Abstract

FABEIRO, Marcela et al. Síndrome de seudobstrucción intestinal crónica en la infancia. Arch. argent. pediatr. [online]. 2006, vol.104, n.2, pp.120-126. ISSN 0325-0075.

Introduction. Chronic idiopathic intestinal pseudoobstruction is defined by recurrent episodes of intestinal occlusion in the absence of mechanical obstruction. It may be classified in myopathic or neuropathic types by the full thickness intestinal biopsy. The aim of this study was to analyze clinic aspects, outcome and therapeutics in a group of patients with chronic idiopathic intestinal pseudo-obstruction. Population, matherial and methods. Twelve patients treated between 1988-2001 were included. Inclusion criteria were: a clinical diagnosis of primary chronic idiopathic intestinal pseudo-obstruction (2 males, 10 females). This is a retrospective study and the information was collected from the clinical records. Results. Median age was 88.6 months (range: 0.6 -238), 4 patients died (33.3%) - 3 for sepsis, one had a sudden death. 50% of the patients had neonatal debut, the 92% had symptoms in the first semester of life. In 33.3% an antenatal ultrasound diagnosis of megacystis had been made. When the diagnosis was made, the patients had one o more of the following symptoms: vomiting, distention, constipation, malnutrition, diarrhea or urinary infections. Eight full thickness intestinal biopsies were made: hystopathological evidence of enteric neurophaty was seen in 1 patient, myopathy in 3, and 4 were undeterminated. Treatment consisted in prokinetics drugs, oral antibiotics, abdominal physiotherapy and decompression. 8/12 patients received parenteral nutrition during more than 6 months (median: 51m.) and 33% enteral nutrition. Thirty surgical procedures were indicated (2.5/patient). Outcome was poor in 67% patients. Conclusions. Chronic idiopathic intestinal pseudoobstruction is an infrequent disease and most of the patients depend on partial or total parenteral nutrition. Mortality is associated with complications of the nutritional support. Clinical symptoms usually are present in infants and a high percentage of extradigestive disease is seen.

Keywords : Chronic intestinal pseudo-obstruction; Nutritional support; Intestinal trasplantation.

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