Interesting Case Series

Cleft Lip and Palate

John Pang, BS, Justin Broyles, MD, and Richard Redett, MD
The Department of Plastic and Reconstructive Surgery,
The Johns Hopkins Hospital, Baltimore, Md
Correspondence: jbroyle3@jhmi.edu

DESCRIPTION
A 2-week-old infant with unilateral cleft lip and palate (CLP) is brought
to clinic by his parents. He was born full-term and the only associated
abnormality is a small atrial septal defect. There is no family history of
clefting.

QUESTIONS
1. Discuss the embryologic basis of clefting, as well as the epidemiology of cleft
lip alone and cleft palate with or without cleft lip. What environmental factors
cause or reduce clefting?
2. What is the relationship between cleft anomalies and otologic problems?
3. Discuss the optimal time to repair cleft lip.
4. Discuss several approaches to repairing cleft palate and their advantages/disadvantages.
5. What clinical variables increase the risk of postoperative fistulas? How
soon after the initial surgery should the fistula be repaired?

DISCUSSION
With an estimated incidence of approximately 1 per 700 live births, CLP is
quite common. Cleft lip and palate is the most common diagnosis (46%),
followed by cleft palate alone (33%) and then cleft lip alone (21%).1 Approximately 30% of clefts occur as part of a genetic syndrome, and these cases
tend to be either cleft lip alone or cleft palate alone. Less than 15% of CLP
cases are associated with malformation syndromes.2
Embryologic development of the lip and palate is a complex process that begins at approximately 4 weeks gestation. The medial nasal processes contact
the maxillary processes to form the primary palate. Secondary palatal shelves
develop as bilateral outgrowths from the maxillary processes and eventually
fuse together with the primary palate anteriorly, dividing the oronasal space
into oral and nasal cavities. Unilateral and bilateral CLP is nearly always
due to failure of the medial nasal processes to contact the maxillary process.
Occasionally, a fibrous band (known as Simonarts band) is observed across
or attached the cleft, suggesting that occlusive epithelial adhesions can result
in CLP.2
More rarely, medial CLP can occur if the 2 medial nasal processes fail to
fuse in the midline. Oblique facial clefts result from failure of the lateral
olfactory placode to fuse with the medial maxillary process. Lateral facial
clefts result from failure of the maxillary and mandibular processes to fuse.
Median mandibular clefts result when the medial ends of the first pharyngeal
arch fail to fuse, possibly because of abnormal growth centers at the archs
medial tips.3
Maternal smoking and alcohol consumption have been associated with a
higher incidence of CLP. Folate deficiency is also associated with CLP, and
supplemental doses (6 mg/d) have been shown to decrease the risk in some
studies. The folic acid antagonist and antiepileptic phenytoin increase the
incidence 10-fold. Maternal corticosteroid use causes a 3-fold increase in
CLP.2,3
Up to 70% of young children with cleft palate suffer from otitis media with
effusion and a hearing loss greater than 20 dB.4 This may be secondary to
Eustachian tube dysfunction leading to dysregulation of middle ear pressure.
While children without cleft palate tend to overcome otologic difficulties
after the age of 6 years, those with cleft palate tend to be symptomatic up to
12 years of age. Approximately one-third of patients require tympanostomy
tubes and 8% require tympanoplasty and/or mastoidectomy.5
Prophylactic tympanostomy tube insertion at the time of CP repair has not
been shown to produce long-term benefits in speech or language development.
Moreover, studies on patients with CP have shown that their otologic problems
dramatically decrease after the age of 10 years, so their long-term otologic
outcome is quite good.5

Historically, the optimal time for cleft lip repair is accepted to be 10 weeks
of age due to the increased risk of postoperative complications during the
neonatal period. However, there have been major advances in neonatal care
since this view was first established in the mid-1960s, including pediatric airway management, anesthesiology, and postoperative care. Recently, several
studies have demonstrated that neonatal repair of full-term neonates is not
unsafe. In a retrospective study of 99 CLP patients repaired within 28 days of
birth, there were 5 cases of postoperative complications, all for airway-related
issues that were easily managed by a pediatric anesthetist and PICU.6 Possible benefits of early-repair include improved feeding and subsequent benefit
for the parent-child relationship, as well as improved cosmesis. However, a
comparison of 29 early repair patients and 34 late repair patients found no
significant difference in cosmesis.7
For patients with clefting of the hard palate, the optimal time to repair the
palate is recommended by most sources to be before 18 months. Later repair
of the hard palate results in improved anterioposterior development of the
maxillary dentoalveolus, and hard palate repair after pubertal peak velocity
was shown to result in less forward displacement of the maxilla.8 However,
delaying repair beyond 3 years of age severely restricts language development
and produces stereotyped cleft speech characteristics.
There are several approaches to repairing the cleft palate. Three commonly used approaches are the von Langenbeck procedure, the Bardach 2-flap
palatoplasty, and the Furlow double-opposing Z-plasty.
The von Langenbeck procedure is a simple palatal closure. Incisions are
made along the cleft edges and the posterior margin of the alveolar ridge.
Bipedicled mucoperiosteal flaps are then raised and joined in the midline
medially. The main advantages of the von Langenbeck procedure are its
simplicity and the relatively little amount of dissection required to perform
it. Disadvantages include a greater risk of anterior fistulas as well as inferior
speech due to the insufficiently long soft palate.
The Bardach 2-flap palatoplasty is a modified von Langenbeck procedure.
Modifications include dissecting the levator veli palatini muscles off the hard
palate to improve the medial rotation of the mucoperiosteal flaps, reducing
tension. This reduces the incidence of fistula formation to as low as 3.4%. In
addition, a muscle sling is created within the soft palate to improve velopharyngeal function and speech quality.
The Furlow double-opposing Z-plasty is growing in popularity. The Zplasty on the patients left creates a flap of oral mucosa containing the underlying muscle, whereas the Z-plasty on the patients right creates a flap of oral
mucosa only (the muscle is left attached to the nasal mucosa). The 2 musclebearing flaps are transposed posteriorly, while the nonmuscle-bearing flaps
are transposed anteriorly, creating a lengthened velum and a functioning levator muscle sling. The Z-plasty produces excellent outcomes with regard
to speech development, maxillary development, and speech development.

However, it is difficult to perform in wide clefts, so some authors have recommended its use for clefts smaller than 8-mm wide and the Bardach repair
for wider clefts.
Postoperative palatal fistulas are not uncommon, with an incidence reported
to be from 3% to 30%. Most commonly located at the junction of the hard and
soft palate, fistulas may cause the patient to develop articulation difficulties
and food regurgitation into the nose. Cleft size correlates with the risk of
fistula development. In general, palatal fistulas should not be closed within 6
months of the initial surgery. Within this time period, the blood supply to the
local tissue can be tenuous. Therefore, it is preferable to wait until 6 to 12
months after the initial surgery to repair the fistula. Buccal and tongue flaps
may be used to close the fistula if there is inadequate local tissue adjacent to
the fistula for simple closure.

REFERENCES
1. Mathes D. Grabb and Smiths plastic surgery. JAMA. 2008;299(20):2450. doi:10.1001/jama.299.20.
2450-a
2. Dixon MJ, Marazita ML, Beaty TH, Murray JC. Cleft lip and palate: understanding genetic and environmental influences. Nature reviews. Genetics. 2011;12(3):167-78. doi:10.1038/nrg2933.
3. Eppley BL, van Aalst JA, Robey A, Havlik RJ, Sadove AM. The spectrum of orofacial clefting. Plast
Reconstr Surg. 2005;115(7):101e-4e. doi:10.1097/01.PRS.0000164494.45986.91.
4. Flynn T, Moller C, Jonsson R, Lohmander A. The high prevalence of otitis media with effusion in
children with cleft lip and palate as compared to children without clefts. Int J Pediatr Otorhinolaryngol.
2009;73(10):1441-6. doi:10.1016/j.ijporl.2009.07.015
5. Sheahan P, Miller I, Sheahan JN, Earley MJ, Blayney AW. Incidence and outcome of middle ear disease
in cleft lip and/or cleft palate. Int J Pediatric Otorhinolaryngol. 2003;67(7):785-93. doi:10.1016/S01655876(03)00098-3.
6. Harris PA, Oliver NK, Slater P, Murdoch L, Moss ALH. Safety of neonatal cleft lip repair. J Plast Surg
Hand Surg. 2010;44(4/5):231-6. doi:10.3109/02844311.2010.499666.
7. Goodacre TEE, Hentges F, Moss TLH, Short V, Murray L. Does repairing a cleft lip neonatally have
any effect on the longer-term attractiveness of the repair? Cleft Palate-Craniofacial J. 2004;41(6):603-8.
doi:10.1597/03-028.1.
8. Liao Y-F, Cole TJ, Mars M. Hard palate repair timing and facial growth in unilateral cleft lip and palate:
a longitudinal study. Cleft Palate-Craniofacial J. 2006;43(5):547-56. doi:10.1597/05-119.
Pang et al. Cleft Lip and Palate. www.ePlasty.com, Interesting Case, January 30, 2013