[Surgical treatment of Hirschprung's disease total form in children]

Khirurgiia
|August 23, 2014
PubMed

Insights

Optimal surgical treatment for total aganglionosis in young children requires timely diagnosis and intervention. Delayed treatment or incorrect diagnosis increases complications and impacts long-term outcomes, especially regarding bowel control.

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Neonatology

Context:

  • Analysis of 24 patients under 2 years old with total aganglionosis (Hirschsprung's disease) treated between 2000-2013.
  • Patients underwent multiple surgical interventions, averaging 7.8±2.1 procedures.
  • Surgical approaches included ileojejunorectal anastomosis for short bowel syndrome and Soave's operation (laparoscopic in 3 cases).

Purpose:

  • To evaluate treatment outcomes and identify factors influencing complications in young children with total aganglionosis.
  • To determine the optimal timing for surgical intervention to improve long-term functional results.
  • To highlight the impact of diagnostic accuracy on surgical success.

Summary:

  • Complications included paraproctitis (2 patients) and perirectal fistula (1 patient). Long-term stool incontinence affected 28% up to 6 months and 12.6% beyond 1 year.
  • Microbiological analysis revealed increased D-lactate-producing gram-positive anaerobes in 35% of patients, linked to acidosis and infectious enteritis.
  • Antibiotic therapy (Alpha-Normix, Trichopolum, Gentamicin) was used for short bowel syndrome patients with recurrent enteritis.

Impact:

  • Timely diagnosis and appropriate neonatal management significantly reduce complications and improve treatment outcomes.
  • Optimal surgical results were observed in children aged 2.5-3 years, correlating with improved voluntary control.
  • Diagnostic errors, particularly in morphological investigation of the large bowel, are a major cause of suboptimal outcomes.