Atypical facial clefts are very rare, affecting 1 child in about 400,000 births. The current standard classification is Tessier’s classification, which was developed in 1976. A drawback of this classification is that it mixes among clefting, hyperplasia, hypoplasia, and hamartoma, and it is also difficult to remember so that surgeons may need to consult a diagram, which provoked researchers to propose a new classification1,2 (Fig. 1).
The nose is affected by the central southbound Tessier’s cleft numbers 0, 1, 2, and 3 or the northbound cleft numbers 14, 13, 12, and 11 leading to either hypoplastic or hyperplastic manifestations. Hypoplastic manifestations are mainly clefting, whereas hyperplastic manifestations include hamartomas or meningoceles.
Results of nasal reconstruction in median facial clefts are hard to predict due to affection of growth by surgical intervention and diminished intrinsic growth potential of the central midface.3
Nasal reconstruction involves harmonious cutaneous and skeletal reconstruction. Adequate skin is needed as an external nasal coverage of good quality and also as an inner nasal lining. Adequate soft tissues are needed to create vascular cushion for bone grafts. Skin reconstruction without placement of an underlying skeletal framework in proper time will eventually lead to contracture of the skin and inability to achieve nasal projection with bone grafts in a later stage.
The literature lacks adequate data about the methodology of skin reconstruction in atypical facial clefts due to rarity of these cases. Monasterio and Taylor4 developed and implemented a treatment philosophy for facial clefts with the following objectives: restoration of the craniofacial skeleton, reconstruction with skin and soft tissue of the same color and texture, generous use of tissue expanders, aesthetic unit and subunit reconstruction, scar location at limits of aesthetic subunits, and symmetric repositioning of key facial landmarks. In this study, the authors have shown that in atypical facial clefts, soft tissues are affected in 73% of cases, combined soft tissues and bony affection occur in 23% of cases, and in 5% of cases only bony structures are involved (Fig. 2).
A composite mucochondrocutaneous lateral alar flap achieved reconstruction of the alar rim with the resulting defect on the lateral nasal wall covered with a transposition flap from the dorsum, and then an alar rim Z-plasty was added to correct notching with no problem of flap viability and minimal scarring.5
Unlike typical clefts of the lip, nose, and palate, which greatly differ in severity but have common anatomical stigmata, atypical facial clefts affecting the lip and nose have a diversity of anatomical aberrations, which are difficult to categorize, and subsequently make establishment of treatment methodology very difficult. In this study, the author demonstrates a variety of techniques for nasal reconstruction in congenital nasal clefts trying to find a strategy for the management of this problem.
PATIENTS AND METHODS
The authors had treated 15 cases of atypical facial clefts affecting the nose between 1996 and 2014.
The patients were divided into 3 main groups (Table 1), which are described subsequently.
This group includes 3 cases of Tessier’s cleft number 0.
Description of Morbid Anatomy
This is a bifid nose deformity with loss of tip projection and wide bifid columella and median cleft of the upper lip leading to wide philtrum and either absent or double nasal septum with deficiency of the lower part; these include case numbers 1, 2, and 3 (Fig. 3).
This group includes 8 cases of Tessier’s cleft numbers 0 to 14 (median facial clefts).
Description of Morbid Anatomy
These cases are characterized by the presence of hypertelorbitism and median clefting of the nose separating it into 2 equal halves with rotation of the axis of the alae of the nose with varying degrees and either hypoplasia or aplasia of the columella and the nasal septum. There were midline hamartomas in 3 cases, and frontonasal meningoencephalocele with a 3-cm oval defect in the anterior cranial fossa in 1 case. These include cases 4 to 11 (Figs. 4 and 5).
This group includes 4 cases of Tessier’s facial cleft cases 3 to 11 (2 cases) and 3 to 12 (2 cases).
Description of Morbid Anatomy
The cleft occurs along the philtral column, which becomes wide or clefted. The nasal floor is deficient, and there is upward shift of alar base. The lacrimal system and the lower canaliculus are affected. The cleft travels through the medial part of lower lid causing coloboma, and there is unilateral hypertelorbitism. One patient (number 12) had anophthalmia of the left eye. These include cases 12 to 15 (Fig. 6).
Details of surgical intervention are described in Table 1.
In this series, nasal reconstruction was done following the general rule of replacing tissues with tissues alike. In minimal skin deficiency, skin is obtained from the same region utilizing local flaps as V–Y advancement in vertical shortening with number 0 clefts (cases 4, 8, and 9), bilateral Z-plasty in horizontal deficiency with number 0 clefts (cases 6 and 7), and local rearrangement of tissues in unilateral number 3 clefts (cases 12 and 13). In case number 11, the nasal skin is approximated by purse string suture after excision of the meningoencephalocele. In major skin deficiency, skin is burrowed from the forehead after tissue expansion in number 3 clefts (cases 12 and 13).
Whenever there is substantial nasal lining deficiency as in case numbers 12 and 13, this is reconstructed by folding of the expanded forehead skin. In some cases, the sole use of bone grafts can achieve reasonable nasal projection without skin coverage (case numbers 1–3), but because bone grafts do not grow with the same rate of the growing face in young children, this should be repeated as required. In most of the cases, the iliac bone grafts are used to augment the nasal dorsum. In some cases, the lower septum and columella were reconstructed by costochondral cartilage graft in addition to bone grafts.
In the largest series reported, Ortiz Monsaterio (1987) reviewed 345 atypical facial clefts treated over 20-year period, only 2.2% (146 patients) had nasal clefts corresponding to types 0, 1, 2, and 3 of Tessier’s classification. They were distributed as follows: 92 patients have Tessier’s 0 (63%), 16 patients have Tessier’s 1 (10.9%), 23 patients have Tessier’s 2 (15.7%), and 15 patients have Tessier’s 3 (10.3%).4 In our study, a total of 32 facial clefts have been encountered, of which 15 patients had atypical nasal clefts, 3 cases with Tessier’s cleft number 0 (20%), 8 cases with Tessier’s cleft numbers 0 to 14 (53%), and 4 cases with Tessier’s cleft numbers 3 to 11 and 3 to 12 (27%). After examining the deformity, we decided the plan of management and sequence of reconstructive steps. The goal of reconstruction is to restore the normal aesthetic anatomy and normal function of the nose, and the reconstruction involves the skeletal framework of the nose (bony for the bridge and cartilaginous for the septum and columella), skin coverage, and nasal lining. Repair of congenital nasal clefts cannot be done regardless of associated anomalies as hypertelorbitism or cleft lip. For example, correction of hypertelorism is considered an important step in repairing nasal cleft. Hypertelorism is usually done before nasal reconstruction, but in case number 4 the surgeons and the patient had agreed that the hypertelorism was too mild for a major surgery, whereas in case number 6 the lower part of the nose was reconstructed by bilateral Z-plasties at the age of 2 years to improve the face till orbital bipartition that can be done 2 years later. When the skin deficiency is large, as in cases of severe hypoplasia of half nose, the expanded forehead flaps can be used. Iliac bone grafts for upper nasal defects and costochondral grafts for lower nasal defects achieve skeletal nasal reconstruction. The iliac bone is characterized by bulkiness and hardness, so that the harvested bone segment can be easily shaped for contouring the nose. Also, harvesting iliac bone graft is characterized by easy access and a well-hidden scar. However, bone grafts do not grow in same proportion to the growing skull of the child, so they are repeated with growth of the child. In cases with bifid nasal bone without hypoplasia, the deformity can be corrected by medialization of nasal bones by bilateral basal osteotomies. Restoring normal aesthetic anatomy usually needs cartilage graft; the costal cartilage was the preferred donor site. Whenever skin is needed for columellar reconstruction, it was obtained from the upper lip, especially in median facial cleft (Fig. 7).
Congenital atypical nasal clefts, unlike typical cleft lip, nose, and palate, are very rare and show diversity of anatomical aberrations, which defy standardization of surgical technique for their correction. The authors are proposing a simple strategy for this problem with satisfactory outcome, which is achieved by judging whether the local skin is adequate for local flaps or burrowing of skin from the forehead will be needed by expansion. Synchronized bone grafts are vital in proper time to prevent soft-tissue contraction and these can be repeated along with the growing head of the child.
Parents or guardians provided written consent for the use of the patients’ image.
1. Tessier R. Anatomical classification of facial, craniofacial and laterofacial clefts. J. Maxillofac. Surg. 1976.4: 69.
2. Losee JE, Kirschner RE, Whitaker LA, et al. Congenital nasal anomalies: a classification scheme. Plast Reconstr Surg. 2004;113:676–689.
3. van den Elzen ME, Versnel SL, Wolvius EB, et al. Long-term results after 40 years experience with treatment of rare facial clefts: Part 2–Symmetrical median clefts. J Plast Reconstr Aesthet Surg. 2011;64:1344–1352.
4. Monasterio FO, Taylor JA. Major craniofacial clefts: case series and treatment philosophy. Plast Reconstr Surg. 2008;122(2):534–543.
Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons.
5. Rashid M, Islam MZ, Tamimy MS, et al. Rotation-transposition correction of nasal deformity in Tessier number 1 and 2 clefts. Cleft Palate Craniofac J. 2009;46:674–680.