eMedicine Specialties > Plastic Surgery > Craniofacial

Craniofacial, Bilateral Cleft Nasal Repair

Author: Lonny L Ross, MD, Assistant Professor, Section of Plastic Surgery, University of Manitoba; Head of Pediatric Plastic Surgery, Children's Hospital of Winnipeg; Director, Manitoba Centre for Craniofacial Difference and Cleft Palate Program
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Updated: Aug 14, 2007

Introduction

Although surgery for the bilateral cleft lip has undergone many advances, correction of the nasal deformities associated with this congenital malformation remains one of the greatest challenges in plastic surgery. Various single- and multiple-stage procedures have been used. Deformities may become apparent after further growth and development of the nose, making the bilateral cleft lip nasal deformity a 4-dimensional problem.

History of the Procedure

Early attempts to repair the cleft lip nasal deformity in conjunction with the lip component resulted in scarring and corrections that did not last. This led to the beliefs that primary repair may interfere with growth of the nasal cartilages and that the nasal deformity should not be corrected until nasal growth is complete. Soon, evidence began to refute these beliefs, and some have claimed that early surgery may assist growth. Unilateral staged procedures were reported first, and these gave way to bilateral staged procedures. Currently, some authors advocate simultaneous bilateral lip and nose repair.

Problem

Surgical correction of nasal deformities associated with bilateral cleft lip is challenging because deformities may become apparent as the nose undergoes further growth and development.

Frequency

The incidence of cleft lip with or without cleft palate in the United States is estimated to be 1 case in 1000 individuals. This varies by race. Caucasian persons have an incidence of 1.34 cases per 1000 individuals, African American persons have an incidence of 0.41 cases per 1000 individuals, and Japanese persons have an incidence of 2.1 cases per 1000 individuals. Approximately 10-25% of cases are bilateral. The incidence of cleft lip with or without cleft palate is also affected by sex; males are affected in 60-80% of cases.

Etiology

The cause of cleft lip with or without cleft palate is still under debate. In the past, the multifactorial/threshold model was believed to be the best model for transmission of cleft lip and palate. This model describes a threshold at which a phenotype is expressed in an individual based on genetic susceptibility and the environment. It was developed because common mendelian inheritance patterns were not present with familial clusters of clefts and other birth defects. However, more recently, some studies suggest that in some situations, major gene loci may be operating.

Cleft lip can be described as either syndromic or nonsyndromic. Race appears to play an important role in nonsyndromic clefting. Individuals of Asian descent generally have underdeveloped derivatives of the median nasal process (MNP), leading to an increased incidence of clefting, whereas African American persons have well-developed MNP derivatives and experience a rate of clefting one fourth that of Asian persons. Animal studies have also demonstrated that nasal cavity width and epithelial activity in the approaching processes also may be involved. In addition, any factors altering the electron transport chain, including hypoxia, possibly phenytoin, and carbon monoxide, can increase the incidence of cleft lip. An increased incidence of cleft lip also may exist among children with fetal alcohol syndrome.

Approximately 14% of cleft lips, with or without cleft palate, are considered syndromic. Trisomies 13 and 21 both affect MNP development, leading to an increased incidence of clefting. One percent of persons with clefts may present with Waardenburg syndrome, in which neural crest cells fail to develop properly. This syndrome is associated with deafness and patchy pigmentation absence. Another rare cause is van der Woude syndrome, which manifests with lip pits due to abnormal salivary glands. The cleft in this instance may be due to failure of the epithelial lining of the facial processes to regress, preventing fusion.

Pathophysiology

The face develops from 5 processes in the human embryo, the single frontonasal process and the paired maxillary and mandibular processes. These 5 processes consist mainly of neural crest tissue and are derived from the first pair of pharyngeal arches at approximately the fourth week of gestation. The frontonasal process begins to develop median and lateral nasal prominences around an olfactory pit at approximately the 33rd day of gestation. The maxillary processes continue to develop medially toward the median and lateral nasal processes of the frontonasal process. The maxillary processes compress the MNPs, and the epithelium is believed to stretch and break down, allowing the processes to fuse in the normal fetus.

A cleft lip may result through processes described by the classic theory, the mesodermal penetration theory, or, more likely, a combination of both. The classic theory of His postulates a failure of fusion, thought to be more important for the development of clefts of the secondary palate.

The mesodermal penetration theory of Pohlmann and Veau emphasizes the importance of mesodermal migration and support of the fusing structures, without which the epithelial layers break down. This theory may be more important for the development of clefts of the primary palate. Schendel et al investigated the muscle at the site of cleft lips by performing a biopsy, the results of which demonstrated nonneurogenic atrophy and disorganization of the muscle fibers at the cleft margin, yielding a metabolic explanation for poor mesenchymal migration due to abnormal local mitochondria. These findings lend more support to the mesodermal penetration theory at the site of primary palatal clefts.

Presentation

See Relevant Anatomy for a list of defects affecting individuals with bilateral cleft lip nasal deformity.

Indications

The indication for surgery is any visible nasal deformity associated with a bilateral complete or incomplete cleft of the lip with or without clefting of the palate.

Relevant Anatomy

Nasal anatomy

The bony structure of the nasal pyramid includes the 2 nasal bones, the frontal processes of the maxilla, the nasal processes of the frontal bone, and the bony septum. The osseous septum is formed by the vomer and perpendicular plate of the ethmoid. This osseous framework is supplemented by an extensive cartilaginous component also essential for nasal form and function: the paired upper lateral cartilages (ULCs); lower lateral cartilages (LLCs), or alar cartilages; and the cartilaginous septum. The LLCs are made up of 2 crura. One is lateral, providing support to the superior portion of the nostril, and one is medial. The paired medial crura form the internal structure of the columella. The septum adds support to the external structure of the nose by preventing the ULCs and LLCs from collapsing into the face.

The nasal cavity begins at the nares, or nostrils, extending posterior to the choanae. The nasal floor consists of the palatine process of the maxilla and the horizontal plate of the palatine bone. The nasal roof extending toward the choanae also includes the cribriform plate of the ethmoid and the body of the sphenoid. The nasal conchae project from the lateral wall into the cavity. Just inside the nares lies the vestibule, the squamous epithelia-lined caudal portion of the nasal cavity. The medial wall consists of the nasal septum and is composed of membranous, cartilaginous, and osseous components.

The nose, lip, and alveolar cleft components comprise clefting of the primary palate. Clefting behind the incisive foramen comprises the hard and soft palate or secondary palate.

Anatomy of the bilateral cleft lip nasal deformity

The following deformities of the bilateral cleft lip nose have been well described by many authors:

• Shortened columella (apparent vs true)
• Broad flat nasal tip with an obtuse mediolateral crural angle
• Inferolaterally displaced alar dome cartilage (ie, bucket handle)
• Caudally displaced medial crus
• Caudally, laterally, and dorsally displaced lateral crura
• Splayed alar domes (increased separation between the genua, with interposed soft tissue)
• Subluxed LLCs off their overlapping ULCs
• Hypoplastic alar cartilages, accessory cartilages, and lobular fat
• Laterally displaced alar base
• Obtuse alar nasi-cheek angle
• Bridge of tissue (Simonart band) that may span an incomplete cleft (ie, nasal floor deficits)
• Lack of nasal sills
• Elongated and buckled lateral crura (M shape vs normal C shape)
• Drooped flared nostril rim (cat's knees)
• Vestibular web due to protrusion of the caudal margin of the LLC into lateral vestibule
• Lack of flare at the base of the columella
• Splayed alar domes and laterally displaced alar bases leading to nostrils that have a horizontal orientation
• Protrusion of the premaxilla, which contributes to the deformity by decreasing the nasolabial angle (not specifically part of the nose)
• Maxillary hypoplasia, which increases the nasolabial angle
• Paucity of alveolar bone at the cleft, leaving the tip and ala to collapse without a foundation

Recently, some authors have questioned whether the columella is truly shortened or if it appears short due to the splaying of the medial crura. They contend that the columellar tissues reside in the nasal tip and that the distal end of the columella has been unzipped due to the splaying of the medial crura.

The nasal deformity can vary considerably. In patients with bilateral incomplete clefts, little, if any, detectable deformity may be present. In patients with severe bilateral clefting, the deformity may be profound. Clefting of varying degrees may occur on each side, leading to asymmetry of the nasal deformity and rotation of the premaxilla.

Contraindications

Any anomalies that compromise the patient's intraoperative or postoperative course may be contraindications to surgery. Diseases that may interfere with normal growth and development may impact the final repair. The child should undergo a complete physical examination to determine the extent of any concurrent medical illnesses and should be eating and gaining weight normally. Marsh and Martin¹ choose to perform repairs after 8 weeks, when the infant has fully switched to extrauterine circulation and is more tolerant of nasal obstructions. Even a small amount of blood loss is always a concern in surgery of children with very small total blood volumes. This also may help guide surgical timing, even more so for secondary palatal surgery in which larger amounts of blood loss may be expected.

Millard suggests the infant be free of respiratory infection for at least 2 weeks and without any skin infection at the time of surgery. He adds that chest radiographs be obtained and preoperative and intraoperative antistreptococcal antibiotics be used for every patient. A complete blood cell count should demonstrate a hemoglobin value of at least 10 g/100 mL and a hematocrit value of 35%. The rule of tens also applies: the patient should be aged at least 10 weeks, have a hemoglobin value of 10 g/100 mL, weigh at least 10 lb, and have a WBC count of less than 10,000/µL.

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