LARYNGEAL CLEFT
Ashutosh Sudhakar Mangalgiri, Raza Razvi, Rajendra Saoji*, Satish B Deopujari**
Department of Anatomy, People`s College of Medical Sciences and Research Centre, Bhopal, Madhya Pradesh, *Pediatric Surgery and **Pediatrics Department, Central India`s Child Hospital and Research Institute, Nagpur, India.
Address for Correspondence
Ashutosh Sudhakar Mangalgiri, Associate Professor of Anatomy, People's College of Medical Sciences and Research Centre, Bhopal, Madhya Pradesh - 462037, India.
Email
ashutoshmangalgiri@yahoo.co.in
Keywords
Laryngeal cleft, Cleft, Congenital Anomaly, Larynx, Tracheo-esophageal cleft
Larynx of an 8 month male foetal cadaver was removed by dissecting above the hyoid bone and below up to the 4th to 5th tracheal ring. An incidental finding of cleft was noted in the posterior part of larynx. The cleft was extending into the interarytenoid musculature. Cricoid cartilage was palpated and confirmed that the cleft was not extending up in to the cricoid cartilage. No other recognizable congenital malformations were observed in a foetal cadaver.

Laryngotracheoesophageal cleft is a rare entity. This congenital condition depending upon the extent of the cleft in the larynx and trachea may become life-threatening and lead to immediate death after the birth (1). The incidence of Laryngotracheoesophageal cleft (LTOC) is 0.3% out of the total congenital anomalies of the larynx (2). Overall 6% of cases of type I laryngeal clefts observed in total paediatric direct laryngoscopies (3). Minor conditions usually present with hoarseness and recurrent respiratory infections and therefore many a time diagnosis is delayed. Symptoms usually resembles with oesophageal atresia & tracheo-oesophageal fistula (1). One should suspect the condition when child presents with a triad of husky cry, feeding difficulty and aspiration pneumonia (1). The symptoms mostly aggravate during feeding.

The arrest of the cranial advancement of the tracheo-oesophageal septum is responsible for the non fusion of the cricoid lamina in the midline, leading to the development of the cleft (4,5,6). The clefts are functionally divided into 4 types. Type I involving only interarytenoid musculature, Type II involves cricoid only, Type III involves proximal laryngo-tracheo-esophagus & type IV involves thoracic tracheo-esophageal septum (7). LTOC may present singly or may present with the other anomalies like, harelip, cleft palate, oesophageal atresia, atresia ani, vulvo-vestibular fistula, sacral hypoplasia (5). Familial conditions of Laryngotracheoesophageal cleft (LTOC) have also been reported (8,9).

The diagnosis of laryngeal cleft can be confirmed by direct laryngoscopy as early as 3 weeks only (1). Barium esophagogram and bronchoscopy are also important investigations for diagnosis. Treatment consists of surgical repair, although some patients with type I laryngeal clefts may be managed on a conservative trial (3). Endoscopic repair of cleft larynx is best used for type I clefts (10). Early surgical intervention would prevent further damage to lungs and oesophagus.
References :
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  2. Evans JN. Management of the cleft larynx and tracheoesophageal clefts. Ann Otol Rhinol Laryngol. 1985; 94: 627-630.
  3. Parsons DS, Herr T. Delayed diagnosis of a laryngotracheoesophageal cleft. Int J Pediatr Otorhinolaryngol. 1997; 39: 169-173.
  4. Zaw-Tun HI. Development of congenital laryngeal atresias and clefts. Ann Otol Rhinol Laryngol. 1988; 97: 353-358.
  5. Roggini M, Carbone I, Bosco S, Cozzi DA, Andreoli G, Capocaccia P. Persistent esophagotrachea: description of a case. Eur Rev Med Pharmacol Sci. 2000; 4: 95-97.
  6. Pezzettigotta SM, Leboulanger N, Roger G, Denoyelle F, Garabedian EN. Laryngeal cleft. Otolaryngol Clin North Am. 2008; 41: 913-933, ix.
  7. Benjamin B, Inglis A. Minor congenital laryngeal clefts: diagnosis and classification. Ann Otol Rhinol Laryngol. 1989; 98: 417-420.
  8. Cameron AH, Williams TC. Cleft larynx: a cause of laryngeal obstruction and incompetence. J Laryngol Otol. 1962; 76: 381-387.
  9. Conlon BJ, O'Dwyer T. The G syndrome/Opitz oculo-genital-laryngeal syndrome/Opitz BBB/G syndrome/Opitz-Frias syndrome. J Laryngol Otol. 1995; 109: 244-246
  10. DuBois JJ, Pokorny WJ, Harberg FJ, Smith RJ. Current management of laryngeal and laryngotracheoesophageal clefts. J Pediatr Surg. 1990; 25: 855-860.
Last Updated : Tuesday, June 01, 2010 Vol 7 Issue 6 Art #33
How to Cite URL :
Mangalgiri S A, Razvi R, Saoji R, Deopujari B S. LARYNGEAL CLEFT. Pediatric Oncall [serial online] 2010[cited 2010 June 1];7. Art #33. Available From : http://www.pediatriconcall.com/Journal/Article/FullText.aspx?artid=311&type=J&tid=&imgid=&reportid=255&tbltype=
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