Abstract

Sir: We report a case of a congenital cleft of the left alar rim that could not be classified using any of the schemes used to name facial clefts. An 8-month-old girl born with a nasal cleft after 37 weeks’ gestation was referred to the Yale Craniofacial Center for evaluation of this deformity (Fig. 1). The decision to repair the cleft was made using a combination of Z-plasty internally along the nasal vestibule/nasal tip and a bifurcation of the flap on the external surface to minimize scarring externally. The patient presented with minimal notching of the alar rim 15 months postoperatively (Fig. 2).Fig. 1.: Preoperative oblique (above) and (below) caudal views of the nasal cleft. Note the full-thickness alar cleft extending from the free border of the alar rim, the nostril asymmetry with a narrowed nostril of the affected side, and the asymmetry of the columella.Fig. 2.: Appearance at 15-month follow-up. (Above) Frontal view of alar rim (and associated notching); (below) left lateral view.Although nasal development is complicated, congenital anomalies are rare, with an incidence of one in every 20,000 to 40,000 live births.1 Despite a variety of schemes to classify facial clefts, the most commonly used is Tessier’s classification. This classification is based on rigid axes that often prove problematic in naming wide-spanning clefts.2 The continuity and the complexity of this cleft suggest that it belongs to what Tessier dubbed “‘renegade’ compound malformations [that] defy classification by the framework” he had proposed. The embryonic appearance of the stomodeum between the fourth and fifth weeks of gestation commences the external development of the nose.3 The neural crest cells, which are the developmental precursors of the nose, begin migrating caudally from the lateral aspects of the frontal prominence to the midface.4 The proliferation of the neural crest cells results in the formation of the nasal placodes which, when covered by ectoderm, transform into the olfactory pits. The invagination of the olfactory pits gives rise to the sides of the nose, the septum, philtrum, and premaxilla of the nose. The globular processes grow posteriorly, becoming the nasal laminae.3 The nasal laminae fuse with the nasofrontal process, forming the nasal septum. The globular processes fuse with the maxillary processes, creating the philtrum and columella. The maxillary processes form the lower region of nasal cavity’s lateral walls and the nares’ posterior portion. We hypothesize that in the fourth and fifth weeks of gestation, the nasal placode and olfactory pit developed aberrantly. The left nasal placode invaginated atypically, creating a gap on the lateral aspect of the placode. Consequently, the mesenchymal nasal ridge lined with ectoderm did not elevate correctly, forming a region in the olfactory pit that was missing the precursors for cartilage and skin. As this olfactory pit invaginated, the gap continued obliquely in a caudal-posterior fashion, creating this cleft. Because the gap had neither mesenchymal nor ectodermal tissue, the patient experienced a full-thickness cleft. Nasal cleft repair in the pediatric population must be guided by the following three principles: creation of a volume with lining, support, and cover; conservation of an adequate airway; and aesthetic incorporation into the face.5 Because no single method has been proven to be the standard of care in pediatric nasal reconstruction, the surgeon must possess thorough knowledge of the various techniques available to select the correct intervention for each child. Anup Patel, B.S. Bianca I. Knoll, M.D. John A. Persing, M.D. Section of Plastic and Reconstructive Surgery Yale University School of Medicine New Haven, Conn. DISCLOSURE None of the authors has a financial interest to disclose in relation to the content of this article.

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