Transanal endorectal pull-through for Hirschsprung's disease using long cuff dissection and short V-shaped partially resected cuff anastomosis: early and late outcomes.

BACKGROUND: Transanal endorectal pull-through was described by De la Torre-Mondragon's technique. In the original transanal pull-through procedure, a long rectal muscular cuff was dissected and left for anocolic anastomosis, which would sometimes lead to postoperative obstructive symptoms and enterocolitis. While a modified short mucosal dissection may increase the risk of injury to pelvic and perirectal nerves and other structures when dissected on the outside of the rectum deep in the pelvis. We report early and late results of the modified transanal procedure for Hirschsprung's disease (HD) over 8 years. METHODS: The clinical course of all children with aganglionic rectum or sigmoid colon receiving the modified transanal pull-through between May 2003 and April 2011 were reviewed. The main modifications were rectal mucosa dissection with a long cuff, coloanal anastomosis with a short cuff and a V-shaped partial resection in the posterior wall of the muscular cuff. Children with preliminary stoma or total colonic disease were excluded. RESULTS: Short- and long-term follow-up was obtained in 137 patients with HD operated upon by the same surgeon. The aganglionic segment was located in the rectum or sigmoid colon in all patients. The mean age at surgery was 165 ± 74 days. The mean operating time was 108 ± 38 min. Mean intra-operative blood loss was estimated to be 15 ± 10 ml. No patient required a blood transfusion. Mean postoperative hospital stay was 7 ± 2 days. Early postoperative complications included perianal excoriation in 38 patients (27.7 %), enterocolitis in two patients (1.4 %), and anastomotic leak in two patients (1.4 %). Late postoperative complications included perianal excoriation in 16 patients (11.7 %), anal stricture in two patients (1.4 %), constipation in four patients (2.8 %), enterocolitis in 10 patients (7.3 %), and soiling problems in six patients (4.4 %). Mean follow-up was 56 months (6 months-9 years). In patients older than 4 years, 85.4 % of them had excellent/good bowel function, 9.4 % had fair bowel function, and 5.2 % of patients had bad bowel function. CONCLUSION: Transanal endorectal pull-through with a long cuff dissection and a short V-shaped resected cuff anastomosis is a safe and effective procedure for HD. It reduced incidence of anastomotic stricture and constipation without an increased soiling incidence.

Laparoscopically assisted anorectal pull-through for high imperforate anus in infants: intermediate results.

PURPOSE: The aim of this study was to evaluate the clinical outcomes and postoperative anal function in infants with congenital high imperforate anus treated with laparoscopically assisted anorectal pull-through (LAARP). METHODS: From January 2004 to July 2007, 33 patients (28 boys and 5 girls, age ranging from 3 to 10 months) with high imperforate anus underwent LAARP. Clinical data of the LAARP group were retrospectively compared with those treated by posterior sagittal anorectoplasty (PSARP; n = 28) during the same time period. Anorectal function of these patients was evaluated using the following 3 methods: the Kelly score, anorectal vector volume manometry, and magnetic resonance imaging between the ages of 3.1 and 4.4 years. RESULTS: The mean operative time in LAARP and PSARP groups was 112.5 ± 12.4 and 120.4 ± 18.5 minutes (P > .05), respectively. The mean length of hospital stay in the LAARP group was shorter than that of PSARP group (11.3 ± 2.1 vs 14.6 ± 2.3 days, P < .01). No significant difference was observed between LAARP and PSARP groups regarding the Kelly score (3.52 ± 1.42 vs 3.49 ± 0.82). Although magnetic resonance imaging revealed lower malposition rates of rectum in the LAARP group than those of the PSARP group at both I-line (3.0% vs 14.3%) and M-line (3.0% vs 10.7%) levels, this was not statistically different (P > .05). Compared with the PSARP group, lower asymmetric index, larger vector volume, and higher anal canal pressure at rest and during voluntary squeeze were observed in LAARP group (P < .05). However, there were no significant differences in the length of high-pressure zone (15.2 ± 5.8 vs 15.1 ± 6.2 mm) and the presence of rectoanal relaxation reflex (84.8% vs 85.7%). CONCLUSIONS: Satisfactory fecal continence can be achieved in patients with high-type imperforate anus after LAARP. Laparoscopically assisted anorectal pull-through has advantages over PSARP, including shorter hospital stay and better position of rectum. However, long-term follow-up is necessary to compare the benefits of LAARP against PSARP.

Laparoscopic extensive colectomy with transanal Soave pull-through for intestinal neuronal dysplasia in 17 children.

BACKGROUND: Open colectomy has been preferred for intestinal neuronal dysplasia type B (IND) due to its low morbidity rate and good functional results. The aim of this study was to investigate the feasibility and results of laparoscopic colectomy with transanal Soave pull-through for the treatment of IND in children. METHODS: Seventeen infants and children suffering from IND were treated by laparoscopic extensive colectomy with transanal Soave pull-through. The diagnosis of IND was made via anorectal manometry, X-ray contrast enema, suction biopsies, and laparoscopic full-thickness biopsies with hematoxylin-eosin staining. The technique used four or five abdominal ports. The sigmoid, transverse, and right colon up to the last ileal cove were mobilized laparoscopically in the extended form of IND. A modified Soave's anastomosis was performed. The patients' data, surgical procedures, operative data, postoperative complications and clinical outcomes were analyzed. RESULTS: Five patients underwent laparoscopic left colectomy with modified transanal Soave procedures, and the other 12 were treated by laparoscopic subtotal colectomy and required a Deloyers' maneuver for the Soave pull-through. The proximal margin of barium stagnation in patients with left colectomy was restricted to the distal end of the descending colon, sigmoid colon, and that in patients with subtotal colectomy was restricted to the proximal end of the descending colon, transverse colon, hepatic flexure, and ascending colon. Postoperative complications included anastomotic leakage, severe perianal erosions, postoperative enterocolitis, and soiling. During a mean follow-up of 4 years, bowel frequency was 4-10 times per day in 3 months postoperatively in patients with subtotal colectomy. The clinical results were good, with no stool incontinence or constipation. CONCLUSIONS: Laparoscopic procedure for left colectomy and subtotal colectomy with transanal Soave pull-through in infants and children with IND is safe, feasible, and effective. The location of barium stagnation in proximal margin may be used as a method to predict initially the proximal margin of the resected bowel segment.

Nuss operation for pectus excavatum: a single-institution experience.

BACKGROUND: The Nuss procedure for repair of pectus excavatum (PE) has been accepted worldwide because of minimal invasiveness and excellent cosmetic results. We summarized our experience with the treatment of 115 patients aged 2.7-18 years. METHODS: All the 115 patients underwent the Nuss procedure successfully from July 2003 to February 2008. They were divided into two groups: children group (below 12 years) and adolescents group (aged 12-18 years). RESULTS: The rate of complications was 14.7% and 37.5% in the children and adolescents groups, respectively (P<0.05). There was significant difference in operation time, length of hospital stay, and analgesic time between the two groups (P<0.05). The initial results of Nuss procedure were excellent. CONCLUSIONS: The Nuss procedure can be performed with excellent early results in children. We suggest that children with PE should accept the Nuss procedure as early as possible when they are over 5 years old.

Duodenal heterotopic pancreas in a child.

BACKGROUND: Heterotopic pancreas is characterized by pancreatic tissue outside the pancreatic bed. However, duodenal heterotopic pancreas in children is rarely reported so far. We describe herein duodenal heterotopic pancreas in a child who suffered from chronic abdominal pain. METHODS: An 8-year-old boy presented with upper abdominal pain and intermittent vomiting, without a history of melena, hematochezia, hematemesis, clay-colored stools, jaundice, hepatitis or dyscrasia. No specific medication or change in position relieved the pain. Based on the elevated serum amylase levels, and the findings of CT, barium meal X-ray examination, magnetic resonance imaging, and upper gastrointestinal endoscopy, a duodenal mass was diagnosed initiatively. Intraoperative frozen section analysis was performed for the diagnosis. The mass was dissected. RESULTS: Intraoperative frozen section analysis and routine pathological examination confirmed the diagnosis of duodenal heterotopic pancreas. The patient had an uneventful recovery and remained asymptomatic postoperatively during a follow-up period of 16 months. CONCLUSIONS: Heterotopic pancreas should be considered in children with a duodenal mass and abdominal pain. Intraoperative frozen section analysis is helpful in the diagnosis of the disease. Surgical treatment of the lesion should be performed to prevent bleeding, ulceration, outlet obstruction or malignant degeneration.

Expression of matrix metalloproteinase 1 and 2 in congenital choledochal cyst and its clinical significance.

BACKGROUND: The pathogenesis of congenital choledochal cyst (CCC) is still not well elucidated. This study was conducted to detect the expression of matrix metalloproteinase-1 and 2 (MMP-1 and MMP-2) in CCC. The roles of MMP-1 and MMP-2 in CCC formation were also discussed. METHODS: Specimens of CCC and gallbladders were taken from 53 patients with CCC, including 21 boys and 32 girls aged from 4 months to 17 years (mean: 6.5 years old). Twenty-eight normal gallbladders served as controls. Immunohistochemical method was used to detect the expressions of MMP-1 and MMP-2. Image analysis was made to quantitatively analyze the protein expression. RESULTS: The expressions of MMP-1 and MMP-2 were located in epithelial cells, matrix cells, and inflammatory cells of the CCC wall. Various expressions were also found in the mucosa membrane cells of the CCC gallbladder. The expressions of MMP-1 and MMP-2 were low in the control group. Quantitatively, the expressions of MMP-1 and MMP-2 in the CCC wall were markedly higher than in the CCC gallbladder and the control group (P<0.01). There was no significant difference between the CCC gallbladder and the control group (P>0.05). CONCLUSIONS: The expression of MMP-1 and MMP -2 in the CCC wall is higher than in the CCC gallbladder and controls. The over-expression of MMP-1 and MMP-2 may be involved in the pathogenesis of CCC.

Expression and clinical significance of stem cell marker CD133 in human neuroblastoma.

BACKGROUND: Recent evidences indicate that CD133, a kind of transmembrane protein, can be used as a marker to isolate stem cells from tumors originating from neural crest. This study was undertaken to explore the expression and clinical significance of stem cell marker CD133 in neuroblastoma (NB). METHODS: Immunohistochemical staining was used to detect the expression of CD133 in 32 patients with NB and 8 patients with ganglioneuroblastoma (GNB). The relationships were analyzed among CD133 expression, international neuroblastoma staging system (INSS) stages, pathological classification, and postoperative survival time of NB patients. RESULTS: The expression rates of CD133 in NB and GNB were 46.9% (15/32) and 37.5% (3/8) respectively, mainly in cytoplasm of neuroblastoma cells. The expression rates of stage 1-2, stage 3-4 and stage 4S were 30.7%, 57.9% and 37.5%, respectively. The differences in various stages were significant (P<0.05). The positive rate of CD133 in patients with unfavorable histology (52.4%) was significantly higher than that in patients with favorable histology (36.8%) (P=0.007). The survival time of CD133 negative patients was significantly longer than that of CD133 positive patients (P=0.026). CONCLUSIONS: CD133 which might be correlated with the development and progression of NB can serve as one of the important indicators for prognosis of NB.

[Separation of gastrothoracopagus conjoined twins: 2 cases report].

OBJECTIVE: To investigate reasonable surgical therapy for conjoined twins. METHODS: Two pairs of gastrothoracopagus were admitted in July 2004 and April 2005 respectively. The first pair was separated by emergency surgery for the rupture of umbilical hernia resulting in the exposure of intestines. The thoracic and abdominal wall was repaired with local skin flaps, and the secondary wound was covered with artificial skin. Skin expanders were embedded in thoracic and abdominal wall 2 months after birth in the second pair. The surgical separation was performed one month after. The deficiencies of pericardium, sternum and abdominal wall were reconstructed by allogenic grafting of pericardium, porous polyethylene implant and monofilament polypropylene patch respectively. The thoracic and abdominal wall was repaired with expanded rotation skin flap. RESULTS: The first twins died of respiratory failure and circulatory and respiratory failure 2 hours and 39 hours after the separation respectively. Both of the second pair survived and were discharge after healing. CONCLUSIONS: The separation of gastrothoracopagus should be performed after skin expansion in the interest of the closure of wound. It's better to use porous polyethylene implant and monofilament polypropylene patch to reconstruct the sternum and abdominal wall respectively.

Nitrofen suppresses cell proliferation and promotes mitochondria-mediated apoptosis in type II pneumocytes.

AIM: To characterize the molecular mechanisms of nitrofen-induced pulmonary hypoplasia. METHODS: After administration of nitrofen to cultured type II A549 pneumocytes, cell proliferation and DNA synthesis were investigated by 3-(4,5- dimethylthiazol-2-yl)-2,5-diphenyl tetrazolium bromide colorimetry, colony formation assay, flow cytometry and [3H]-thymidine incorporation assay. Apoptosis was measured by terminal transferase-mediated dUTP nick-end-labeling, acridine orange-ethidium bromide staining and flow cytometry. Expression of proliferating cell nuclear antigen (PCNA) and apoptosis-related genes was assayed by immunofluorescence, RT-PCR and Western blot. RESULTS: Nitrofen inhibited the cell proliferation of A549 cells in a dose- and time-dependent manner, accompanied by downregulation of PCNA. As a result, the DNA synthesis of nitrofentreated A549 cells decreased, while cell cycle was arrested at G0/G1 phase. Moreover, nitrofen induced apoptosis of A549 cells, which was not abolished by Z-Val-Ala- Asp(OCH3)- fluoromethylketone. In addition, nitrofen decreased the expression of Bcl-x( L), but not of Bcl-2, Bax, and Bak, resulting in a loss of mitochondrial membrane potential and the nuclear translocation of apoptosis-inducing factor (AIF). Meanwhile, nitrofen strongly activated the p38 mitogen-activated protein kinase (p38-MAPK). Pretreatment of cells with SB203580 (5 micromol/L) blocked nitrofen-induced phosphorylation of p38-MAPK and abolished nitrofen-induced AIF translocation and apoptosis in A549 cells. CONCLUSION: Nitrofen suppresses the proliferation of cultured type II pneumocytes accompanied by the downregulation of PCNA, and induces mitochondria-mediated apoptosis involving the activation of p38-MAPK.

Diagnosis and treatment of biliary atresia: a retrospective study.

BACKGROUND: Biliary atresia (BA) is the end results of an inflammatory process, which affect the intrahepatic and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tract with the development of biliary cirrhosis. Early diagnosis of BA is difficult, and there is no specific therapy for BA at present. The purpose of this study was to investigate the diagnosis, treatment and postoperative outcome of BA and to explore new diagnostic and therapeutic strategies. METHODS: Thirty-one children with biliary atresia from our hospital and 201 children with such disease from other hospitals in China were reviewed retrospectively in terms of diagnostic modalities, operative age, operative methods and long-term survival rates after operation. RESULTS: The operative age of the 31 patients varied from 13 to 270 days. It was <60 days in 6 children (19%), 61-90 days in 14 (45%), 91-120 days in 8 (26%), and >120 days in 3 (10%). No children underwent transplantation for BA. Their 1-6 year survival rate on average was 32.3% (10/31). The early operative rate in China was 12.9% (30/232). One child received liver transplantation with a long-term survival rate of 31.9%. CONCLUSIONS: Early recognition of babies with BA is critical for optimal intervention for preventing progressive fibrosis. Clinical presentation, imaging and laparoscopy are helpful in enhancing the early diagnostic rate of BA patients. The long-term survival rates can be obtained after the improvement of operative methods, liver transplantation for children with advanced BA, and development of other medical strategies.

[First-line Xeloda (Capecitabine) treatment for advanced and recurrent colorectal cancer].

OBJECTIVE: To evaluate the efficacy and safety of capecitabine as first-line therapy in patients with advanced and recurrent colorectal cancer. METHODS: From December 2000 to November 2001, sixty patients with advanced and recurrent colorectal cancer received first-line capecitabine treatment given at a dose of 1250 mg/m(2) twice daily, on days 1 - 14 every 21 days. At least 2 cycles were administered. RESULTS: The overall response rate was 23.3% with 14 PR, 24 SD (40.0%) and 15 PD. The median survival time was 14.7 months. The survival rate was 63.9% at 12-months and 33.4% at 24-months. Grade III-IV adverse effects were diarrhea in 4 patients (6.6%), anemia in 2 (3.3%) and hand-foot syndrome (HFS) in 1 (1.7%); Grade I-II adverse effects were hyperpigmentation in 20 (33.3%), HFS in 18 (30.0%) and diarrhea in 10 (16.7%). CONCLUSION: Capecitabine is an efficacious and better-tolerated alternative treatment for the patients with advanced and recurrent colorectal cancer.