D. von Schweinitz

Type IV laryngotracheoesophageal cleft associated with microgastria and left lung hypoplasia: report of a long-term survivor.

Laryngotracheoesophageal clefts (LTEC) are extremely rare congenital anomalies associated with a high attendant morbidity and mortality. LTEC are characterized by a midline communication between the esophagus and trachea extending from the larynx over a variable distance down to the bifurcation. The common proximal aerodigestive channel is a result of an incomplete separation of the primitive foregut into trachea and esophagus during fetal development. Clinical severity strongly depends on the length of the cleft. Many classifications have been proposed in order to distinguish the various clefts. Petterssonʼs anatomy-based classification divided laryngotracheoesophageal clefts into three groups according to their length. Later the classification was modified by Armitage [1] and Evans [9]. A clinically more relevant classification was offered by Benjamin and Inglis [10]: type I involves the supraglottic larynx, type II extends beyond the level of the vocal cords, type III is a total cricoid cleft with or without extension to cervical trachea, type IV is characterized by a complete extension of the cleft to the level of the carina with a common esophageal and tracheal lumen. Minor clefts account for the majority and only 3% of all clefts are type IV. While type I may be repaired endoscopically or even treated conservatively, type II–IV clefts are fatal conditions and require surgical repair [3,7,21]. A review of the literature revealed a few reported cases of successful surgical repair of type IV LTEC but only a handful of long-term survivors [4,13,15,27]. In this article we report on a 6-year-old long-term survivor after surgical repair of a LTEC extending to the bifurcation (type IV, Benjamin Inglis classification).

Re-operations for Hirschsprung's disease: long-term complications.

INTRODUCTIONnIn some patients with Hirschsprungs disease (HD), the initial surgical procedure fails, and the patients suffer from repeated or persistent symptoms. These patients complain of severe inflammation, intestinal obstruction, fecal or urinary incontinence, abdominal pain or dystrophy. However, little data has been published on the long-term follow-up results after re-operations for HD.nnnMATERIALS AND METHODSnWe followed 8 cases between 2004 and 2006, of complicated HD requiring repeated surgery and recorded prior procedures, histological results, indications for re-operation, postoperative follow-up as well as long-term clinical outcomes including stool patterns, nutrition and micturition.nnnRESULTSnThe follow-up period ranged from 3.0 to 5.5 years (mean: 4.4 years). Indications for repeat procedures were as follows: blind rectal pouch after a Duhamel operation (n = 2), persistent aganglionosis (n = 4), long-segment stenosis (n = 1) after a Rehbein operation, and anal stenosis following TERPT (transanal endorectal pull-through) (n = 1). In one patient who had a Duhamel-Martin operation, extirpation of the rectum and a definitive terminal ileostomy was necessary. A Duhamel procedure was performed in five patients with a primary Rehbein and 1 patient with a primary Duhamel operation. Complete stool continence was achieved in 4 patients. Partial fecal incontinence persisted in one patient with associated trisomy 21. 1 patient with total colonic aganglionosis and 1 patient with a pelvic fistula and a previous subtotal colectomy reported soiling 1-2 times per week after a repeat operation. 4 patients in our series experienced postoperative complications following repeated surgery [perianal ulceration (n = 2), repeated botulinum toxin injection for sphincter achalasia (n = 1) and functionally impaired colonic transit without stenosis (n = 1)]. Micturition was normal in 7 patients, 1 patient with associated trisomy 21 was partially continent, and 1 patient reported infrequent urge incontinence.nnnCONCLUSIONSnAll patients improved after further surgical intervention. However, resolution of their symptoms was delayed and partial stool incontinence or soiling persisted in 3 patients. Most complications leading to repeat procedures are preventable, especially residual aganglionosis. Therefore, great efforts should be made to minimize complications when planning and performing the primary surgery.