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.     

Functional outcome after operation for Hirschsprung disease—transanal vs transabdominal approach

Background

It has been hypothesized that the extensive transanal dissection in transanal endorectal pull-through (TEPT) for Hirschsprung disease (HD) can impair the anal sphincters in neonates and thereby cause incontinence. Theoretically, transabdominal endorectal pull-through might have less impact on the sphincters. The aim of this study was to compare functional outcome in HD patients operated with either TEPT or laparotomy-assisted endorectal pull-through (LEPT) with particular focus on soiling and fecal incontinence.

Patients and Methods

Anorectal function in 52 children older than 3 years is reported. The patients were operated for HD with either TEPT (n = 28) or LEPT (n = 24) and followed prospectively. Functional outcome was recorded by standardized interviews. The Krickenbeck criteria were used to classify voluntary bowel movements, soiling, and constipation.

Results

The median age at follow-up was 5.7 years (3.1-13.2) for TEPT and 10.1 years (7.7-16.2) for LEPT. Twenty-nine patients reported soiling at final follow-up. There was no difference in the rate of soiling between children operated with TEPT (54%) or LEPT (58%). Constipation was reported in 11 children (TEPT, 25%; LEPT, 17%).

Conclusions

The functional outcome and in particular the rate of soiling did not differ between patients operated with LEPT or TEPT.     

Duhamel operation vs neonatal transanal endorectal pull-through procedure for Hirschsprung disease: which are the changes for pathologists?

Background

The aim of this study was to evaluate whether performing definitive surgery for Hirschsprung disease (HD) in neonatal period with a transanal endorectal pull-through (TEPT) procedure had modified our diagnostic relevance, particularly during intraoperative frozen sections (IOFS), compared to classic Duhamel (DH) surgery performed in older children.

Methods

We collected pathologic data for 47 children who underwent surgery for neonatal nontotal HD over a 5-year period.

Results

Twenty-nine patients underwent TEPT and 18 the DH operation. Mean age at operation was 19 days for TEPT and 4 months for DH operation. The mean number of IOFS was 2.6 for TEPT and 2.4 for DH operation. Gross examination could be fully completed in all TEPT cases, but was incomplete in 5 DH cases. The average total lengths of bowel, and aganglionic, transitional, and ganglionic segments were 12.3, 7.3, 3, and 2 cm for TEPT, and 17.6, 9.3, 3.5, and 4.8 cm for DH operation, respectively. Discordance between IOFS and paraffin-section analysis occurred in 5 cases (3 TEPT and 2 DH operation).

Conclusion

When TEPT was used, the gross examination and sampling was more accurate, leading to a clearer pathology report. The TEPT procedure facilitates the work of the pathologist without modifying the results of IOFS, if some precautions are taken.     

Single-stage transanal endorectal pull-through for Hirschsprung's disease: perspective from a developing country

Background/Purpose

The aim of this study was to evaluate the feasibility, results, and cost-effectiveness of totally transanal endorectal pull-through (TEPT) in the management of rectosigmoid and midsigmoid Hirschsprung's disease (HD) in a low-income country.

Methods

Between March 2004 and December 2005, 19 children underwent totally TEPT procedure. The patients' ages ranged from 6 days to 13 years. The primary diagnosis in all 19 patients was HD confined to the rectosigmoid region in 15 and midsigmoid in 4. None had a preoperative colostomy. Follow-up period ranged from 4 to 20 months (mean, 8 months).

Results

Ages ranged from 0.25 to 65 months, with a mean of 16.24 months. Weights ranged from 3.4 to 13 kg, with a mean of 6.5 kg. Mean time from diagnosis to pull-through procedure was 26 days (range, 6-39 days). The mean length of rectosigmoid resection was 30 cm (range, 20-50 cm). The mean operative time was 95 minutes (range, 75-140 minutes). Mean intraoperative blood loss was 25 mL (range, 15-40 mL). There was one death unrelated to the procedure. One patient had enterocolitis 3 months postoperatively. Average frequency of defecation was 3 (range, 1-6) stools per day. TEPT was associated with a shorter operating time, less blood loss, early return to feeds, and an overall reduced cost.

Conclusion

The safety and cost-effective benefits of transanal endorectal pull-through in the treatment of HD are of special interest for a developing country. Our data also suggest that functional outcome following TEPT is highly satisfactory and comparable with other established procedures.     

Clinical outcome in children after transanal 1-stage endorectal pull-through operation for Hirschsprung disease

Background/Purpose

Recently, the transanal 1-stage pull-through operation has been widely used in Hirschsprung disease (HD), and it is obviously superior to traditional approach in early term for its noninversion. However, the procedure is relatively so new that it makes assessment of the functional outcome and stooling patterns difficult. The aim of this study was to evaluate the clinical outcomes of the transanal 1-stage endorectal pull-through operation in the management of rectosigmoid HD.

Methods

Fifty-eight children (39 boys and 19 girls) aged 12 months to 13 years (mean, 2 years) who underwent transanal 1-stage endorectal pull-through operation for HD were followed up from 6 to 24 months. Clinical outcome was assessed by interviews and questionnaires. All patients had an aganglionic segment confined to the rectosigmoid area which was confirmed by the preoperative barium enema and postoperative pathological examination.

Results

Forty-six patients had satisfactory results without complications. In all the children, the mean stool times were 1 to 2 per day; only 4 had mean stool times of 8 to 10 per day. Postoperative soiling was present in 9, constipation in 5, and HD-associated enterocolitis in 3. There were no incontinence, cuff infection, anastomotic leak, and mortality in any of the patients. In the 12 symptomatic patients, there were 4 children with length of aganglionic segment less than 30 cm, and 8 had 30 cm or more. In the 46 asymptomatic patients, 42 had length of aganglionic segment less than 30 cm, and 4 had 30 cm or more. There was a significant difference between the group with less than 30 cm and the group with 30 cm or more of aganglionic segment. For statistical analysis, the Fisher exact test showed P < .05.

Conclusions

The transanal 1-stage endorectal pull-through is a feasible and safe procedure in children with rectosigmoid HD. The clinical outcome is satisfactory. A gradual recovery could be noted in the stooling patterns along with the time after surgery. The younger the patient operated on and the shorter the aganglionic segment, the lower do the stooling disorders occur and the faster does the stooling function recover.     

A follow-up study on postoperative function after a transanal Soave 1-stage endorectal pull-through procedure for Hirschsprung's disease

Objective

The aim of the study is to appraise bowel movements in children with Hirschsprung's disease (HD) after a transanal Soave 1-stage endorectal pull-through (TAS) procedure.

Methods

From October 2000 to October 2004, 44 patients with HD underwent a TAS procedure, 35 had a Soave operation via a laparotomy, 29 underwent a Soave procedure via laparoscopy, and 39 had an Ikeda-Soper procedure; the patients were followed up from 1 to 5 years after surgery. Evaluation of the perioperative therapeutic effect and postoperative bowel movements between the 4 groups were analyzed by Kelly's score and anorectal manometry.

Results

The TAS procedure required less operative time and costs than the Soave procedure via laparotomy or laparoscopy (P < .05) and less than that of Ikeda-Soper procedure for the duration of the preoperative preparation, the duration of the surgical procedure, intraoperative blood transfusions, days of postoperative fasting, antibiotic use, and days and cost of hospitalization (P < .01). There were no differences in short-term and long-term complications between the same Soave procedures; the Soave procedure exceeded the Ikeda-Soper operation in the incidence of enterocolitis 3 months postoperatively (P < .05), but there was no difference between the TAS procedure and the Ikeda-Soper procedure. There was no difference in bowel movements 12 months postoperatively and the rectal anal inhibitory reflex, high-pressure zone length, resting anal canal pressure, and the sensation threshold 1 year postoperatively between the TAS procedure and the Ikeda-Soper procedure, but the active contractile pressure was lower after the TAS procedure than the Ikeda-Soper procedure.

Conclusions

The TAS procedure is more suitable than the Soave operation via laparotomy or laparoscopy and Ikeda-Soper procedure and is feasible in infants with short segment type and common type HD.     

Transanal endorectal pull-through in children with Hirschsprung's disease—technical refinements and comparison of results with the Duhamel procedure

Purpose

Transanal endorectal pull-through (TEPT) has drastically changed the treatment of Hirschsprung's disease (HD). A short follow-up of children submitted to TEPT reveals results that are similar to the classic transabdominal pull-through procedures. However, few reports compare the late results of TEPT with transabdominal pull-through procedures with respect to complication rates and the fecal continence. The aims of the present work are to describe some technical refinements that we introduced in the procedure and to compare the short and long-term outcome of TEPT with the outcomes of a group of patients with HD who previously underwent the Duhamel procedure.

Methods

Thirty-five patients who underwent TEPT were prospectively studied and compared to a group of 29 patients who were treated with colostomy followed by a classical Duhamel pull-through. The main modifications introduced in the TEPT group were no preoperative colon preparation, operation conducted under general anesthesia in addition to regional sacral anesthesia, use of only one purse-string suture in the rectal mucosa before transanal submucosal dissection, and no use of retractors and electrocautery during the submucosal dissection.

Results

The most frequent early complications of TEPT group were perineal dermatitis (22.8%) and anastomotic strictures (8.6%). The comparison with patients who underwent Duhamel procedure revealed no difference in the incidence of preoperative enterocolitis, the patients of the TEPT group were younger at the time of diagnosis and of surgery, they had shorter operating times, and they began oral feeding more quickly after the operation. The incidence of wound infection was lower in the TEPT group. Moreover, the TEPT and Duhamel groups showed no difference in the incidences of mortality, postoperative partial continence, and total incontinence. Although the incidences of complete continence and postoperative enterocolitis were not different, a tendency to the increased incidence in the TEPT group was observed.

Conclusions

This study further supports the technical advantages, the simplicity, and the decreased incidence of complications of a primary TEPT procedure when compared to a classical form of pull-through. Some technical refinements are described, and no preoperative colon preparation was necessary for the patients studied here. The results show that the long-term outcomes of the modified TEPT procedure are generally better than those obtained with classical approaches.     

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.     

Extended left hepatectomy of the left and middle hepatic venous drainage areas along the anterior fissure

Background

Extended left hepatectomy including the middle hepatic vein (MHV) may potentially induce right paramedian sector congestion of the remnant liver.

Methods

To prevent venous congestion in the right paramedian sector, we performed extended left hepatectomy including the left hemiliver and anterior segment, which drain into the MHV and left hepatic vein (LHV), for 15 patients.

Results

In 11 of 15 patients (73%), temporary clamping of the common trunk of the MHV and LHV and the proper hepatic artery provided the anterior fissure. Regeneration rate of the middle segment was similar to that of the right lateral sector (10.8% vs 11.2%) on postoperative computed tomography (CT) after 3 months.

Conclusions

This procedure could represent a useful method for preventing postoperative venous congestion.     

A new method to close an intestinal wall defect using fibrin glue and polyglycolic acid felt sealant

Purpose

This study aimed to propose an alternative method of suture closure for an intestinal wall defect using 2 absorbable materials.

Methods

An oval intestinal wall defect was created in the duodenum, ileum, or colon of rabbits. The defect was first covered by polyglycolic felt, which was then completely covered by fibrin glue to make a fibrin glue and polyglycolic acid felt (FGPAF) sealant without any suture procedures. The rabbits with a simple suture closure for the defect were used as controls. The bursting pressure of the treated intestine was measured, and macro- and microscopic observations were carried out for 6 months.

Results

Seven rabbits treated with the FGPAF sealant used in each of the 3 operated regions survived without any signs of peritonitis or intestinal obstruction, similar to the controls. The mean bursting pressure of the segment with wall defect closed by single layer sutures and the segment with FGPAF sealant was 69.7 mm Hg (n = 6) and 70.6 mm Hg (n = 7), respectively. A histologic study at 1 week after operation revealed that the FGPAF circumferentially adhered to the edge of the defect with fibrous tissue extension into the sealant; whereas when performing a laparotomy at 1 month after operation, a round mass consisting of the remnant FGPAF mixed with plant residues of daily chows was found loosely attached to the serousal surface.

Conclusion

These results suggest that the present technique may be useful for the new technique of intestinal wall closure.     

Modified Soave procedure through the posterior sagittal approach for Hirschsprung's disease

Purpose

The objective of this study is to describe the surgical technique and clinical results of a modified Soave procedure through the posterior sagittal approach (PSAP) for Hirschsprung's disease.

Methods

The patient was placed in a prone jackknife position. The rectum was approached via PSAP keeping the external sphincter intact. The rectum was isolated and then divided approximately 2 cm from the dentate line. The rectum and sigmoid were freed up to the normal segment. The aganglionic and ganglionic segments were confirmed intraoperatively by frozen biopsy. The rectal mucosectomy was started 0.5 to 1 cm proximal to the dentate line, leaving 1 cm of the muscular cuff. The colon was then pulled through the anus. The aganglionic segment and transitional zone were removed. Coloanal anastomosis was fashioned 0.5 to 1 cm proximal to the dentate line.

Results

Ninety-six patients were operated on from January 2000 to July 2002, using the described technique. Patient ages ranged from 2 to 36 months (median age, 8½ months). The aganglionic segment was located in the rectum in 56 patients and in the sigmoid colon in 40 patients. The average length of bowel resected was 20 cm (range, 10-35 cm). Combined laparotomy was performed in 2 patients and combined laparoscopy surgery in 1 infant. There were no operative or postoperative deaths. Anastomotic leakage occurred in 2 patients. Spontaneous defecation occurred in all patients.

Conclusions

Posterior sagittal approach provides excellent exposure of the operative field and decreases the major complications seen with the abdominal approach. The modified Soave procedure can easily be performed with PSAP.     

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.     

Early and late outcomes of primary laparoscopic endorectal colon pull-through leaving a short rectal seromuscular sleeve for Hirschsprung disease

Aim

To report early and late outcomes of laparoscopic colon pull-through leaving a short rectal sleeve for Hirschsprung disease.

Methods

Laparoscopic endorectal colon pull-through was performed using 4 ports. The ganglionic and aganglionic segments were initially identified by seromuscular biopsies obtained laparoscopically. The rest of the procedure was carried out according to Georgeson's technique. However, we left a short rectal seromuscular sleeve of 1.5 to 2 cm above the dentate line.

Results

From January 2001 to December 2007, 200 patients were operated upon by the same surgeon. Ages ranged from 14 days to 36 months old. The aganglionic segment was located in the rectum in 112 patients, in the sigmoid colon in 80 children, and in the left colon in 8 patients. The median operating time was 152 minutes. There were no perioperative deaths. Conversion to open surgery was required in four patients. There was minimal blood loss during the surgery. Oral intakes of clear fluid were started 12 hours after surgery and advanced to formula on the second day. In 1 patient, a small intestinal perforation occurred 3 days after surgery, requiring a diverting ileostomy. The mean hospital stay was 6.6 days (range, 4-12 days). Follow-up ranging from 5 to 85 months was obtained in 157 patients; 124 patients (79%) had 1 to 4 defecations a day, 17 (11%) had 5 to 6, and 8 had more than 6. Fecal incontinence occurred in 3 patients (2.0%), constipation in 5 patients (3.0%), and enterocolitis in 15 patients (9.5%). Anastomotic fistula occurred in 2 patients.

Conclusion

Laparoscopic endorectal pull-through leaving a short rectal seromuscular sleeve is a safe and effective procedure for Hirschsprung disease.     

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.     

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.     

Primary gastric tumors of infancy and childhood: 54-year experience at a single institution

Background/Purpose

Primary gastric tumors are rare in infancy and childhood. Because of the infrequent occurrence of these tumors, the clinician may be unfamiliar with optimal management strategies. We review our experience over the past 54 years and the current literature.

Methods

During the period extending from 1952 to 2006, 21 infants and children with primary gastric tumors were treated at Children's Hospital Los Angeles. The series includes 8 cases previously reported and 13 additional cases seen since the initial report. Follow-up information is included.

Results

There were 12 males and 9 females, aged 12 days to 18 years, who were diagnosed with gastric tumors. The patients presented primarily with weight loss, vomiting, or an abdominal mass. Morphological analysis revealed gastric stromal tumors (n = 6), teratomas (n = 4), lymphomas (n = 4), adenocarcinomas (n = 2), inflammatory myofibroblastic tumors (n = 2), embryonal rhabdomyosarcoma (n = 1), and hamartomas (n = 3). There were 16 patients still alive (mean follow-up, 22.3 months), whereas 6 died from active disease despite multimodal treatment. The deaths occurred in patients with stromal tumors, adenocarcinomas, lymphomas, and rhabdomyosarcoma.

Conclusions

Gastric tumors in children are rare. A high index of suspicion is needed to diagnose these tumors. Most malignant tumors present at an advanced stage and carry a substantial rate of mortality. They should be completely resected whenever feasible. In the case of some malignancies, chemotherapy may play a major role. Metastatic evaluation should be performed in all patients with malignant gastric tumors.     

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 bowel function and quality of life in children with Hirschsprung's disease

Background/Purpose

Little is known about the quality of life (QOL) of children with Hirschsprung's disease (HD) as they grow older. The purpose of this study was to measure the QOL and bowel function of these children as they mature.

Methods

All children who were surgically treated for HD at British Columbia Children's Hospital, Vancouver, British Columbia, Canada between 1986 and 2003 were invited to participate. Each family was sent 3 previously validated questionnaires exploring current QOL and bowel function.

Results

Fifty-one families participated (49%), with children between the ages of 3 and 21 years. Fecal continence improved significantly with age (P = .04) and was the strongest predictor of QOL scores of all variables in our study. There was no statistically significant difference in QOL scores between children with HD and healthy children, although a clinically relevant impairment in QOL may be present, especially in psychosocial scores.

Conclusions

Fecal continence is an important predictor of overall QOL in children surgically treated for HD. Although continence tends to improve with age, a number of older children still have ongoing continence problems, and they seem to be a group at risk for impaired QOL. Our study indicates that interventions for children with incontinence may offer gains in QOL as well as bowel function.     

Persistent obstructive symptoms after surgery for Hirschsprung's disease: development of a diagnostic and therapeutic algorithm

Background

Although most children with Hirschsprung’s disease (HD) do well after pull-through surgery, some continue to have persistent obstructive symptoms that may lead to significant morbidity. The author’s goal was to develop an organized algorithm for the workup and ultimate management of these symptoms.

Methods

All children referred to the author with severe obstructive symptoms after a pull-through for HD were evaluated using an algorithm based on 5 potential etiologies, and appropriate therapeutic interventions were instituted.

Results

A total of 49 children were evaluated over 7 years. Mechanical obstruction was found using rectal examination and barium enema in 7, of which, 2 had resection of a Duhamel spur, and 5 had a repeat pull-through (after failed dilatation). Rectal biopsy results showed aganglionosis in 10, of whom, 8 underwent repeat pull-through, and 2 refused further surgery. Abnormal intestinal motility or intestinal neuronal dysplasia was found proximal to the aganglionic segment in 10 children using colonic and small bowel manometry or laparoscopic biopsies; 4 of these had additional colon resected and repeat pull-through, and 6 were treated with a bowel management routine, cecostomy, or stoma formation. Internal sphincter achalasia was felt to be the primary cause of symptoms in 14, and all were treated with intrasphincteric botulinum toxin. Eight were found not to fall into any of the above groups and were treated medically for presumed functional megacolon; 2 of these ultimately had a colostomy.

Conclusions

An organized approach to the diagnosis and management of obstructive symptoms in a child after a pull-through for HD permits accurate diagnosis and effective treatment in the majority of cases.     

Spanish and Latin American Nursing Personnel and Deceased Organ Donation: A Study of Attitude

Objective

To analyze the attitude of nursing personnel about organ donation and transplantation in hospitals in Spain and Latin America, and factors that affect this attitude.

Methods

Data were selected from 12 hospitals and 32 primary care centers participating in an international study (Proyecto Donante, Murcia) in 4 countries including Spain (n = 650), Mexico (n = 428), Cuba (n = 89), and Costa Rica (n = 27). The sample was random and stratified by type of service among nursing personnel (n = 1194). Attitude was evaluated using a psychosocial questionnaire.

Results

Of nursing personnel surveyed, 77% (n = 922) were in favor of organ donation. No differences were found according to whether they were directly involved in transplantation-related services (P < .05). Attitude in favor of organ donation varied between countries: 92% in Cuba, 85% in Costa Rica, 80% in Mexico, and 73% in Spain (P < .001) This attitude was also related to donation of a family member's organs (P < .001), having discussed organ donation and transplantation within the family (P < .001), the concept of brain death (P < .001), fear of body mutilation (P < .001), and manipulation of the body after death (P = .001).

Conclusion

Attitude toward deceased organ donation among nurses varies between countries. There is a discrepancy between those in favor vs actual donation rates in countries and work centers. These fears may become worse when donation is seen as common in daily clinical practice.