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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 1  |  Issue : 2  |  Page : 132-134

Lower facial cleft: A rare cleft associated with foregut duplication cyst


1 Department of Pediatric Surgery, KLE University, Belgaum, Karnataka, India
2 Department of Maxillofacial Surgery, KLE University, Belgaum, Karnataka, India

Date of Web Publication2-Aug-2014

Correspondence Address:
Dr. Vijay C Pujar
Department of Paediatric Surgery, KLE University, Belgaum, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2348-2125.137923

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  Abstract 

Facial clefts are very common facial anomalies encountered in practice. However, lower facial clefts are very rare. Tessier has grouped these defects as cleft no. 30. Only 65 cases have been reported in the literature until 1996. Median cleft of the lower lip, mandible and bifid tongue with ankyloglossia has been reported in this anomaly. We report this rare cleft associated with bifid tongue with foregut duplication cyst over the floor of mouth.

Keywords: Facial clefts, foregut duplication, lower


How to cite this article:
Pujar VC, Kotrashetti SM, Joshi SS. Lower facial cleft: A rare cleft associated with foregut duplication cyst. J Cleft Lip Palate Craniofac Anomal 2014;1:132-4

How to cite this URL:
Pujar VC, Kotrashetti SM, Joshi SS. Lower facial cleft: A rare cleft associated with foregut duplication cyst. J Cleft Lip Palate Craniofac Anomal [serial online] 2014 [cited 2019 Aug 26];1:132-4. Available from: http://www.jclpca.org/text.asp?2014/1/2/132/137923


  Introduction Top


Facial clefts generate curiosity as it causes facial deformity causing low self-esteem. The median clefts of lower lip and mandible are rare and are considered as the result of failure of mesodermal migration or merging of the paired mandibular processes.

This median cleft of lower jaw was first described by Couronne in 1819. [1] In 1976, Tessier published a detailed description of the classification of craniofacial clefts, [2] wherein the lower midline facial cleft was numbered as 30 and are considered as caudal extensions of the no. 14 cranial cleft and no. 0 facial cleft. Cleft no. 30 includes mandibular process clefts, intermandibular dysplasia, and midline branchiogenic syndrome.


  Case Report Top


A new born female baby born through normal vaginal delivery was brought to our department with a complex facial deformity involving the lip and palate. Baby had excessive cry and was unable to take feed since birth. There was a mass arising from the floor of the mouth.

On examination baby had cleft of the lower lip, cleft soft palate, bifid tongue and a soft fleshy mass arising from the floor of the mouth measuring 4 cm × 3 cm. Baby also had cleft mandible with two nodules on either side of cleft [Figure 1]. There was a small island of tongue tissue over the lower alveolus with a frenulum attached to the bifid tongue [Figure 2]. Complete workup was done to rule out cardiac and other associated anomalies.
Figure 1: Lower facial cleft

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Figure 2: Lower cleft with cyst

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At 4 months of age, excision of soft tissue mass from the floor of mouth with repair of bifid tongue, repair of cleft of lower lip was done [Figure 3]. Repair of cleft mandible was planned at a later date. Oral feed was started on the 2 nd postoperative day and the patient was discharged on the 4 th day. Histopathology confirmed presence of tongue tissue over the lower alveolus and a foregut duplication cyst over the floor of the mouth lined by heterotrophic gastric mucosa. At follow-up baby showed normal appearing tongue, lower lip and floor of mouth. Child had no deglutition problems and gaining weight [Figure 4].
Figure 3: Repaired bifid tongue

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Figure 4: Postoperative appearance

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  Discussion Top


Orofacial clefts are developmental anomalies that involve the lips and face and constitute the commonest variety of craniofacial clefts. [3] Midline cleft of lower jaw is one of the rarest craniofacial anomaly. Up to 1996, almost two centuries later, only 65 cases have ever been recorded worldwide. [1],[4] Soft tissue involvement of this rare anomaly may be as mild as a vermillion notch in the lower lip. However, more often it involves the entire lower lip and chin. The anterior part of tongue may be bifid and attached to the split mandible by a dense fibrous band. Ankyloglossia [5] and total absence of the tongue has also been reported [6] with midline mandibular clefts. The anterior neck strap muscles are often atrophic and replaced by dense fibrous bands which may restrict chin flexion. However, the neck movements and muscles appeared normal in our case.

Hard tissue involvement is typically a cleft between central incisors extending up to mandibular symphysis and is thought to be caused by failure of fusion of first branchial arch. However, associated neck anomalies are felt to be caused by failure of fusion of lower branchial processes. Duplication cysts are remnants of gastrointestinal tissue that persist after the 6 th week of intrauterine life, when the rest of the tissue has canalized and the tract has been formed. It may also be due to a delay in canalization or fusion of the affected tissue. [7]

The treatment, as advocated by Armstrong and Waterhouse is to tackle the condition in a staged manner. [8] Soft tissue correction in the first stage and mandibular reconstruction at 10 years of age to minimize damage to the developing tooth buds in second stage.


  Conclusion Top


Lower facial cleft are rare facaial deformities. Foregut duplication cyst in association of this lesion is very rare. We report this rare anomaly managed by soft tissue reconstruction.

 
  References Top

1.Monroe CW. Midline cleft of the lower lip, mandible and tongue with flexion contracture of the neck: Case report and review of the literature. Plast Reconstr Surg 1966;38:312-9.  Back to cited text no. 1
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2.Tessier P. Anatomical classification facial, cranio-facial and latero-facial clefts. J Maxillofac Surg 1976;4:69-92.  Back to cited text no. 2
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3.Ibrahim A, Ijekeye FO, Asuku ME. Tessier cleft numbers 7 and 30: Case report and literature review. Niger J Plast Surg 2008;4:52-7.  Back to cited text no. 3
    
4.Oostrom CA, Vermeij-Keers C, Gilbert PM, van der Meulen JC. Median cleft of the lower lip and mandible: Case reports, a new embryologic hypothesis, and subdivision. Plast Reconstr Surg 1996;97:313-20.  Back to cited text no. 4
    
5.Millard DR Jr, Lehman JA Jr, Deane M, Garst WP. Median cleft of the lower lip and mandible: A case report. Br J Plast Surg 1971;24:391-5.  Back to cited text no. 5
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6.Rosenthal R. Aglossia congenital: Report of a case of the condition combined with other congenital malformations. J Dis Child 1932;44:383-5.  Back to cited text no. 6
    
7.Velcek FT, Klotz DH, Hill CH, Ladogana LE, Kottmeier PK. Tongue lesions in children. J Pediatr Surg 1979;14:238-46.  Back to cited text no. 7
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8.Armstrong AP, Waterhouse N. Tessier 30 median mandibular cleft: Case report and literature review. Br J Plast Surg 1996;49:536-8.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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Abstract
Introduction
Case Report
Discussion
Conclusion
References
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