Intussusception in cystic fibrosis.

Abstract

Two cases of acute intussusception in older children with cystic fibrosis are reported. Both cases presented with symptoms and signs consistent with meconium ileus equivalent, which delayed the final diagnosis. Both cases required abdominal surgery but made full and uneventful recoveries.Intussusception occurring after the age of 2 years is relatively rare, but occurs in at least 1% of the population with cystic fibrosis.' We report two cases of intussusception occurring in patients with cystic fibrosis. A specific aetiology could not be found in either case. A boy was diagnosed as suffering from cystic fibrosis at the age of 6 months by three positive sweat tests (his sweat sodium concentrations were 100, 105, and 108 mmol/l). He had been on pancreatic supplements with daily physiotherapy since then and regular courses of oral antibiotics for intercurrent infections. He had a history of intermittent bouts of abdominal pain with constipation. He was admitted to hospital at age 4 years complaining of colicky abdominal pains and constipation. Abdominal examination was normal. A clinical diagnosis of meconium ileus equivalent was made. Acetylcysteine was commenced (5 ml three times a day). The patient developed loose stools of increasing frequency over the subsequent three days, but otherwise showed no symptomatic improvement. A vague mass was identified in the left upper quadrant which was thought to be faeces. This theory was supported by a plain radiograph of his abdomen. Daily bowel washouts were commenced and the patient's condition improved sufficiently over the subsequent five days for him to be discharged from hospital. The next day he was readmitted with an occurrence of the original symptoms. Examination showed a distended abdomen but nothing else. A plain radiograph of his abdomen showed a complete absence of gas in the splenic flexure and descending colon but no dilated loops of bowel or fluid levels. Abdominal ultrasound showed echogenic masses throughout the colon that were thought to be due to a faecally loaded colon. Subsequent plain radiographs of his abdomen showed dilated loops of bowel and fluid levels. A laparotomy was performed because of the acute obstruction. An ileocaecal intussusception was found with rupture of the apex of the intussusception through the wall of the sigmoid colon. A resection of bowel was performed with ileocolic anastamosis. As the sigmoid colon at the site of rupture was of doubtful viability a colostomy was fashioned with a view to later repair and closure. This was done one month later. His recovery was uneventful. He was discharged on normal diet and pancreatic enzyme supplements and the problem has not recurred. DiscussionAcute intussusception is a common surgical problem in the first two years of life but is rarely observed in the older child.2 It is a recognised complication in the older children who suffer from cystic fibrosis and its incidence has been put at 1% with an average age of onset between 9 and 12 years. ' Despite this fact, it is a complicatio...

CT of intussusception in the pediatric patient: diagnosis and pitfalls

“…In the second example, of the patient with cystic fibrosis and pericecal disease, the water-soluble enema confirmed the absence of intussusception and revealed a cecal fistula tract and considerable surrounding inflammation. Intussusception is a reported complication of cystic fibrosis, and the clinical presentation can be confused with other disease processes such as distal intestinal obstruction syndrome or appendicitis [10,11].…”

Inverted appendix mistaken for a polyp during colonoscopy and leading to intussusception

“…An inverted appendix along with chronic non-ischaemic intussusception is unusual in healthy children but a recognised complication of CF [4]. In patients with CF, submucosal gland hyperplasia and inspissated secretions can make the appendix bulky and heavy, and thus predispose to intussusception [5,6]. An intussusception in CF may not cause complete obstruction, is rarely acute, can reduce spontaneously and is often overlooked [6].…”

“…In patients with CF, submucosal gland hyperplasia and inspissated secretions can make the appendix bulky and heavy, and thus predispose to intussusception [5,6]. An intussusception in CF may not cause complete obstruction, is rarely acute, can reduce spontaneously and is often overlooked [6]. Both appendiceal disease and intussusception may cause diagnostic problems in patients with CF and should be considered in the differential diagnosis of abdominal pain.…”

Darmveränderungen bei Mukoviszidosepatienten in Korrelation mit Enzymsubstitution und Klinik

“…Sonografische Befunde im Bereich des terminalen Ileums, Zäkums und Colon ascendens Darmkomplikationen bei CF [7,8,10,12,13,15,17,21] Wenn die in der Neugeborenenperiode auftretenden Probleme eines Mekoniumileus oder die bereits intrauterin stattfindende Mekoniumperitonitis außer Acht gelassen werden, stellt in den folgenden Jahren das distale intestinale Obstruktionssyndrom (DIOS), synonym Mekoniumileusäquivalent, das häufigste Krankheitsbild dar.…”