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     CLINICAL IMAGE

Bilateral Choanal Atresia with Tessier Type 3 Facial Cleft: A Rare Association


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Authors:  Munisamy Ragavan,* ArunKumar S, Balaji NS

Institute: Department of pediatric surgery, MIOT hospital Manapakkam, Chennai- 600089, India.

Address for Correspondence: Munisamy Ragavan MS,DNB, MRCS,MCh Department of pediatric surgery, MIOT hospital, Manapakkam, Chennai- 600089, India.

*Email: dr_ragavan_2001@rediffmail.com

* Corresponding Author

 


ISSN:2226-0439

Journal of Neonatal Surgery

Volume 1(3), Jul-Sep 2012


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Submitted On: 31-03-2012

Accepted On: 05-04-2012

 Published on: 01-07-2012

Local ID: jns-1-51

J Neonat Surg 2012; 1(3): 48

© Ragavan et al, 2012

Conflict of Interest: None declared

Source of Support: Nil

 


How to cite:

Ragavan M, Arunkumar S, Balaji NS. Bilateral choanal atresia with Tessier type 3 facial cleft a rare association. J Neonat Surg 2012; 1: 48.

 



Figure 1: Baby with Tessier type 3 facial cleft and choanal atresia after resuscitation for cyanosis.

 


Five-day-old male neonate born to non-consanguineous parents by normal vaginal delivery was presented with deformity of lip, face and eyelid on the right side, and cyanosis. The cyanosis had improved soon after endotracheal intubation. Examination revealed deformity of upper lip, upper jaw and lower eyelid of right eye; this type of craniofacial cleft is called as Tessier type 3 (Fig. 1). There was associated bilateral choanal atresia. The baby accidently self-extubated himself after 2 days, but could maintain thereafter with oxygen administration through hood. We plan to manage choanal atresia endoscopically at least on one side first before undertaking the reconstruction of the facial cleft part.

 

Craniofacial cleft may involve the mouth, cheeks, eyes, ears and forehead and may continue into the hairline. In 1976, Tessier classified the clefts between 0 and 14 based on extent of the cleft using the mouth, nose and eye sockets as landmarks with the midline designated as zero [1]. The Tessier 3 and 4 facial clefts, also called naso-ocular, or nasomaxillary clefts, result from the disruption of the lateral nasal and maxillary processes [2]. Tessier cleft types 3 and 4 are most difficult and challenging malformations to correct for the reconstructive surgeon. Various techniques such as Z-plasty, local flaps, cheek rotation flap including the lower eyelid, rotation and advancement flap of the cheek, and the tissue expansion methods have been described for the repair of such clefts [3]. Sometimes, neo-conjunctivorhinostomy and cranioplasty for plagiocephaly may also be required. In the absence of any well-laid guidelines for management of such rare cases, the proposed ‘split approach' of the affected areas of the cleft into three components: a) lid component; b) lip component; and c) nasomalar component, is very helpful. Individual management of the aforesaid demarcated areas is easy as compared to the surgery of the entire craniofacial cleft [4]. A simplified classification for craniofacial clefts based on a different surgical paradigm appropriate to each regional location has been described recently [5]. Both the facial cleft and the choanal atresia seen in our case have the same embryopathogenic context- an anomaly of migration of the neural crest cells [6]. The knowledge of this association is important as the choanal atresia could influence the outcome.


 

REFERENCES

  1. P. Tessier. Anatomical classification of facial, craniofacial and laterofacial cleft. J Maxillofac Surg. 1976; 4: 69-92.

  2. Zhou YQ, Ji J, Mu XZ, Zhang RH, Wei M, Yu ZY. Diagnosis and classification of congenital craniofacial cleft deformities. J Craniofac Surg. 2006 ;17:198-201

  3. Longaker MT, Lipshutz GS, Kawamoto HK. Reconstruction of Tessier no. 4 clefts revisited. Plast Reconstr Surg. 1997; 99:1501-7.

  4. Mishra RK, Purwar R. Formatting the surgical management of Tessier cleft types 3 and 4. Indian J Plast Surg. 2009; 42: S174-83.

  5. Bütow KW, Botha A. A classification and construction of congenital lateral facial clefts. J Craniomaxillofac Surg. 2010; 38:477-84. 

  6. Bonafos G, Capon-Degardin N, Fayoux P, Pellerin P. Choanal atresia and rare craniofacial clefts: report of three cases with a review of the literature. Cleft Palate Craniofac J. 2004; 41:78-83.


This is an Open Access article distributed under the terms of the Creative Commons Attribution unported License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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