Reoperation for Hirschsprung's disease.

BACKGROUND/PURPOSE: Reoperation for Hirschsprung's disease traditionally has been used for patients with anastomotic leaks or stricture or with severe constipation from retained aganglionic segment or neuronal dysplasia, but there is little information regarding its use for other complications and the long-term outcome in these patients. METHODS: In a 23-year period, 107 infants and children underwent Soave (68 patients) or Duhamel (39 patients) pull-through procedures. The age at operation was newborn to 6 years (mean, 10 months). Eighty percent had aganglionosis limited to the rectosigmoid colon. Follow-up was by office visit or telephone (mean, 8.5 years). RESULTS: Twenty-three of the 68 patients with Soave pull-through (34%) underwent reoperation for intractable enterocolitis (10 patients, all 10 cured); anastomotic stenosis (four patients, three cured, one continued diversion); anastomotic leak (four patients, four cured); retained aganglionic segment (three patients, three cured); one necrosis of pull-through converted to Duhamel and cured; and one rectal prolapse that was diverted. Fifteen of the 39 patients with Duhamel procedure (38%) underwent reoperation for severe constipation (seven patients, six cured, one diverted); persistent rectal septum (four patients, 4 cured); and intractable enterocolitis (four patients, three cured, one diverted). Overall, 21 of 23 patients (91%) with reoperation after Soave procedures were cured, whereas 13 of 15 patients (87%) who underwent reoperation after Duhamel procedure were cured, and four patients remain diverted. CONCLUSIONS: These data show that aggressive reoperation can result in a high cure rate in Hirschsprung's disease. Although there is no significant difference in the rate of reoperation after Duhamel and Soave procedures, the patients with Soave pull-through required more complex reoperations, with several requiring more than one procedure. An aggressive approach to reoperation in patients with Hirschsprung's disease clearly is justified.

Heart transplantation among 233 infants during the first six months of life: the Loma Linda experience. Loma Linda Pediatric Heart TransplantGroup.

Two hundred thirty-three heart transplantations were performed in infants during their first 6 months of life at Loma Linda University between November, 1985 and June, 1999. Survival has now exceeded 13 years. Nearly 70% of infants are expected to live at least 10 years. Those transplanted during the first 30 days of life have about a 15% survival advantage at 10 years. Scarcity of donors continues to limit the transplantation effort. While acute rejection is the most common cause of late mortality, posttransplant coronary artery disease (PTCAD) is the leading cause of graft loss affecting 22 recipients (9.5%). The majority of patients are asymptomatic prior to diagnosis of PTCAD and are either retransplanted or dead within 6 months. Retransplantation (9 of 11 retransplantations for PTCAD) has been highly successful, with 10 year actuarial survival of 91%. Posttransplant lymphoproliferative disease (PTLD) has been found in only 7 patients (3%), most commonly in lymph nodes. Causes of late mortality include acute rejection (n = 16), PTCAD (n = 9), infection (n = 7), PTLD (n = 2), chronic graft dysfunction (n = 2), arrhythmia (n = 1), recurrent pulmonary vein stenosis (n = 1), and other noncardiac causes (n = 4). Infant psychomotor development is mildly delayed although cognitive development is normal. School-age children are performing at the level of their peers with average achievement and low average intelligence testing. Heart transplantation is durable therapy for newborns and infants with structurally incurable and end-stage myopathic heart disease.

Critical analysis of the operative treatment of Hirschsprung's disease.

OBJECTIVE: To critically analyze complications and long-term results of the operative treatment of Hirschsprung's disease. DESIGN: Medical records of patients with Hirschsprung's disease were reviewed retrospectively. Follow-up was obtained using a standardized telephone questionnaire. SETTING: Major pediatric referral center. PATIENTS: Eighty-two infants and children (68 boys, 14 girls) were treated for Hirschsprung's disease during a 20-year period (1975 to 1994). The age at diagnosis was younger than 30 days in 47 neonates (57%), 30 days to 1 year in 22 infants (27%), and older than 1 year in 13 children (16%). Aganglionosis was limited to the rectosigmoid region in 66 patients (81%). Fifty-five Soave (endorectal) and 27 Duhamel (retrorectal) primary pull-through operations were performed. MAIN OUTCOME MEASURES: Postoperative complications, reoperations, hospitalization, and current bowel habits. RESULTS: Eighteen children (67%) undergoing the Duhamel operation recovered uneventfully compared with 33 children (60%) undergoing the Soave operation. The complications following the Duhamel operation included enterocolitis in five cases (19%), rectal achalasia in four cases (15%), and persistent rectal septum in two cases (7%). Additional operations, which included myomectomy, rectal septum division, diverting enterostomy, and sphincterotomy, were required in seven patients (26%). Only one patient required more than one reoperation. In contrast, complications following the Soave operation included enterocolitis in 15 cases (27%), rectal stenosis in 12 (22%), anastomotic leak in four (7%), late perirectal fistula in three (5%), rectal prolapse in one (2%), and recurrent severe constipation in one (2%). Sixteen patients (29%) required additional operations, including diverting enterostomy, myomectomy, redo pull-through, sphincterotomy, fistulectomy, and revision of rectal prolapse. In this group nearly two reoperative procedures per patient were required. Telephone follow-up (mean, 89.3 months) after pull-through operations in 61 patients (74%) showed a mean of 2.8 stools per day, with 13 patients (21%) requiring daily medications. CONCLUSIONS: The most common operations (Soave and Duhamel) for Hirschsprung's disease result in an uneventful recovery in only 60% to 67% of patients. Although both Soave and Duhamel pull-through operations have nearly identical reoperation rates (26% vs 29%), complications after Soave pull-through operations often require multiple, more extensive procedures. Short-term total continence rates for both procedures are less than 50%, however, 100% became continent by 15 years after the pull-through procedure. Further refinement in operative technique and close follow-up are warranted.