually one of shock, by both the caregivers and the parents.  It is important for the caregivers to not “whisk” away the child, as this is the most common complaint of parents (4).  The pediatrician is usually the first person consulted, and is ideally suited for counseling in the immediate postpartum period.  After confirmation of the defect,  the pediatrician establishes feeding.  Children with isolated clefts of the alveolus and lip usually feed normally by breast or bottle, as the defect is usually completely filled by breast tissue or bottle.  Children with clefts of the secondary palate, either isolated or in combination with clefts of the lip and primary palate, usually have severe difficulties feeding.  Breast feeding is not possible, as the neonate cannot generate an adequate suck.  A soft bottle with a large opening is usually required, as this fills the palatal defect and requires minimal sucking by the neonate.  The child must feed sitting up, and frequent burping is required.  If the child cannot feed with this method, a palatal prosthesis which spans the defect may be required.  A nasogastric tube is rarely required.  Despite these attempts, more than 25% of CLAP children have feeding difficulties with poor weight gain until palatal repair.

        Presurgical orthopedics (palatal prosthetic plates) may be used in the neonatal period.  Advocates claim the plates mold the palate into a more ideal position, leading to less difficulty feeding and an easier surgical repair.  Others claim they improve facial growth and children require less intervention throughout time (4,8,10).  Unfortunately, none of these claims have been substantiated by long term studies (1,4,8,10).  Regardless, presurgical orthopedics have become standard of care in many institutions (1).       

        Timing and protocols for repair of the lip vary by institution and surgeon (1-4, 7, 9-12, 15).   In the United States, most surgeons follow the “rule of tens” and repair the lip when the baby is 10 weeks old, weighs 10 pounds and has a hemoglobin of 10.    A lip adhesion may be performed prior to definitive lip repair in order to turn a complete cleft lip into an incomplete cleft, thereby placing the palate into a more favorable position and lessening the tension of the final repair.   Final lip repair is delayed 4 months in order to allow maturation of the scar.  Most surgeons in the United States do not perform lip adhesion, believing it leads to undo scarring and is less efficient than presurgical nasoalveolar molding (1-4). 

        Palatal repair is performed anywhere from 6 months onward.  The timing of the repair is controversial, with traditional belief being delay in palatal repair results in improved midfacial growth whereas earlier repair results in better speech (2,3,7,10,12,13,15,17).  Recent studies refute these claims, finding timing of palatal repair does not affect midfacial growth (2,10,11).  In the United States and Europe, most repair the palate between 8 and 12 months (2-4,7,10-11).

        The Toddler Years.  The most important process in the toddler years is the development of adequate speech, although the management of recurrent or chronic ear infections and the assurance of adequate growth are also crucial.    Most children require intervention by more than one specialty, including speech pathology, pediatrics, otolaryngology, the oromaxillofacial surgeon and the cleft surgeon.     

        Adequate speech usually requires speech therapy, and the speech patholgist sees the child frequently until age seven.   The child is trained to speak slowly and forcefully in order to maximize palatal elevation and minimize hypernasality.   Consonants are the most difficult sounds, as they require full palatal lift.  They are sometimes called “cleft errors of speech”.  Despite the extensive training provided, up to 20% of patients still require  pharyngoplasty  to reduce VPI (1,3).    

        A pharyngoplasty corrects VPI, and is performed for intractable cases not responsive to speech therapy.  There are two ways to diagnose VPI which may respond to pharyngoplasty:   modified barium swallow or flexible fiberoptic laryngoscopy.   The modified swallow of  a patient with VPI shows moderate to severe regurgitation.  Fiberoptic laryngoscopy shows good lateral nasopharyngeal wall movement but poor approximation of the soft palate to the posterior nasopharyngeal wall.  It is performed with the patient fully awake (2). 

        If a child with CLAP falls below the 5% in growth, growth hormone deficiency should be suspected (4), as it is 40 times more common in this population.   These children should be evaluated by the pediatrician.

        The Grade School Years.  The most common problems of CLAP children during the grade school years involve orthodontic management, including alveolar bone grafting, and psychological growth (4).

        When a cleft of the alveolus is present, most children have malformed, maldeveloped or congenitally absent teeth (1, 4).   Regular dental intervention is required, and orthodontic manipulation is usually required to attain adequate occlusion. 

        Alveolar bone grafting was developed in the United States in the 1970’s as a means to replace missing alveolar bone, stabilize the alvoelus and improve occlusion.  Cancellous bone is taken from the iliac crest and placed in the alveolar defect.  After three months, the bone is indistiguishable from surrounding bone (4).   Teeth can be orthodontically moved into the new bone, reducing the number and size of required dentures and bridges.

        CLAP children suffer a high rate of depression and poor self esteem (3,4,12).   Child abuse is also more common in this population.  A psychotherapist is essential to ensure adequate social development, and psychotherapy is usually begun during the grade school years (4). 

        The Teenage Years.   The teenage growth spurt is marked by midface retrusion, often dramatically altering appearance.  Palatal repair is likely responsible, but early palatal repair is essential for development of adequate speech.   Finding the ideal timing for palatal repair so that midface retrusion is minimized is currently an area of intense research, with many conflicting studies in the literature (1-2, 5, 7, 10-12, 15).   The belief that palatal repair is responsible for midfacial growth disturbance is supported by the observation that adults with unrepaired palates have a more normal midfacial appearance. 

        The treatment of midfacial retrusion requires maxillary advancement through a combination of LeForte I, II and III osteotomies.  This surgery is usually performed in young adulthood, after growth is complete.  Exceptions are sometimes made in severe cases and the surgery is performed earlier.   It is also essential to address occlusion prior to palatal repair, and this is usually done with orthodontics.  

        Rhinoplasty, when required, is usually the last surgery performed. It is delayed until the later teenage years, when growth is complete.

Surgical Techniques

        The child with CLAP usually undergoes numerous surgical procedures.  The first procedure is repair of the lip, which occurs around 10 weeks.   The palate is usually repaired between 6 and 18 months, with newer studies advocating earlier repair (2).   Pharyngeal flaps, if required, are performed around age 4.  Alveolar bone grafting occurs between ages 9 and 11, followed by definitive orthodontics at age 12-13.   Maxillary advancement and rhinoplasty are delayed until the later teenage years. 

         Unilateral Cleft Lip Repair.  The most common method of repair for the unilateral cleft lip in the United States is the Millard rotation advancement flap (2-4, 12, 15).  The technique is essentially a perialar rotation advancement flap with reapproximation of the orbicularis oris muscle and repositioning of the malrotated nose.  The cosmetic goals include closure of the mucosal defect, creation of  the philtrum and formation of Cupid’s bow.   The nose is also repositioned to a more normal contour. 

        The surgery begins with marking of the lip landmarks.  Point 1 is the normal alar base and point 2 is the high point of Cupid’s bow on the noncleft side.  Point 3 is the midpoint of Cupid’s bow.  Point 4 is the highpoint of Cupid’s bow on the medial side of the cleft and point 5 is the highpoint of Cupid’s bow on the lateral side of the cleft.  Points 6 and 7 represent the perialar incisions on the cleft side.  Point 8 represents the attachment of the perialar advancement flap on the noncleft side.  Advancement should not commence past the normal noncleft philtrum.  If necessary, a backcut may be required to point 9 in order to attain sufficient rotation.  After the mucosal incisions, the lower lateral cartilage on the cleft side is freed.   Scar tissue is removed from the interdomal area to eliminate the bifid tip and to increase tip projection.  The perialar flap is secured to the base of the columella.  The lip is closed in three layers.  The buccal mucosa is closed first, followed by reapproxmation of the orbicularis oris muscle.  The skin is then closed.   Stay sutures over pledgets are sometimes placed over the dome and the lateral alar cartilages (3, 12, 14).  

         Bilateral Cleft Lip Repair.  The bilateral cleft lip repair is a bilateral perialar advancement flap similar to the unilateral Millard flap.   The goals are reapproximation the orbicularis oris muscle, closure of the mucosal defect, creation of a philtrum and repositioning the nose.  The perialar flaps are reapproximated to elevated mucosa of the premaxilla.  

        The surgery begins by marking the landmarks.   Point 1 is the nadir of  Cupid’s bow.  Points 2 and 3 represent the high point of cupids bow.  Points 6 and 8 represent the high points of Cupid’s bow on the lateral side of the clefts.  Points 7 and 9 represent the medial portion of the perialar advancement flaps.  After the mucosal incisions, the flaps are undermined in the supraperiosteal plane.  The flaps are advanced and reapproximated in three layers.  The buccal mucosa is attached to the posterior mucosal flaps of the premaxilla.   The orbicularis is approximated in the midline.   The mucosa is approximated with attention to placement of Cupid’s bow.  The philtrum are created by the suture lines of the advancement flaps (3, 12, 14).

        Unilateral Cleft Palatal Repair..  The most common method of repairing clefts of the palate involve mucoperiosteal flaps based upon the descending palatine artery.  The goals of the surgery are complete isolation of the nasal cleft from the oral cavity and formation of a functional soft palate.    The repair is a two layered palatal and nasal advancement flap which isolates the nose from the oral cavity.  The alveolar cleft is not repaired during this phase. 

        The surgery begins by incising the mucosa on both sides of the cleft along the mucosal junction of the nose and palate.  The incision is carried anteriorly to the alveolus and posteriorly to the uvular remnant.  The incision is carried along the alveolus to the maxillary tuberosity.  A mucoperiosteal palatal flap is elevated and the palatine artery pedicle is isolated.   The nasal cleft mucosa is elevated as is the mucosa covering the nasal septum.  The velar musculature is elevated from its abnormal attachment to the hard palate and rotated horizontally.  The palatal mucosa on the nasopharyngeal side is reapproximated as is the levator musculature.  The nasal and septal mucosa are reapproximated.  The palatal mucoperiosteal flaps are reapproximated in midline from the alveolus to the uvula.  If undue tension is present, further isolation of the vascular pedicle along with dissection in the space of Ernst (pterygoid-superior constrictor space) may give adequate tissue length.  Dissection of the velar tendons from the hamulus or infracturing the hamulus may provide the needed length.   Exposed palate is packed with surgicel (3, 12, 14).

        Bilateral Cleft Palatal Repair.  Bilateral cleft palate repair is similar to unilateral palatal repair.  Bilateral nasal septal flaps are required.   

        Isolated Secondary Palatal Repair.    Isolated secondary cleft palates are repaired with V to Y advancement flaps (described above).   Alternatively, a Z-plasty may be used.  The Z-plasty lengthens the palate but does not reorient the muscles horizontally and may be less physiologic than V-Y flaps (13).   

        Superior Based Pharyngeal Flap.  The superiorly based pharyngeal flap is used to correct VPI when good lateral pharyngeal movement is present.  The goal is to allow good flow of air at rest but to close off the nasopharynx during swallowing.  The flap is a passive obturator of the central portion of the nasaopharynx.  Prevention of VPI requires contraction of the lateral pharyngeal musculature against the pharyngeal flap to close the nasopharynx (13, 14). 

        The surgery begins by splitting the uvula in the anterior-posterior direction and then dissecting the palate in the submucosal plane to create a wide flap.  The palatal flap is retracted and a superiorly based pharyngeal flap is formed.  The inferior portion of the flap is sutured to the horizontally cut soft palate and then the palate is reapproximated.   The open area of the donor site may be closed or left open.  There should be sufficient space laterally to pass an endotracheal tube, which acts as a stent.   The stent may be removed in one to two days (14).  

        Sphincter Pharyngoplasty.  Sphincter pharyngoplasty is designed to prevent VPI and is a newer technique than the superiorly based pharyngeal flap.  The sphincter pharyngoplasty may have dynamic function.  The palatopharyngeus is redirected horizontally.  Redirection prevents the downward traction of the palatopharyngeus upon the palate and also narrows the nasopharyngeal inlet (13).

        The surgery begins by identifying Passavant’s Ridge (the upper edge of the superior constrictor).  The palatopharyngeus  is identified posterior to the tonsil and a superiorly based flap is formed.  A horizontal incision is made in the posterior pharyngeal wall and the flap is inset. 

        Alveolar Bone Grafting.   Alveolar bone grafting is designed to replace missing alveolar bone.  This allows stabilization of the alveolus along with orthodontic movement of teeth into the new bone graft (4). 

        The surgery begins with an incision over the iliac crest.  Care must be taken to stay superior to the anterior superior iliac spine, as this portion tends to have the more critical muscular and tendinous attachments.  The musculature is cleared from the crest in and osteotomies are performed and the superior, inferior and medial portions.   The crest is then opened like a book, with periosteal attachments laterally.  The cancellous bone is removed from the cavity and saved. This is the graft material.  The bony crest is closed and the periosteum reapproximated and bone wax is used to control bleeding.  The wound is dried meticulously and closed in layers.    The alveolar cleft is identified and the cleft mucoperiosteal flaps elevated.   The flaps are divided in midline allowing sufficient tissue to close the nasal and palatal clefts.   Alloderm is used over incision sites. The cancellous bone graft is ground to a fine consistency and packed into the alveolar defect.  The mucosa is closed.   A palatal orthopedic prosthesis is worn to protect the incision site for two weeks.   

        LeForte Osteotomies and Midfacial Advancement.  LeForte osteotomies are the preferred method for advancement of the midface.  A type I osteotomy is all is required, followed by orthodontic realignment. 

        The surgery begins with a sublabial incision and elevation of the maxilla in the subperiosteal plane.  The elevation is continued to the zygomatic arches and the pyriform aperature is isolated.  Osteotomies are performed above the alveolus and through the pyriform aperature.  The osteotomies may be performed in one piece or in numerous pieces.  The more osteotomies performed  the more technically demanding the surgery becomes and the higher the rate of postoperative malocclusion.  The descending palatine artery and the soft tissue of the pterygomaxillary fossa are avoided, as these are the predominant blood supply of the maxilla (14).

Otologic Disease

        Children with cleft palate suffer from a greater incidence of middle ear disease and hearing loss, with the primary pathology being eustachian tube dysfunction due to abnormal insertion of the palatal musculature (3-5, 9-10, 12, 13, 16-17).  An aggressive approach is required, including frequent myringotomy and tympanostomy tube placement (10). 

        Recent studies are challenging these beliefs.  They have found the repaired palatal musculature restores palatal, and therefore eustachian tube, function (13).   Other studies have found the middle ear fluid to be mucoid in nature and high in glycoproteins, making the fluid more viscous and difficult to clear (17).  These studies are consistent with decreasing need for tubes after age 3, including what is believed to be essentially normal middle ear function by the teenage years (5, 10, 17).

        Robinson et al  performed a prospective study of 150 cleft palate children whose palates were repaired between 2 and 18 months.  They found 92% of these children developed chronic otitis media with effusion which required tympanostomy tubes (5).  These findings were supported by a retrospective study by Muntz et al, who found 96% of cleft palate children developed chronic middle ear effusions (17).   Both studies support the decreasing incidence of middle ear disease with age.  Both advocate aggressive management of middle ear disease in this population. 

Presurgical Orthopedics (Baby Palate Plates)

        One of the more exciting – and subsequently disappointing – advances in the treatment of children with cleft palates has been presurgical orthopedics (4, 8). These are palatal plates which adjust the palate and alveolus to a more normal shape.   They are fitted shortly after birth and adjusted on a weekly basis.  When first described more than 20 years ago many believed they would obviate the need for surgery, but this has proven to be untrue.   They have proven to lessen the extent of the surgical procedure.   They are now viewed as adjuvants for surgery (1,4,8).  

        The objective of presurgical orthopedics is to normalize anatomy as much as possible.  Soon after birth a mold of the upper alveolus and palate is taken, and a plate is formed.  The child wears the plate continually, and the orthodontist adjusts the prosthesis every week.  The process usually takes approximately three months, which delays surgical repair of the lip 1-2 months.   The bilateral cleft lip and palate deformity has been shown to be particularly amenable to this process, as the premaxilla and alveolus are repositioned to such an extent as to greatly lessen closure tension during surgery. 

        Grayson et al recently published a series of children where he added nasal molding to the standard palatal and alveolar molding.  He claims to have dramatically improved columellar height and tip symmetry with this process (1). 

Facial Growth and Palatal Repair

        Children with CLAP have midface retrustion by the teenage years.  The cause of the deformity is not known, but has traditionally been ascribed to early palatal and lip repair (1-3, 11-12).  The lip repair, usually undertaken at about 10 weeks, causes posterior pressure on the maxilla, retarding growth.  The same is believed true of palatal repair.  

          Rohrich et al published a long term study comparing timing of surgical closure to facial growth and speech impairment.  Group one averaged repair at 10 months.  Group two averaged repair at 48 months.  Forty four patients were followed.  No difference in facial growth was seen between the two groups, but the older repair group had significantly impaired speech.         

Conclusion and Future Directions

        “Cleft palate” is usually a misleading description.  Most children have defects of the lip, alveolus, palate and nose.  The team approach is preferred, and most children require numerous interventions by many specialties until young adulthood. 

        Most surgeons in the United States repair the lip at 10 weeks and the palate around 10 months.  Ventilation tubes are required in more than 90% of children with CLAP, followed by speech therapy.  Pharyngoplasty, if required, is usually performed around age four.  Alveolar bone grafting is undertaken around age 10, followed in 2-3 years by definitive orthodontic management.  Midfacial advancement and nasal repair are usually delayed until the later teenage years. 

        Presurgical nasoalveolar molding is a newer concept with excellent early results.  Long term data are not yet known. 

                 

References

1.      Grayson BH;  Santiago PE:  Presurgical nasoalveolar molding in infants with cleft lip and palate  Cleft Palate-Craniofacial Journal. 36:486-98, November, 1999.

2.      Marrinan EM; LaBrie RA:  Velopharyngeal function in nonsyndromic cleft palate:  relevance of surgical technique, age at repair and cleft type  Cleft Palate Craniofacial Journal.  35:95-100.  March, 1998. 

3.      Bailey BJ et al.  Head and Neck Surgery-Otolaryngology.  Lippincott-Raven, 1998.

4.      Habel A; Sell D:  Management of cleft lip and palate  Archives of Diseases in Childhood  74:360-364,  1996.

5.      Robinson PJ; Lodge S et al:  The effect of palate repair on otitis media with effusion  Platic and Reconstructive Surgery.  89: 640-645,  1992.

6.      Curtis E. et al.   American Journal of Disabled Children 102:853.   1961.

7.      Millard DR.  The embryonic rationale for primary correction of the cleft lip and palate  Annals Roy Coll Surgeons Eng.  76:  150-60,  1994.

8.      Cutting CB.  Grayson BH et al.  Presurgical columellar elongation in one stage bilateral cleft lip and nose repair.  Plast Reconstr Surg. 101: 630-639.  1998.

9.      Hotz M et al.  Treatment of Cleft Lip and Palate.  Hans Huber Press, 1996.

10.  Nunn DR et al.  The effect of very early cleft palate closure on the need for ventilation tubes in the first years of life.  Laryngoscope.  105: 905.   1995. 

11.  Rohrich RJ et al.  Timing of hard palatal closure:  a long term analysis.  Plast. Reconstr. Surg.  98(2): 236-46.  1996. 

12.  Cummings CW et al.  Otolaryngology-Head and Neck Surgery,  1998. 

13.   Huang MH et al.  A fresh cadaveric study of the paratubal muscles:   implications for eustachian tube function in cleft palate.  Plast.  Reconstr. Surg.  100(4): 833-42.   1997.

14.  Bailey BJ et al.  Atlas of Head and Neck Surgery – Otolaryngology.  Lippincott, 1998.

15.  Lee KJ et al.  Essential Otolaryngology  Head and Neck Surgery Appleton and Lange, 1999.

16.  Hutton DA et al.  Preliminary characterization of mucin from the effusion of cleft palate patients. Otol. Head and Neck Surg.  109:1000, 1993. 

17.  Muntz HR et al.  An overview of middle ear disease in cleft palate children. Facial Plast. Surg.  9: 177,  1993.