Laparoscopic Soave Research Articles

Mowat-Wilson syndrome associated with Hirschsprung disease

Mowat-Wilson syndrome (MWS) is a rare autosomal dominant genetic disease caused by mutations in the zinc finger E-box binding homeobox 2 (ZEB2) gene. The syndrome is associated with Hirschsprung disease (HSCR). To investigate and report the clinical characteristics of MWS associated with HSCR and the treatment outcome of patients after radical operation for HSCR. The clinical features and gene mutation results of a patient with MWS and HSCR diagnosed by ZEB2 genetic analysis were literature review. The patient was female and presented abdominal distension, constipation, patent ductus arteriosus and a distinctive facial appearance, including ocular hypertelorism, prominent forehead and nasal tip, low-set ears, and uplifted earlobes. The patient was pathologically diagnosed with HSCR after undergoing laparoscopic Soave procedure at 9 months old. After surgical treatment, the defecation function returned to normal until the follow-up to 1 year and 10 months of age. A heterozygous mutation in the ZEB2 gene on chromosome 2 of the patient results in amino acid change c.244G > T (p.E82X), which is an unreported new mutation. Gene examination should be performed in children with HSCR combined with appearance changes. c.244G > T (p.E82X) may be a new disease-related locus.

Laparoscopic-assisted Soave procedure for Hirschsprung disease: 10-year experience with 106 cases

BackgroundThe purpose of this study was to summarize the clinical experience and 10 year follow-up results of laparoscopic assisted Soave procedure for the treatment of long-segment Hirschsprung disease (HD).MethodsFrom January 2010 to February 2020, 106 children with long-segment HD participated in this study. The laparoscopic-assisted Soave procedure was performed for the treatment of long-segment HD. The follow-up time was two weeks, one month, and three months after the operation, and then every six months to one year.ResultsThe operation was successful for all 106 children. All patients were discharged 5–7 days after the operation. The median time in surgery was 150 (100–190) minutes, and the median volume of bleeding was 6 (3–10) ml. The short-term postoperative daily defecation frequency was 4–11 times, 3–7 times within 6 months, and 2–3 times after 6–12 months. Postoperative complications included anastomotic leakage in two cases, perianal dermatitis in 13 cases, anastomotic stenosis in four cases, adhesive bowel obstruction in two cases, enterocolitis in 16 cases, soiling in 11 cases, and constipation recurrence in three cases.ConclusionsThe laparoscopic-assisted Soave procedure is a safe and effective surgical method for treating long-segment HD, and it causes little trauma or bleeding and has a fast postoperative recovery. Yet some complications may occur. Preoperative diagnosis, intraoperative and postoperative standardized processing can reduce the postoperative complications.

Resuturing Without Enterostomy for the Treatment of Early-Stage Anastomotic Leaks After Laparoscopic Soave Procedure in Hirschsprung's Disease.

Background: Anastomotic leak (AL) rates gradually decreased with surgical skills and perioperative management progression, but it is still inevitable. As the traditional management of AL after the pull-through procedure of Hirschsprung's disease (HD), enterostomy could lead to multiple surgeries, repeated hospitalizations, increased costs, and enterostomy-associated complications. This study aimed to explore the safety and feasibility of resuturing without enterostomy treating early AL after the laparoscopic Soave procedure. Methods: From October 2014 to June 2019, 10 patients who had AL after the laparoscopic Soave procedure were included. Six patients underwent simply resuturing with presacral drainage; the reoperation time was 1-5 days after primary surgery. Four patients who had diffused peritonitis or severe inflammations received resuturing with an ileostomy, and the reoperation time was 6-11 days. Results: Common early symptoms of AL included persistent fever, sacrococcygeal pain, and abdominal pain. The median delay to reoperation was 1.0 (0-2.25) day. Five patients had leaks at the 3-6 o'clock position, two had leaks at the 6-9 o'clock, and the other three had leaks at the 6 o'clock. The median postoperative fever durations were similar in patients without or with an ileostomy, and the median length of intensive care unit (ICU) stays, duration of antibiotic use, and postoperative length of stay were significantly longer in patients with ileostomy. The mean follow-up time was 38.5 ± 16.7 months (15-69 m). As of the time of writing, no reoccurrence was identified. Conclusion: For patients without diffuse peritonitis, severe inflammations, early diagnosis and timely resuturing of AL within 5 days after the laparoscopic Soave procedure of HD could be a safe, effective, and pleasing treatment.

Intraluminal manipulator-assisted laparoscopic surgery for Hirschsprung disease

Abstract Background Total colonic aganglionosis (TCA) and long segment aganglionosis (LSA) are rare forms of Hirschsprung disease (HD). Laparoscopic resection of total or the longer segment of the colon has not been unestablished for these forms. Methods We retrospectively reviewed five infants with TCA or LSA who underwent the blunt tip dissector (BD) assisted laparoscopic Soave pull-through procedure, and patients’ characteristics and procedure outcomes were analyzed. Results One infant with TCA had Mowat–Wilson syndrome. Ileostomy for TCA and transanal catheter placement for LSA allowed full enteral feeding with good weight gain before operation. The BD manipulator used in the laparoscopic surgery was simple to use and powerful enough to create tension, expand operating space, and improve access to specific parts of the mesentery or pelvis. No intraoperative complications such as perforation or bleeding were noted; thus, the operation was completed uneventfully, without conversion to open surgery in all cases. All the patients had excellent cosmetic results, without late complications. Conclusions The BD manipulator is safe and effective to facilitate laparoscopy-assisted Soave pull-through procedure in children with TCA and LSA. This approach may be applied to the other pediatric diseases, which require resection of the longer segment of the colon.

Transumbilical enterostomy for Hirschsprung's disease with a two-stage laparoscopy-assisted pull-through procedure.

BACKGROUNDA one-stage laparoscopic operation has recently been considered a favorable option for the management of patients with Hirschsprung's disease (HD) due to its superior cosmetic results. One-stage transanal endorectal pull-through for the treatment of rectosigmoid HD has been widely used in newborns without complications. However, enterostomy is required in some HD cases for enterocolitis and dilated colon. Our transumbilical enterostomy (TUE) and two-stage laparoscopy-assisted anorectoplasty were effective and achieved a similar cosmetic effect to one-stage laparoscopy on the abdominal wall in patients with anorectal malformation, but the effect in patients with HD is unclear.AIMTo evaluate the safety, efficacy and cosmetic results of TUE in two-stage laparoscopy-assisted pull-through for HD.METHODSFrom June 2013 to June 2018, 53 patients (40 boys, 13 girls; mean age at enterostomy: 5.5 ± 2.2 mo) who underwent enterostomy and two-stage laparoscopy-assisted pull-through for HD with stoma closure were reviewed at our institution. Two enterostomy approaches were used: TUE in 24 patients, and conventional abdominal enterostomy (CAE) in 29 patients. Eleven patients with rectosigmoid HD had severe preoperative enterocolitis or a dilated colon. 26 patients had long-segment HD, and 16 patients had total colonic aganglionosis (TCA). The patients with left-sided HD underwent the two-stage laparoscopic Soave procedure, and the patients with right-sided HD and TCA underwent the laparoscopic Duhamel procedure. Demographics, enterostomy operative time, complications and cosmetic results were respectively evaluated.RESULTSThere were no differences between the groups with respect to gender, age at enterostomy, weight and clinical type (P > 0.05). No conversion to open technique was required. Two patients experienced episodes of stomal mucosal prolapse in the TUE group and 1 patient in the CAE group (8.33% vs 3.45%, P > 0.05). No parastomal hernia was observed in either of the two groups. Wound infection at the stoma was seen in 1 case in the TUE group, and 2 cases in the CAE group (4.17% vs 6.90%, P > 0.05). No obstruction was noted in any of the patients in the TUE group, whereas obstruction was found in 1 patient in the CAE group. Enterocolitis was observed in 3 and 5 patients in the TUE and CAE group, respectively (12.50% vs 17.24%, P > 0.05). There was no significant difference between the TUE group and CAE group in terms of the incidence of soiling and constipation (P > 0.05). The cosmetic result using the scar score in the TUE group was better than that in the CAE group (6.83 ± 0.96 vs 13.32 ± 1.57, P < 0.05).CONCLUSIONTUE is a safe and feasible method for the treatment of HD, and the staged enterostomy and two-stage laparoscopy-assisted pull-through achieved a similar cosmetic effect to the one-stage laparoscopic procedure.

Laparoscopic Soave procedure for long-segment Hirschsprung's disease - single-center experience.

IntroductionHirschsprung’s disease (HD) is one of the most common and severe diseases treated in pediatric surgery. Since the introduction of the laparoscopic technique into pediatric surgery, laparoscopic-assisted radical surgery for HD has demonstrated unique advantages. Long-segment and total-colon HD are the best indications for laparoscopic-assisted surgery.AimTo summarize clinical experience of the laparoscopic Soave procedure for long-segment HD in a single center.Material and methodsThe data of children with long-segment HD who underwent the laparoscopic Soave procedure in our department from January 2013 to May 2018 were reviewed. The clinical features, surgical procedures and follow-up results were summarized and analyzed.ResultsThirty-one children underwent the laparoscopic Soave procedure; none of them were converted to open surgery. The average hospitalization time was 8.7 days after the operation, and no immediate postoperative complications occurred. There was no recurrence of constipation. Six (19.3%) cases were complicated with HD associated enterocolitis in the first 3 months postoperatively; 5 (16.1%) cases had soiling in the first year. There were 0 cases of adhesive bowel obstruction, 0 cases of anastomotic stenosis, and 0 cases of constipation.ConclusionsA small volume centre may achieve satisfactory results for long-segment HD. The laparoscopic Soave procedure is a minimally invasive, safe and effective treatment.

Clinical efficacy of laparoscopic Soave's surgery for the treatment of congenital Hirschsprung's disease in infants

Objective To investigate the clinical efficacy of laparoscopic Soave's surgery for the treatment of congenital Hirschsprung's disease in infants (age ＜ 2 months).Methods The clinical data of 368 infants with congenital Hirschsprung's disease who received laparoscopic Soave's surgery at the Children's Hospital of Xuzhou City from June 2005 to June 2012 were retrospectively analyzed.Three Trocars with diameter of 0.5 cm were used.The colic ligament was freed and the mesentery was ligated laparoscopically,then the rectal mucosa was stripped for 5-7 cm in length,and was pulled out through the rectal muscle sheath.Patients were followed up via out-patient examination at postoperative month 1,3,6,12 to learn the frequency of defecation,constipation and anal stenosis.The follow-up was ended in June of 2013.Results Two patients were converted to open surgery,and the remaining 366 patients received laparoscopic surgery successfully,with no incidence of wound infection.The operation time,volume of blood loss and duration of hospital stay were (100 ±20)minutes,(5.4 ± 1.5) mL and (7.3 ± 1.5) days,respectively.The average time for antibiotics usage was (3.5 ± 1.6) days and the time for infusion was (3.8 ± 1.4) days.All the infants were followed up,and the mean time for postoperative follow-up was 4.5 years (range,1.0-8.0 years).Eleven patients were complicated with Hirschsprung's disease associated enteritis,7 patients with constipation,5 with anal stenosis,4 with fecal pollution.The condition of the infants was improved after conservative treatment.Conclusion Laparoscopic Soave's surgery is suitable for the treatment of congenital Hirschsprung's diseases in infants aged under 2 months,it has the advantages of small trauma,quick recovery,better cosmetic effects and few complications. Key words: Congenital Hirschsprung's diseases ; Laparoscopy ; Infant