Malformations anorectales a l'Hopital de Panzi: aspects epidemiologiques et therapeutiques. A propos de 15 cas

Introduction : Les malformations anorectales (MAR) regroupent des anomalies diverses allant de la simple malposition anale a l'absence totale du rectum et de l'anus. Leur frequence est estimee a 1/5000 naissances. Le but de cette etude etait de decrire les aspects epidemiocliniques et therapeutiques des malformations anorectales diagnostiquees et prises en charge a l'Hopital de Panzi entre le 1er janvier 2004 et le 31 decembre 2008. Materiel et methodes : Une etude retrospective des dossiers de patients admis pour malformations anales a permis de colliger 15 cas. Les parametres d'interet enregistres comprenaient : - l'identite; l'age et le sexe ; - les donnees cliniques dont le motif de consultation et l'examen physique ; - l'imagerie avec l'invertogramme en cas de MAR totalement obstructive; la fistulographie en cas de fistule observee; ou encore la colographie chez les patients ayant beneficie d'une colostomie en premiere intention. Le traitement qui consistait en une derivation de selles avant la creation d'un neoanus soit par un double abord abdomino-perineal (technique de Stephens-Douglas); soit par une plastie YV; etait egalement renseigne. Resultats : La frequence hospitaliere de la malformation anorectale etait de 7;73. L'age median des enfants etait de 12 jours (extremes de 1 jour et 2 ans). Il s'agissait de dix garcons et cinq filles. Les circonstances de decouverte etaient dominees par un syndrome occlusif (10 cas); l'emission de selles par un orifice perineal ectopique (4 cas); une meconiurie (1 cas). L'examen clinique et radiologique avaient permis de classer les enfants en MAR haute (8 cas); MAR basse (5 cas) et MAR intermediaire (2 cas). Quatre enfants presentaient des malformations associees a type d'agenesie sacree (2 cas); une hernie ombilicale (1 cas) et une communication interventriculaire (1 cas). La colostomie etait systematique (15 cas); l'abaissement etait realise soit selon la technique de Stephens - Douglas (10 cas); soit par une plastie Y-V (2 cas). Six cas de deces ont ete deplores dont trois apres la colostomie et les trois autres sont survenus apres la cure definitive de la malformation anorectale. La continence anale; evaluee chez 9 enfants apres un recul moyen de 16 mois; etait normale chez 6 d'entre eux. On notait une souillure des couches dans 3 observations. Conclusion : La MAR est une realite dans notre contexte et le diagnostic est facilite par une integration des donnees cliniques simples et d'imagerie. La precocite du diagnostique et le traitement chirurgical de reconstitution representent les mesures adequates de prise en charge

Congenital lower intestinal tract anomalies: review of surgical management of 50 Iraqi patients in Al-Najaf City

Congenital lower intestinal tract anomalies are significant cause of morbidity and mortality in children, classified in two groups, major one is anorectal malformation, other one Hirchsprung's disease, usually associated with other anomalies and should be treated as early as possible. To show the prevalence and our experience with congenital lower intestinal tract anomalies in AL-Najaf city. A total 50 patients had various types of congenital lower intestinal tract anomalies, 22 of them high types [anorectal] treated by 3 stages operations[preliminary colostomy, nearly new definitive surgery infracoccygeal approach and closure colostomy], minor surgery for low types [12] and for Hirchsprung's disease treated by usual pull through operation[Swenson's]. The anorectal anomalies [A.R.M] are the most common anomalies of lower intestinal tract, high type cases were 25, low type were 12 cases and Hirchsprung's disease were13 cases. Male were 30 cases and female were 20 cases, age of presentation between first day of life and 18 years of life. Infracoccygeal approach was the safe and non complicated operation in dealing with high type of anorectal malformation and the good results obtained when the operation was done early period of life [around one year]

Malformaciones congénitas anorrectales y sus asociaciones preferentes: experiencia del Hospital Clínico de la Universidad de Chile, período 1979-1999 / Anorectal congenital malformations and their associations in a Chilean University Hospital between 1979 and 1999

Background: Anorectal atresia is a relatively frequent malformation in the newborn. According to the Latin American Collaborative Study for Congenital Malformations (ECLAMC), its frequency is 4.1 per 10,000 born alive. Aim: To determine the frequency of anorectal malformations at birth, and compare the figures with those of other maternity hospitals in Chile participating in ECLAMC, with the figures of the whole ECLAMC and with figures from other worldwide monitoring systems. Patients and methods: All births occurred in the University of Chile Clinical Hospital between January 1979 and August 1999, were reviewed. Results: During the study period, 70,242 children were born, 4,486 had a malformation and 54 had an anorectal malformation (7.7 per 10,000 born alive). Fifty nine percent had other associated malformations (of the urinary tract in 42.5 percent, skeletal in 26 percent and cardiovascular in 18.5 percent). Five stillborn babies had other severe malformations. Twenty one children had a fistula. Forty three percent were male, 39 percent female and 18 percent had ambiguous sex. When compared with normal controls, malformed newborns had a lower birth weight, lower gestational age and a higher mean maternal age, a higher frequency of metrorrhagia during the first trimester of pregnancy, a higher number of siblings with malformations and a higher degree of consanguinity among parents. Conclusions: The participation of recessive genes in the etiology of anorectal malformations is suggested