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Intra-abdominal complications of cystic fibrosis
Insights
Cystic fibrosis (CF) patients face intra-abdominal issues like meconium ileus (MI) and rectal prolapse. Improved survival rates for MI highlight advancements in CF care.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Genetics
Background:
- Patients with cystic fibrosis (CF) are living longer, leading to increased observation of intra-abdominal complications.
- Intra-abdominal complications are a significant concern in children diagnosed with CF.
Purpose of the Study:
- To evaluate the spectrum and outcomes of intra-abdominal complications in pediatric patients with cystic fibrosis (CF).
- To assess the efficacy of various management strategies for these complications.
Main Methods:
- Retrospective review of 69 pediatric CF patients (1972-1983) with intra-abdominal complications.
- Analysis of complication types, including meconium ileus (MI), meconium ileus equivalent, rectal prolapse, and inguinal hernia.
- Evaluation of treatment outcomes, including surgical and non-operative interventions.
Main Results:
- Meconium ileus (MI) was the most common neonatal complication (33/36), with an 86% survival rate in this study period, a significant improvement from previous decades.
- Other complications included rectal prolapse (12), inguinal hernia (10), and meconium ileus equivalent (7).
- Gastrografin enema showed success in managing simple MI and meconium ileus equivalent; surgical interventions were necessary for complicated MI and other conditions.
Conclusions:
- Intra-abdominal complications are frequent in pediatric CF patients.
- Management strategies for meconium ileus (MI) have improved, leading to better survival rates.
- Continued vigilance and tailored treatment are essential for addressing diverse intra-abdominal issues in CF.
Abstract:
In recent years patients with cystic fibrosis (CF) have experienced longterm survival and have demonstrated a number of intra-abdominal complications. This report evaluates the intra-abdominal complications seen in 69 of 189 children with cystic fibrosis from 1972 to 1983. Forty-one patients were boys and twenty-eight girls. Complications occurred in 36 neonates, with meconium ileus (MI) noted in 33 and giant cystic meconium peritonitis (GCMP) in 3. Meconium ileus equivalent occurred in seven older children presenting with bowel obstruction. In addition, rectal prolapse occurred in 12, inguinal hernia in 10, intussusception in 3, cholelithiasis in 3, GE reflux in 4, stress ulcer in 1 and appendicitis in 1. Three infants with GCMP survived resection and enterostomy. Infants with MI were divided into simple (15) or complicated (18) cases. Nonoperative therapy using gastrografin enema was successful in three of eight with simple MI. Operative enterotomy and irrigation was successful in three cases while resection and enterostomy was done in nine. MI was complicated by atresia, volvulus and/or perforation in 18 cases requiring resection and anastomosis or enterostomy. Survival for MI was 86% compared to 36% in 25 MI patients treated in the previous two decades. Meconium ileus equivalent was successfully managed using gastrografin enema in five of seven children. Only 3 of 12 children with rectal prolapse required repair. Two cases of intussusception were reduced while one required resection. Three of 10 children had hernia recurrence due to chronic pulmonary problems.(ABSTRACT TRUNCATED AT 250 WORDS)