Long-term outcomes in total colonic aganglionosis: a 32-year experience

Background/Methods

A 32-year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed.

Results

Thirty-six patients (27 boys, 9 girls) had TCA. The level of aganglionosis was distal ileum (26), mid-small bowel (8), midjejunum (1), and entire bowel (1). Enterostomy was performed in 35 of 36. Eight developed short bowel syndrome. Twenty-nine (81%) had a pull-through at 15 ± 6 months (modified Duhamel 20, Martin long Duhamel 4, and Soave 5). Six had a Kimura patch. Postoperative complications (including enterocolitis) were more common after long Duhamel and Soave procedures. Seven (19%; 2 with Down's syndrome) died (3 early, 4 late) from pulmonary emboli (1), sepsis (1), fluid overload (1), viral illness (1), liver failure (1), arrhythmia (1), and total bowel aganglionosis (1). Mean follow-up was 11 ± 9 years (range, 6 months-29 years). Twenty-four (83%) of 29 patients exhibited growth by weight of 25% or more, 21 (91%) of 23 older than toddler age had 4 to 6 bowel movements per day, and 17 (81%) of 21 were continent. In 5 of 6, the Kimura patch provided functional benefit with proximal disease.

Conclusion

Long-term survival was 81%. The highest morbidity occurred with long Duhamel or Soave procedures. The modified Duhamel is our procedure of choice in TCA. Bowel transplantation is an option for TCA with unadapted short bowel syndrome.     

Redo pull-through in Hirschsprung's [corrected] disease for obstructive symptoms due to residual aganglionosis and transition zone bowel

Background

Reoperations in Hirschsprung disease may be required for residual aganglionosis or transition-zone bowel found at the distal pull-through. We aimed to review the management of patients who had this complication and offer suggestions on how to avoid it.

Methods

Ninety-three patients with Hirschsprung disease were referred to our institution with recurrent problems after a pull-through done elsewhere. All required reoperations with a variety of indications, and of these, 25 had residual aganglionosis/transition-zone histology. This was the only indication for redo in 16 children.

Results

Children (range, 2-17 years) presented 6 to 66 months after the initial pull-through. The predominant symptoms were enterocolitis (n = 9 [56%]), constipation (n = 7 [44%]), failure to thrive (n = 5 [31%]), and impaction (n = 4 [25%]). The rectal biopsy performed as part of their post pull-through work up showed hypertrophic nerves (n = 16), absent ganglion cells (n = 6), and normal ganglion cells (n = 10). The original frozen-section biopsy, determining the level of the pull-through, only sampled the seromuscular layer in 3 children, leading to misdiagnosis. Reoperations involved a transanal resection (n = 15) and a posterior sagittal approach (n = 1). In all cases, obstructive symptoms were resolved, and no patient has had recurrent enterocolitis.

Conclusion

Patients' post pull-through with recurrent obstructive symptoms may have residual aganglionosis or transition-zone bowel. Reoperation can result in the resolution of these symptoms. A full-thickness biopsy at the time of the initial pull-through to include the mucosa and submucosa may increase the possibility of identifying hypertrophic nerves.     

Is complete resection of the aganglionic bowel in extensive total aganglionosis up to the middle ileum always necessary?

Background/Purpose

Total colonic aganglionosis is an unusual form of Hirschsprung disease, reflected by the small number of published case studies. The goal of this study was to analyze our cases of the last 22 years and report on our experience with 3 cases of extended total aganglionosis proximal to the middle ileum in which remaining aganglionic bowel was left in situ.

Materials and Methods

In a period of 22 years (from January 1988 to April 2010), we operated on 15 children with total aganglionosis. These children were among 163 children with Hirschsprung disease. In 3 patients with extended aganglionosis proximal to the middle ileum, remaining aganglionic bowel was left in situ. Data were collected from the medical records, including each patient's demographics, medical history, primary treatment, complications, and follow-up.

Results

Eleven of the 15 children received an ileostomy as first treatment, and 4 children underwent a primary Duhamel procedure. Early postoperative complications included an abscess after an open procedure in a patient with trisomy 21 syndrome and an incorrect leveling resection that required an ileostomy. There were 2 deaths at 6 and 16 months postoperatively. The mortality rate was 13.3%. Of the 15 patients, 7 (46%) presented with at least 1 episode of postoperative enterocolitis, and only 2 of these patients had more than 3 episodes. We treated 3 patients with extended aganglionosis up to midileum (20%) in which the proximal resection level was in the aganglionic segment of ileum at the level of the ileostomy. The ileostomies were shown to be functioning satisfactorily before the pull through, so going more proximal to the ileostomy might have meant that the patient would present short bowel problems. These 3 patients had good bowel function postoperatively.

Conclusion

In principle, resection of the aganglionic bowel is mandatory to relieve obstruction and subsequent complications. There are situations, however, where the complications of a short bowel begin to outweigh the benefits of resection. In these cases, leaving remaining aganglionic bowel in situ may be an advantageous option for the patients with total aganglionosis extending to the midileum whose ileostomy functions well.     

Long-term clinical outcome in patients with Hirschsprung's disease and associated Down's syndrome

Background/Purpose

Down's syndrome (DS) is the most common chromosomal abnormality associated with Hirschsprung's disease (HD). The purpose of this study was to review the long-term clinical outcome in patients with HD and associated DS.

Methods

Between 1975 and 2003, 39 (15%) of the 259 patients with HD had been associated with DS. Follow-up was carried out by means of examination of patient's records and personal/telephone interviews with the patient's parents or guardians.

Results

Twenty-six (67%) patients presented in the newborn period and 13 (33%) after the neonatal period. Twenty-eight (72%) patients had rectosigmoid HD, 10 long segment, and 1 total colonic aganglionosis. Thirty-two patients had other associated anomalies, 24 of these having cardiac anomalies. Definitive pull-through operation was performed in 33 patients. Parents of 1 child refused surgical intervention and parents of 2 children decided against pull-through operation after colostomy. Three children died before pull through. Thirteen patients had one or more episodes of enterocolitis after pull-through operation. At the time of follow-up (6 months to 28 years), 3 patients were found to have reverted to stoma because of poor bowel control or recurrent enterocolitis. Of the remaining 30 patients, 3 were lost to follow-up and 4 were too young to be assessed for bowel control. Assessment of bowel function in 23 patients revealed normal control in 8 (4 of these soiled for 6-17 years after definitive surgery), soiling in 8, and constipation requiring enemas or laxatives in 7.

Conclusions

The vast majority of patients with HD associated with DS continue to have disturbances of bowel function after definitive pull-through operation.     

Total colonic aganglionosis with or without small bowel involvement: a 30-year retrospective nationwide survey in Japan

Purpose

We conducted a nationwide survey in Japan, to identify recent trends in the diagnosis and treatment of patients who had total colonic aganglionosis with or without small bowel involvement (TCSA). These data were compared with 2 similar studies conducted within the last 30 years.

Methods

Patient data were collected in 3 phases; phase I included 135 patients between 1978 and 1982; phase II, 107 patients between 1988 and 1992; and phase III, 101 patients between 1998 and 2002.

Results

The incidence of TCSA was 1:59,059, 1:58,084, and 1:58,375, and the male-female ratios were 1.5:1, 1.5:1, and 2.2:1 in each phase, respectively. Patients with associated anomalies increased from 15.2% (phase I) to 22.8% (phase III). The incidence of preoperative enterocolitis decreased over time. For treatment, Duhamel's procedure and ascending colon patch methods have increased over time, whereas Martin's procedure has decreased. The mortality rate dropped from 40.9% to 15.8%; however, a high mortality rate persists in those cases with small bowel involvement (35.5%).

Conclusions

A marked decrease in the overall mortality rate was observed during the study period. However, further efforts are still required especially in cases involving aganglionosis extending orally to 75 cm from Treitz's band.     

Hirschsprung disease: do risk factors of poor surgical outcome exist?

Introduction

Long-term results after surgical treatment of Hirschsprung disease (HSCR) are not always as good as a surgeon may perceive. Several studies have tried to correlate preoperative features to the surgical outcome of HSCR, but none came to definitive conclusions. Our study is aimed at identifying risk factors of poorer long-term outcome after surgery for HSCR.

Materials and Methods

One hundred two patients with HSCR were included. Eighty had rectosigmoid aganglionosis and 22 had total colonic aganglionosis (TCSA). Preoperative variables were sex, associated anomalies, delayed meconium passage, preoperative enterocolitis, preoperative bowel obstruction, age at surgery, and number of pull-through procedures performed. Outcome measures were surgical complications, postoperative enterocolitis, perineal excoriations, constipation, continence, psychological self acceptance, and patients' perspectives. These were evaluated with regard to preoperative features and length of aganglionosis.

Results

Results of patients with TCSA proved to be significantly worse than those of patients with classic HSCR (constipation and poor continence excluded). Male patients with TCSA proved to have a significantly higher incidence of complications (100% vs 38.5%) and poor psychological self acceptance (100% vs 46.2%). Patients with associated anomalies (central nervous system) experienced a significantly higher incidence of postoperative constipation (43% vs 13%). Patients who described failure or delayed meconium passage complained of significantly worse continence (28.3% vs 7.1% of fair to poor continence). Preoperative enterocolitis proved to be significantly correlated to postoperative enterocolitis (16.4% vs 37.1%) and perineal excoriations (13.7% vs 30.9%). Patients with preoperative intestinal obstructions complained of significantly worse psychological self acceptance (37.8% vs 12.5%).

Conclusions

Length of aganglionosis has the greatest impact on overall surgical outcome of HSCR. Other minor risk factors have been identified, namely, male sex, associated central nervous system anomalies, failure to pass meconium, and preoperative enterocolitis occurrences. Age at surgery and redo procedures proved not to significantly interfere with the outcome. Our study provides comprehensive and useful data to inform parents and families of a baby with HSCR with regard to expected results and long-term outcomes of surgery basing on preoperative features. Further studies on larger series are strongly recommended.     

Laparoscopic localization and microlaparotomy for focal isolated perforation in necrotizing enterocolitis: an alternative approach to a challenging problem

Background

Necrotizing enterocolitis has a wide clinical spectrum of manifestation. We report a novel method of managing focal isolated perforation in necrotizing enterocolitis by using diagnostic laparoscopy to localize the site of perforation and by making a microincision over the perforation to perform exteriorization or limited resection and primary anastomosis.

Methods

We included low-birth weight infants presenting with sudden clinical deterioration and pneumoperitoneum. Patients with gross abdominal wall signs were excluded on the probability that they had extensive disease. Diagnostic laparoscopy was performed using a 3.5-mm trocar and 1.9-mm telescope. A 1-cm incision was made over the site of perforation, and local surgical debridement was performed.

Results

There were 3 extremely low-birth weight patients (580, 700, and 780 g) and 1 larger infant (1.6 kg). In all cases, an isolated perforation was detected. None had widespread disease. The 3 smaller infants had exteriorization and enterostomies. The larger patient had resection and primary anastomosis. All patients recovered uneventfully.

Conclusion

Diagnostic laparoscopy can be safely performed in extremely low-birth weight infants. It allows precise identification of the site of perforation to perform a limited microlaparotomy at this site, significantly reducing the surgical trauma of extensive bowel handling. We report a novel method of managing this vexing problem.     

Ten-year experience in the management of total colonic aganglionosis

Purpose

The aim of this study was to review the 10 years' experience in the management of patients with total colonic aganglionosis (TCA) and follow-up of their health condition.

Methods

Cases of 25 patients with TCA in the Children's Hospital of Fudan University from 1996 to 2005 were reviewed and analyzed. The confirmed diagnosis was established by an intraoperative frozen-section biopsy of the rectum, colon, appendix, and ileum. The data included in this study accounted for sex, age, signs of presentation, any familiar history of Hirschsprung disease (HD) or associated abnormalities, and ileal involvement. Plain x-ray films, barium enema, and anorectal manometry were provided for evaluation. The results of surgical management were analyzed for weight at definite operation, blood requirement during operation, the total parenteral nutrition duration, and the pre- and postoperative complications of these patients. Follow-up data were collected regarding growth development, stool frequency, stool consistency, fecal soiling, incontinence, enterocolitis, and anal stricture.

Results

Among 25 patients, 8 (32%) females and 17 (68%) males were diagnosed as having TCA. Sixteen patients (64%) were evaluated at the neonatal period, whereas 9 patients (36%) were evaluated after the neonatal period. All 25 patients received at least 1 plain abdominal radiograph or barium enema at the university hospital before operation.However, there was no specific pathognomonic finding that may provide a definite diagnosis. Nineteen (76%) patients underwent initial laparotomy at our institute and 6 patients (24%) were operated on beforehand at other hospitals. Twenty-three (92%) patients were diagnosed as having TCA and underwent ileostomy, whereas 2 (8%) patients underwent primary pull-through procedure. Eighteen (72%) patients had undergone definite surgery. Pre- and postoperative complications included enterocolitis (44.4%), perianal excoriation (77.7%), electrolyte imbalance (50%), and anastomotic leak (16.6%). Average duration of total parenteral nutrition before operation was 17.77 ± 12.54 days and after operation was 10.27 ± 5.23 days. Mean follow-up time was 27.6 ± 35.39 months. Two patients had 5 to 6 bowel movements per day. Seven had a frequency of stool ranging between 1 and 3 bowel movements per day. Their bowel movements returned to normal about 12 to 18 months after surgery. On follow-up, the height and weight development of the patients was found to be normal.

Conclusions

Gradual progress was observed in all the patients that took part in the study, and all patients had positive results eventually.     

Comprehensive review of procedures for total colonic aganglionosis

Purpose

A variety of procedures have been described for the treatment of total colonic aganglionosis (TCA); however, there is no consensus as to a superior operative method. The objective of this review is to evaluate the operative methods used to treat TCA and their effectiveness with respect to morbidity, mortality, rates of enterocolitis, and functional outcomes.

Methods

We searched Medline (1950-2007) and the Cochrane Library for studies reporting the operative treatment of TCA. Search terms used were “aganglionosis,” “Hirschsprung's disease,” “total colonic,” and “long-segment.” Studies evaluated were limited to those that solely discussed the operative outcomes for the treatment of TCA. A meta-analysis was then performed for morbidity outcomes in those studies describing a single operative procedure.

Results

The search yielded 271 articles related to TCA. One hundred twenty-two articles discussed operative treatment of Hirschsprung's disease, and only 26 of these articles discussed operative management of TCA. Nineteen articles were isolated for statistical analysis on the basis of results for perioperative outcomes for a single procedure. These procedures included Martin (4), colonic patch modifications (4), Duhamel (2), endorectal pull-through (1), Kimura (2), Boley (2), Rehbein (2), Soave (1), and J-pouch ileoanal anastomosis (1). Overall mortality was 1.9%. Meta-analysis revealed morbidity rates ranging from 10% to 64% depending on procedure category without significant outliers. Higher morbidity rates were found for the Martin-type procedure using the descending colon but with noted higher within-group variance due to small sample sizes. Enterocolitis was noted in 7 studies with a mean of 22%.

Conclusions

There is no superior operative method for the treatment of TCA with respect to perioperative morbidity, mortality, enterocolitis, and functional outcomes. The operative technique performed should be selected on center and surgeon familiarity and expertise.     

Total colonic Hirschsprung's disease: a 28-year experience

Purpose

The aim of this study was to review outcomes after surgical treatment of total colonic Hirschsprung's disease (TCH).

Methods

Twenty-five records of patients with TCH treated between 1974 and 2002 were reviewed. Follow-up data were collected using a standardized questionnaire. Objective functional outcome was assessed using a scoring system.

Results

Twenty patients had aganglionosis of the colon and distal ileum, 5 of whom had a more extensive condition. One of these 5 patients underwent an endorectal pull-through (ERPT), 1 underwent intestinal transplantation, and 3 died. Four of the remaining 20 patients underwent a primary ERPT, 16 received a stoma as neonates followed by ERPT in 12, and a Martin-Duhamel procedure or Swenson's operation in 3 (median age, 10.5 months); 1 remains with an ostomy. Postoperative complications included enterocolitis (55%), anal stricture (25%), and perineal excoriation (20%). Mean follow-up were 17.5 years (±11.1 years). Eighty-nine percent were free of recurrent enterocolitis. Frequency of bowel movements is 1 to 5 per day in 82% of the patients, 18% have 6 or more bowel movements per day. Occasional soiling is noted in 40% (one third of those requiring nighttime diapers). Overall functional outcome was good in 83%. Those patients with the longest follow-up periods had the best stooling scores (P = .04).

Conclusions

Surgical treatment of TCH is associated with a number of complications including recurrent enterocolitis and anal strictures. Long-term outcome is quite favorable.     

Reoperations in Hirschsprung disease

Background

We sought to identify causes of preventable complications related to operations for Hirschsprung disease.

Methods

We reviewed the cases of 51 patients with Hirschsprung disease who underwent a primary procedure elsewhere, had a complication, and were referred for reoperation.

Results

Thirty-five patients had 1 failed operation, 10 had 2, and 6 had 3. Initial operations were Soave (20), Duhamel (15), Swenson (5), transanal endorectal (4), myectomy (3), unknown (3), and laparoscopic Swenson (1). Thirty-one patients presented with a stoma. Patients without a stoma (20) had fecal impaction (8), recurrent enterocolitis (6), and fecal incontinence (6). None had both enterocolitis and incontinence. Reoperation was performed posterior sagittally (40) or transanally (5). Indications included stricture (21), megarectal Duhamel pouches (12), fistulae (11 [8 rectocutaneous, 2 rectourethral, and 1 rectovaginal]), pouchitis (2), and retained aganglionic bowel (8). After reoperation, 14 were continent, 11 had a stoma (8 permanent), 6 had voluntary bowel movements but soiled occasionally, 6 received rectal irrigations to avoid enterocolitis, 6 were incontinent but clean with bowel management, and 2 were lost to follow-up.

Conclusion

Stricture, megarectal pouch, fistula, and retained aganglionic bowel are preventable complications. Enterocolitis is partially preventable but can occur after a technically correct procedure. Fecal incontinence is a preventable complication likely because of anal canal damage.     

Posterior myotomy/myectomy for persistent stooling problems in Hirschsprung's disease

Background

Hirschsprung’s disease (HD) patients after pull-through (PT) may have recalcitrant constipation or recurrent enterocolitis (EC). Posterior myotomy/myectomy (POMM) are possible options for these problems. This study analyzed the outcome of POMM in HD patients post-PT.

Methods

Records of 348 HD patients were reviewed, and 32 were found to have undergone a POMM post-PT (1981 to 2002). Outcomes after this procedure were assessed. Statistics used linear and logistic regression.

Results

Of the 32 patients, 29 had complete records for analysis. Of those with pure constipation (12), 6 had aganglionosis post-PT. Of those with recurrent EC (17) only 1 had aganglionosis post-PT. POMM was performed at a mean of 3.1 years post-PT (11 myotomy, 18 myectomy). Average follow-up was 8.6 years (range, 0.7 to 21). Type of POMM had no correlation with overall functional outcome (P = .44). Of those with chronic constipation, 60% had good results after POMM; the remainder required a redo-PT or colostomy. Interestingly, most patients with retained aganglionosis and chronic constipation did not respond after POMM (83%). Of those with recurrent EC, 75% became free of symptoms; none of the patients not responding have required redo-PT.

Conclusions

POMM to treat chronic constipation or recurrent EC in patients with HD post-PT is moderately successful. Because of the unsuccessful outcome with POMM in patients with a combination of constipation and aganglionosis, one should defer to a redo-PT in this group.     

Murine model of Hirschsprung-associated enterocolitis. I: Phenotypic characterization with development of a histopathologic grading system

Purpose

The aim of the study was to characterize enterocolitis in the Ednrb-null (Ednrb−/−) mouse with aganglionosis of the colon and to develop and validate a semiquantitative histopathologic grading system to assess enterocolitis.

Methods

We isolated colon and ileal specimens of Ednrb−/− and control mice (Ednrb+/+) and performed histochemical staining (H&E) on tissue sections. After establishing inflammation grading criteria, 2 blinded pathologists independently assessed the severity and depth of inflammation of proximal colon segments on 2 separate occasions. Interclass correlations (ICCs) and coefficient of variation (CV) were calculated to determine interrater and intrarater agreement. We then prospectively applied the enterocolitis grading system to Ednrb−/− mice that became clinically ill. A cohort of Ednrb−/− mice were observed until they developed clinical illness, at which time they were euthanized and had multiple organ homogenates cultured for bacteria, and colon and small bowel were histopathologically graded for enterocolitis. Spearman's rank correlations comparing enterocolitis scores with level of bacteremia were performed.

Results

Intra- and interrater ICCs of the histologic scoring system were satisfactory (0.61 and 0.94, respectively), as were intra- and interrater CVs (18% and 9%, respectively). Of the Ednrb−/− mice, 65% developed bacteremia. Those with bacteremia had significantly higher enterocolitis scores than those without bacteremia (P < .01). Ednrb−/− mice that developed bacteremia showed a strong positive correlation between total enterocolitis scores and number of bacterial colony forming units in peritoneal lavage, liver, kidney, and aerobic spleen.

Conclusions

The Ednrb−/− mouse with aganglionosis develops enterocolitis and has features similar to Hirschsprung-associated enterocolitis in humans. Our grading system is a reliable way to assess enterocolitis. By performing microsurgical pull-through, we can now perform controlled, hypothesis-driven, mechanistic studies to evaluate etiologic factors affecting enterocolitis in the Ednrb−/− mouse.     

Endorectal pull-through for Hirschsprung's disease—a multicenter, long-term comparison of results: transanal vs transabdominal approach

Purpose

Previous studies have reported decreased continence in patients undergoing transanal endorectal pull-through (TERP) for Hirschsprung's disease compared to the older transabdominal approach (TAA). To address this, we examined long-term stooling outcomes in a large, multicenter cohort of patients undergoing either TERP or TAA.

Methods

Data were collected from 5 large pediatric institutions. Patient families were surveyed using a stooling score system (0-40, best to worst total score). Inclusion criteria included patients older than 3 years and those who had more than 6 months of recovery after pull-through. Those with total colonic aganglionosis were excluded. Statistical analysis included univariate and multivariate linear regression (significance, P < .05).

Results

Two hundred eighty-one patients underwent TERP (192) or TAA (89). Interviews were completed in 149 (104 [52%] TERP vs 45 [52%] TAA). The TAA group had a significantly greater number of daily bowel movements for each respective postoperative year and experienced more early complications (3% vs 1% with >1 complication; P = .061) and late complications (19% vs 4% with >1 complication; P < .001). Although the TAA group had a higher mean enterocolitis score (3.3 ± 0.4 vs 1.8 ± 0.2; P < .001), this was not borne out by multivariate regression analysis (P = .276). Parental survey showed that there were no significant differences between procedures in mean total, continence, or stooling pattern scores.

Conclusion

Transanal endorectal pull-through was associated with fewer complications and fewer episodes of enterocolitis. In contrast to prior studies, TERP patients did not have a higher rate of incontinence. These results support use of TERP as an excellent surgical approach for children with Hirschsprung's disease.     

Inflammatory changes secondary to postoperative complications of Hirschsprung's disease as a cause of histopathologic changes typical of intestinal neuronal dysplasia

Purpose

The aim of this study was to clarify the pathogenesis of intestinal neuronal dysplasia (IND).

Methods

The bowel habits of 36 postoperative HD patients were assessed retrospectively. Twenty-five had no complaints. Seven had persistent enterocolitis and were the focus of our study. They were divided into group A (n = 2) if they were severe and had associated postoperative surgical complications, and group B (n = 5) if they were mild. The histological changes were assessed.

Results

The 7 patients who had persistent enterocolitis postoperatively had no AchE activity in the mucosa, and there was normal distribution of submucosal and myenteric ganglia in the proximal resection margin. Rectal biopsies performed postoperatively for investigation of persistent enterocolitis in group A showed inflammatory changes and typical histopathologic features of IND such as abundant acetylcholinesterase (AchE)-positive nerve fibers in the lamina propria associated with giant submucosal ganglia and hyperganglionosis, and in group B there was increased AchE activity without hyperganglionosis or giant ganglia.

Conclusions

This is the first report of histopathologic changes typical of IND occurring in response to persistent enterocolitis related to postoperative complications of surgery for HD.     

Long-term surgical outcomes in congenital diaphragmatic hernia: observations from a single institution

Background/Purpose

Surgical complications are common in survivors of congenital diaphragmatic hernia (CDH), but little is known about long-term incidence patterns and associated predictors.

Methods

A cohort of 99 CDH survivors was prospectively followed at a single-institution multidisciplinary clinic. Data were gathered regarding the adverse surgical outcomes of hernia recurrence, chest and spinal deformity, and operative small bowel obstruction (SBO), and then were retrospectively analyzed in relation to perinatal and perioperative markers of disease severity to determine significant predictors. Statistical methods used included univariate and multivariate regression analysis, hazard modeling, and Kaplan-Meier analysis.

Results

At a median cohort age of 4.7 (range, 0.2-10.6) years, 46% of patients with patch repairs and 10% of those with primary repairs had a hernia recurrence at a median time of 0.9 (range, 0.1-7.3) years after repair. Chest deformity was detected in 47%. Small bowel obstruction and scoliosis occurred in 13%. Recurrence and chest deformity were significantly more common with patch repair, liver herniation, age at neonatal extubation greater than 16 days, oxygen requirement at discharge, and prematurity. The strongest predictor of SBO was patch repair. Multivariate analysis showed that patch repair was independently predictive of recurrence and early chest deformity (odds ratios of 5.0 and 4.8, confidence intervals of 1-24 and 1-21, P < .05). Use of an absorbable patch was associated with the highest risk of surgical complications.

Conclusions

For long-term survivors of CDH, specific perinatal and operative variables, particularly patch repair, are associated with subsequent adverse surgical outcomes.     

Role of transanal endorectal pull-through in complicated Hirschsprung's disease: experience in 18 patients

Background/Purpose

In Hirschsprung's disease (HD) redo pull-through (PT) is indicated for anastomotic complications and for persistent aganglionosis after previous definitive surgery. This study was undertaken to evaluate the role of transanal approach to redo PT procedure in the management of complicated cases of HD over the last 7 years.

Patients and Methods

Between November 1998 and September 2005, 225 patients with HD were operated using the transanal endorectal PT (TEPT) approach. Eighteen patients had a redo PT owing to persistent aganglionosis.The present study evaluates the role of TEPT approach in patients with persistent aganglionosis (n = 18). Three patients needed a colostomy (n = 2) or ileostomy (n = 1) before the final operation. All the 18 patients underwent transanal mobilization (TEPT) of the colon. Six patients required additional mobilization of the proximal colon (n = 4) and the ileum (n = 2) during the redo PT operation.

Results

Median follow-up was 43 months (range, 3-72 months). Sixteen patients have had a good outcome with stool pattern 1 to 4 times daily. One patient had obstructive symptoms for 4 months postoperatively but then settled. One patient has occasional soiling.

Conclusions

In this series, TEPT and posterior midline split of the muscle cuff were used with good results. This has the advantage of avoiding injury to the pelvic muscles and nerves. The TEPT approach is combined with transabdominal mobilization of the intestine depending on the length of the aganglionic segment. The outcome has been favorable, but long-term follow-up is necessary for full assessment of those patients.     

Prophylactic glycopyrrolate prevents bradycardia after spinal anesthesia for Cesarean section: a randomized, double-blinded, placebo-controlled prospective trial with heart rate variability correlation

Study Objective

To determine if prophylactic glycopyrrolate prevents bradycardia after spinal anesthesia.

Design

Prospective, randomized, double-blinded, placebo-controlled study.

Setting

Large university-affiliated community hospital.

Patients

81 consecutive term parturients (not in active labor) who were scheduled for elective Cesarean section.

Interventions

Parturients received 1.0 to 1.5 L of intravenous Ringer's lactate and either glycopyrrolate 0.4 mg or an equal volume of saline, with caregivers blinded to the immediate sequelae of study drug. Each patient received intrathecal bupivacaine (12 to 14 mg) with morphine sulfate (0.1 to 0.2 mg).

Measurements

Continuous heart rate (HR) and blood pressure monitoring occurred for 20 minutes, with the minimum HR recorded for each 5-minute epoch. Heart rates < 60 beats per minute defined bradycardia. Heart rate variability (HRV) analysis occurred offline.

Main Results

None of 34 patients administered glycopyrrolate and 6 of 35 (17%) patients receiving saline experienced bradycardia (P = 0.02476). Time domain, frequency domain, and nonlinear and embedded spectrum entropy analyses all reflected the decrease in HRV accompanying administration of glycopyrrolate.

Conclusion

Bradycardia after spinal anesthesia occurs commonly. Prophylactic glycopyrrolate may prevent the bradycardia, but not necessarily the hypotension.     

Waardenburg syndrome with extended aganglionosis: report of 3 new cases

Purpose

The Waardenburg-Shah syndrome is an autosomal recessive disease with varied penetration where Hirschsprung's disease and the Waardenburg syndrome are seen together. Although the length of the involved intestinal segment varies in this syndrome, most patients had total colonic aganglionosis with or without small bowel involvement. We present in this study 2 siblings and one first-degree relative for a total of 3 male patients with Waardenburg syndrome and total colonic aganglionosis with or without small bowel involvement, together with their clinical characteristics and treatment methods.

Patients

The patients who presented with intestinal obstruction findings within the first 48 hours after birth were operated on with 2 patients under elective conditions and 1 as an emergency. The ganglionic segment lengths were 6, 8, and 20 cm, respectively. Aganglionic enterostomy was performed, and the Ziegler operation was used for these patients. The enterostomies started to function on the third postoperative week, and they started to gain weight. However, all died because of sepsis on the 5th to 12th month.

Conclusion

Waardenburg-Shah syndrome patients have a higher incidence of total colonic aganglionosis with or without small bowel involvement. The Ziegler operation may be used in patients with inadequate ganglionic bowel length to gain some time for the child to grow and to decrease total parenteral nutrition complications.     

The UK helicopter ambulance tasking study

Introduction

Helicopter ambulances are a scarce and expensive resource and their use carries significant risk for crew and patients.

Aims

To establish and compare the tasking criteria, dispatch arrangements and crew configuration for all helicopter ambulance services in the United Kingdom.

Methods

Structured telephone interview of all helicopter ambulance services in the United Kingdom.

Results

Replies were received from all 16 UK air ambulance services. Crew configuration varies between services. Nine services had paramedic only crew, 3 had physician/paramedic crew and 4 had a paramedic crew with variable physician input.Only 2 of the 16 services used a paramedic in the dispatch process.There were 67 different tasking criteria used for air ambulance dispatch across all air ambulances with a range from 4 to 23 for individual air ambulance services.

Conclusion

Given the financial burden and physical risk of air ambulance use, there should be a more standardised approach to the tasking, dispatch and crew configuration of air ambulances in the UK.