Hirschsprung Disease ; surgery ; Humans ; Laparotomy ; Postoperative Care ; Rectum ; surgery

Adult megacolon is a rare disease with heterogeneneous etiology. The treatment schemes of megacolon caused by different causes are also different, but surgery is the final and the most effective method. Due to the lack of early understanding of the disease, many patients have not been clearly diagnosed as adult megacolon and have not been properly treated. This article classifies adult megacolon according to the etiology and summarizes its surgical options. For adult Hirschsprung's disease, modified Duhamel, the Jinling procedure, low anterior resection, or pull-through low anterior resection can be used. For patients with idiopathic megacolon, one-stage subtotal colorectal resection can be selected with adequate preoperative preparations. Some patients admitted to the hospital with emergency intestinal obstruction can be treated with conservative treatment or decompression under colonoscopy followed by selective surgery. For patients with aganglionosis, the procedure is subtotal colorectal resection, the same as that of idiopathic megacolon. The procedure is to remove both the dilated proximal intestine and the stenotic distal intestine, then an ileorectal anastomosis or ascending colon rectal anastomosis is performed. For toxic megacolon, colostomy can be done for mild cases, and for severe infections, subtotal colorectal resection is required. Latrogenic megacolon is mostly caused by segmental stenosis or lack of peristalsis, resulting in chronic dilatation of the proximal end and the formation of megacolon. It is necessary to choose a reasonable surgical procedure according to the specific conditions of the patient. The first choice for the treatment of acute colonic pseudo-obstruction syndrome is decompression under colonoscopy. For those with the secondary changes in the intestine, ostomy is still the most effective surgical procedure, but should be performed with caution.
Anastomosis, Surgical ; Colostomy ; Hirschsprung Disease/surgery* ; Humans ; Megacolon/surgery* ; Rectum/surgery*

OBJECTIVETo investigate the surgical outcomes after on transumbilical laparoscopic pull-through procedure for pediatric hypoganglionosis(HYP).

METHODSTwelve children with HYP had received transumbilical laparoscopic pull-through procedure from June 2009 to June 2010. Specially designed curved and elongated laparoscopic instruments were used during the procedures. All the patients were followed up over 10 months. Data were collected and analyzed. The diagnosis of hypoganglionsis was pathologically confirmed.

RESULTSNo conversions to laparotomy or traditional laparoscopic surgery were required and there were no damages to the abdominal blood vessels, intestine, ductus deferens, or ureters. The average duration of operation was 140 min. The mean intraoperative blood loss was 45 ml. The mean length of specimen was 40 cm. Postoperatively there were no complications such as anastomotic leak, anastomotic stricture, constipation, seepage, or fecal in continence. The average hospital stay after surgery was 9 days. During 10 to 22 months of follow-up(median 16 months), no postoperative recurrence was noticed. No obvious scar was seen 1 months after surgery.

CONCLUSIONIt is safe and effective for children with hypoganglionosis to undergo transumbilical laparoscopic pull-through procedure.

Anal Canal ; surgery ; Child ; Child, Preschool ; Colon ; surgery ; Female ; Hirschsprung Disease ; surgery ; Humans ; Laparoscopy ; methods ; Male ; Treatment Outcome ; Umbilicus ; surgery

OBJECTIVETo evaluate the safety and efficacy of fast track surgery (FTS) combined with laparoscopy in the treatment of infant Hirschsprung disease.

METHODSClinical data of 72 infants with Hirschsprung disease undergoing elective pull-through surgery from June 2010 to June 2013 were retrospectively summarized. The patients were divided into two groups: fast track surgery combined with laparoscopy group (FTS group, n=33) and laparoscopic surgery with traditional management perioperatively (control group, n=39). Postoperative intestinal function recovery, hospital stay, cost of hospitalization, complications were compared and postoperative recovery was followed-up for four weeks.

RESULTSThere were no significant differences in intraoperative blood loss and operative time between FTS and control group (both P>0.05). The recovery of bowel movement was earlier in the FTS group but the difference was not statistically significant (P=0.078). The hospital stay was shorter [(10 ± 2) d vs. (14 ± 4) d] and cost of hospitalization was lower [(15 316 ± 2273) Yuan vs. (18 641 ± 3082) Yuan] in FTS group than those in control group(P<0.01). Postoperative complications and recovery conditions during 4 weeks follow-up were similar between the two groups.

CONCLUSIONFast track surgery combined with laparoscopy in the treatment of infant Hirschsprung disease is safe and effective.

Female ; Hirschsprung Disease ; surgery ; Humans ; Infant ; Laparoscopy ; Male ; Retrospective Studies ; Treatment Outcome

OBJECTIVETo examine the long-term outcomes of total colonic aganglionosis (TCA) and to evaluate their nutritional status.

METHODSEleven pediatric patients treated for TCA between January 1999 and December 2010 were included in the study and followed up. Physical measurements including height, weight and laboratory tests were assessed. Anorectal functions were evaluated with Kelly score and quality of life(QOL) using questionnaire.

RESULTSThe length of follow-up ranged from 8 to 147 months. The children had satisfactory anorectal function (Kelly score, 5-6). One child had a Kelly score of 3. The children who were followed up less than 48 months had significant higher Kelly scores compared with those with more than 48 months follow-up(P<0.05). QOL was good in nine patients (QOL score, 9-10) and moderate (score, 7-8) in 2 patients. Weight-for-age was normal in 2 patients, mild malnutrition in 6 patients, and moderate malnutrition in 3 patients. Height-for-age was normal in 6 patients, mild malnutrition in 3 patients, and moderate malnutrition in 2 patients. The serum albumin was(49.0±2.7) g/L in children with well-educated parents, significantly higher than those with poorly-educated parents(44.3±1.9) g/L(P<0.05).

CONCLUSIONSLong-term outcomes of children with TCA are satisfactory with good anorectal function and quality of life. Low body weight is more common than low height. Children with well-educated parents have better nutrition status.

Follow-Up Studies ; Hirschsprung Disease ; surgery ; Humans ; Infant ; Male ; Nutritional Status ; Treatment Outcome

OBJECTIVETo investigate the diagnosis and surgical treatment of adult Hirschsprung disease (AHD).

METHODSClinical data of 10 patients with AHD undergoing operation from May 1985 to May 2005 were analyzed retrospectively.

RESULTSThere were 7 males and 3 females with an age ranged from 14 to 40 years. All the cases had constipation, and were diagnosed by barium enema. Aganglionosis was located in distal sigmoid and rectum in 2 cases, in rectum in 7 cases, unknown in one case. Colostomy was performed in one, Ikeda s operation in 6, Rehbein operation in two,modified Swenson operation in one. After radical operation,7 patients had excellent continence function, one had good function, one had poor function.

CONCLUSIONSThe diagnosis of adult HD mainly depends on the history of constipation, barium enema and manometry examination. The pull-through procedures are effective surgical treatments for adult HD. The operation type should be selected individually.

Adolescent ; Adult ; Female ; Hirschsprung Disease ; diagnosis ; surgery ; Humans ; Male ; Young Adult

OBJECTIVETo investigate the diagnosis and surgical management of adult Hirschsprung's disease.

METHODSClinical data of 15 patients with adult Hirschsprung's disease were reviewed retrospectively from June 1992 to June 2004.

RESULTSPatients age ranged from 17 to 54 years old. The main manifestations included long-term (ranged from 9.5 month to 50 years) constipation and abdominal distention. Acute abdominal pain occurred in six patients, but no sign of de hydration and malnutrition occurred in all patients. Bowel stenosis and dilation could be examined by barium enema. Soave procedure was performed in 3 patients, subtotal colectomy with coloanal anastomosis was performed in twelve patients. The function of defecation was improved in all patients after operation.

CONCLUSIONSThe diagnosis of adult Hirschsprung's disease mainly depends on the history of constipation from infant and barium enema. Subtotal colectomy with coloanal anastomosis is an effective and safe operative procedure.

Adolescent ; Adult ; Female ; Hirschsprung Disease ; diagnosis ; surgery ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

OBJECTIVETo investigate the effect of partial internal sphincter myomectomy on transanal one-stage pull-through operation for Hirschsprung disease (HD).

METHODSA prospective group of 153 pediatric patients with HD in Guangdong Dongguan People's Hospital between 2003-2012 were enrolled, who underwent transanal one-stage pull-through operation. Children were divided into partial resection group (77 cases) undergoing partial internal sphincter myomectomy and simple incision group (76 cases) undergoing simply internal sphincter dissection, respectively. Differences of postoperative complications and continence between two groups were compared.

RESULTSPostoperative complications such as rectal muscularis infection [1.3% (1/77) vs. 11.8% (9/76), P<0.05], enterocolitis [2.6% (2/77) vs. 13.2% (10/76), P<0.05], anastomosis stenosis[3.9% (3/77) vs. 22.4% (17/76), P<0.01] and abdominal distension [10.4% (8/77) vs. 25.0% (19/76), P<0.05] were lower in partial resection group as compared to simple incision group. The time of antibiotics administration was also lower in partial resection group [(3.9±1.1) d vs. (4.6±1.1) d, P<0.01]. Difference in the continence between the two groups was not statistically significant (kelly score, 5.1±0.5 vs. 5.2±0.6, P>0.05).

CONCLUSIONSCompared with simply internal sphincter dissection in operation, partial internal sphincter myomectomy with transanal one-stage pull-through operation for HD can reduce the postoperative complications and does not increase the damage of the continence.

Adolescent ; Anal Canal ; surgery ; Child ; Child, Preschool ; Female ; Hirschsprung Disease ; surgery ; Humans ; Male ; Postoperative Complications ; Prospective Studies ; Treatment Outcome

Anal Canal ; surgery ; Child ; Child, Preschool ; Colon, Sigmoid ; surgery ; Digestive System Surgical Procedures ; methods ; Female ; Hirschsprung Disease ; surgery ; Humans ; Infant ; Laparoscopy ; Male ; Rectum ; surgery ; Treatment Outcome

OBJECTIVETo evaluate the efficacy, safety, and defecation after one-stage transanal endorectal pull-through(TOSEPT) for Hirschsprung disease.

METHODSClinical data of 56 patients with Hirschsprung disease undergoing TOSEPT in the Third Hospital of Guangzhou Medical College between 2005 and 2011 were retrospectively analyzed. According to age at operation, the patients were divided into newborn group(n=21, surgery performed within 1 month after birth) and non-newborn group(n=35). Recovery period was defined as the period required for normal defecation pattern after operation. Intraoperative and postoperative parameters were compared.

RESULTSThe mean operative time was(121.5±39.2) minutes. The mean length of bowel resection was(17.6±4.2) cm. The mean intraoperative blood loss was(34.6±5.2) ml. The mean postoperative hospital stay was(7.2±3.6) days. Postoperative complication occurred in 6 patients(4 had enteritis and 2 had recurrent constipation) in whom 1 were considered as failure of TOSEPT because of redo-surgery or persistent problems in defecation. The remaining 53 patients had normal defecation pattern after(9.2±5.8) weeks of postoperative recovery period. Neonatal cases had significantly shorter operative time and postoperative hospital stay, and longer postoperative recovery period than non-neonatal cases(P<0.05). There were no significant differences in intraoperative blood loss and postoperative complication rate between the two groups(P>0.05).

CONCLUSIONSTOSEPT is effective and safe in the management of patients with Hirschsprung disease. However, a postoperative recovery period is required for a normal defecation pattern. Although neonatal cases have significantly shorter operative time and postoperative hospital stay than non-neonatal cases, but longer postoperative recovery period should be consider when evaluating the outcome of TOSEPT.

Anal Canal ; surgery ; Child, Preschool ; Defecation ; Female ; Follow-Up Studies ; Hirschsprung Disease ; surgery ; Humans ; Infant ; Infant, Newborn ; Male ; Postoperative Period ; Rectum ; surgery ; Retrospective Studies ; Treatment Outcome