CASE REPORT

TESSIER CLEFT NO. 30; PRESENTING WITH MEDIAN CLEFT OF THE LOWER LIP AND MANDIBLE, ANKYLOGLOSSIA AND MIDLINE CERVICAL WEB CAUSING NECK CONTRACTURE

Utpal Kumar Bordoloi.

  1. Associate Professor, Department of Plastic Surgery, Assam Medical College,Dibrugarh, Assam and Consultant Plastic Surgeon,Srishti Hospitals and Research Center, Dibrugarh.

CORRESPONDING AUTHOR

Dr. Utpal Kumar Bordoloi.

Consultant Plastic Surgeon,

Srishti Hospitals andResearch Center,

Dibrugarh, Assam.

E-mail:,

Ph: 0091 9435030597.

ABSTRACT: Tessier cleft no. 30 is a rare congenital deformity and presents with varying degree of severity from isolated median cleft of the lower lip to cleft of the manubrium sterni involving the mandible, tongue, floor of the mouth, hyod bone, thyroid cartilage and strap muscles of the neck. the cleft is frequently associated with ankyloglossia and median webin the neck extending from chin, causing neck contracture. I report a case of median cleft of lower lip, severe ankyloglossia, cleft of mandibular symphysis and a fibrotic bendextending from the chin to the suprasternal notch with a web, causing contracture of the neck; in a thirteen year old girl. The deformity was corrected in single stage, consisting of release of the tongue from floor of the mouth and lower alveolus, fixation of the mandibular cleft with titanium miniplate, repair of the cleft of the lower lip, excision of the fibrotic bend and correction of neck contracture with multiple z-plasties.

KEY WORDS:Median Cleft of lower lip; median cleft of mandible; Tessier cleft no. 30.

INTRODUCTION: I report a case of Tessier cleft no. 30; a rare congenital deformity; presenting with median cleft of the lower lip, cleft of the mandibular symphysis, severe ankyloglossia and a fibrotic bend extending from the chin to the suprasternal notch with a median web of skin causing contracture of the neck. The deformity was corrected by a single stage surgical procedure and the post operative period was uneventful.

CASE HISTORY: RRP, a thirteen year old female from Dhemaji district in north-eastern part of Assam, attended with median cleft of the lower lip and contracture of the neck due to a bend and a web of skin in the neck. On examination, her tongue was attached to the floor of the mouth and lower alveolus causing difficulty in speech. The median fibrous bend and the associated web of skin extended from the chin and the hyoid bone to suprasternal notch (Figure-1,2). Mobility could be elicited between the both halves of the mandible. Her dental occlusion was normal and all four lower incisors were present. No other congenital abnormality could be detected clinically and her general health was good. X-ray showed a complete cleft of the symphysis of the mandible (Figure-3).The surgical procedure was performed under general anaesthesia. The tongue was released from the lower alveolus and the floor of the mouth by releasing a thick and flat fibrous bend (Figure -4), which was continuous with the bend in the neck. The tongue as such was normal. The surgical defect in the floor of the mouth and the tongue could be repaired directly. The mandible was fixed with titanium mini plate after freshening of the edges. The cleft of the lower lip was repaired in three layers. Finally, the fibrous bend was excised by splitting the skin web. Multiple Z-Plasties were performed to correct the neck contracture. The post operative period was uneventful (Figure-5, 6).

DISCUSSION: Tessier cleft no. 30 coincides with the caudal extension of the cleft no. 016. Couronne in 1869 first described the median cleft of the lower jaw and there are very few case reports in literature since then16. It is caused by failure of midline union of the first pair of branchial arches. Associated failure of fusion of lower pairs of branchial arches account for often reported caudal extension of the deformity14,16. Severity of the deformity varies. Thus, there may be a midline notch in the lower lip to complete midline cleft of the lower lip1; associated with or without notching of the mandible2 to complete cleft of the symphysis of the mandible5,,8, bifid or absence of the tongue5,9, absence of the hyoid bone, hypoplasia of the thyroid cartilage, hypoplasia of the strap muscles of the neck, bifid or absent manubrium sterni7 and bifid sternum with ventriculo-septal defect2. Various authors report ankyloglossia where the tongue is attached to the floor of the mouth and lower alveolus. A fibrotic bend in the neck with web of skin causing contracture of the neck3,5,12 is also reported in several cases. Other associated anomalies include cleft lip, cleft palate, Pierre-Robin anomaly, hemifacial microsomia, dermoid cyst of neck13, ectopic salivary gland2, haemangioma of lower lip4, Syndactyly, brachydactyly, clubfoot and congenital anomalies of the heart and the great vessels2.

The present reported case presented with median cleft of the lower lip (involving the full height of the lower lip), complete cleft of the mandibular symphysis, ankyloglossia, and midline cervical web causing neck contracture. There were no other associated congenital anomalies. Family and maternal history was not suggestive.

Like any facial cleft surgery, correction of soft tissue is the primary treatment. Mandibular surgery can be deferred till the age of ten years unless there is Emergency like breathing difficulty or feeding problems10,2. Surgical procedures on mandible include fixation of both mobile bone segments with stainless steel wire6, Vitallium or Titanium plate with or without bone graft5. One stage reconstruction using rib graft has been reported in literature17. In my case the gap between the mandibular halves appeared in jaw open position only. The both halves of the mandible could be fixed with titanium mini plate after freshening of the edges. Bone graft was not required and dental occlusion was normal.

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