Redo pull-through in total colonic aganglionosis due to residual aganglionosis: a single center's experience.

BACKGROUND: Reoperation for total colonic aganglionosis (TCA) may be required for residual aganglionosis after an initial radical operation. We aimed to investigate the symptoms, management, and outcomes of patients who required a redo pull-through (Redo PT). METHODS: Nine TCA patients underwent Redo PT at our center between 2007 and 2017. Their medical records were reviewed. Parental telephone interviews that included disease-specific clinical outcomes were conducted, and post-operative complications and long-term outcomes (including height-for-age/weight-for-age and bowel-function score) were compared to those of single-pull-through (Single PT) patients (n = 21). RESULTS: All the nine Redo PT patients suffered obstruction within 1 month after the initial operation that could not be alleviated by conservative treatment. All abdominal X-ray/contrast barium enemas showed proximal bowel dilatation, indicating residual aganglionosis. The median ages at the initial operation and Redo PT were 200 and 509 days, respectively. Reoperation consisted of an intraoperative frozen biopsy and a modified laparotomic Soave procedure in all patients. Post-operative complications included perianal excoriation (n = 3), intestinal obstruction (n = 2), enterocolitis (n = 2), and rectovestibular fistula (n = 1). Seven Redo PT patients were followed up for a mean time of 7.1 ± 2.3 years; six (85.7%) had good growth and four (57.1%) had good bowel-function recovery. Post-operative complications and long-term outcomes were almost equal between the Redo PT and Single PT groups (all P > 0.05). CONCLUSION: TCA patients with recurrent obstructive symptoms and dilated proximal bowel may have residual aganglionosis after an initial operation. Redo PT is effective and provides good long-term outcomes comparable to those of patients who benefited from Single PT.

Ten-Year Experience in the Prevention of Post-Kasai Cholangitis.

BACKGROUND: Cholangitis is the most common complication after the Kasai procedure. It can be life-threatening and may affect long- and short-term outcomes of children with biliary atresia. We summarize our experiences in the prevention of early-onset cholangitis. PATIENTS AND METHODS: From January 2002 to March 2013, children with biliary atresia (BA) who underwent the Kasai procedure in the General Surgical Department were included in a retrospective cohort study. These patients were divided into group A (therapy 1) and group B (therapy 2) depending on the infection prevention protocol and occurrences of cholangitis within the six months after surgery were recorded. Two hundred eighteen children were included in this cohort study. Seventy-six children (35 females and 41 males) were included in group A. One hundred forty-two children (65 females and 77 males) were included in group B. Therapy 1 was our primary protocol and included a third-generation cephalosporin, metronidazole, and human immunoglobulin. Therapy 2 was a modification of therapy 1 that involved imipenem-cilastatin and human immunoglobulin. Statistical analyses were performed. A p value below 0.05 was regarded as significant. RESULTS: In group A, 45 children developed cholangitis within the six months after the Kasai procedure. In group B, 14 of these children experienced post-operative cholangitis. A χ2 analysis was used to examine the difference in the incidence of cholangitis between groups A and B. There was a substantial difference in the morbidity of post-operative cholangitis between groups A and B (59.2% vs. 9.9%, p = 0.000). CONCLUSION: Cholangitis in the early period after a Kasai procedure can be prevented effectively with an advanced prophylactic protocol.

STROBE-anastomotic leakage after pull-through procedure for Hirschsprung disease.

This study was undertaken to explore the causes, diagnosis, and treatment of anastomotic leakage after pull-through (PT) procedure for Hirschsprung disease (HD).A retrospective analysis of patients with anastomotic leakage after a PT procedure in the General Surgery Department of Beijing Children's Hospital from July 2013 to June 2016 was undertaken. The surgical characteristics, diagnosis, and treatment were retrospectively analyzed.Among the 213 patients who underwent PT procedures, 5 patients had a documented anastomotic leakage. The median age of these 5 patients at the time of the PT procedure was 6.8 years old, and this was higher than those without anastomotic leakage (1.7 years old). In all patients, rectal examination in the lithotomy position revealed an anastomotic dehiscence at the 6 o'clock position. The abdominal ultrasonography demonstrated retrorectal pneumatosis with or without an abscess in 4 patients. All patients were treated with ileostomies and anastomotic resuturing. The median delay to management was 4 days (range: 1-29 days). Four patients (4/5, 80%) were cured, and 1 (delay, 29 days) of these 4 patients developed postoperative ileus. The remaining patient (delay, 9 days) was required to undergo a repeat PT procedure. For the 4 cured patients, the median follow-up time was 20 months (range: 15-37 months), and these patients defecated 3 times daily at most without soiling.Older children with HD might be prone to anastomotic leakage. The findings of the rectal examination and ultrasonography were distinctive and useful for the diagnosis. Early ileostomy and resuturing of the anastomosis could be used to treat anastomotic leakage.

Assessment of liver fibrosis by Fibroscan as compared to liver biopsy in biliary atresia.

AIM: To evaluate liver stiffness measurement (LSM) using non-invasive transient elastography (Fibroscan) in comparison with liver biopsy for assessment of liver fibrosis in children with biliary atresia (BA). METHODS: Thirty-one children with BA admitted to the Department of Pediatric Surgery of Beijing Children's Hospital from March 2012 to February 2013 were included in this study. Their preoperative LSM, liver biopsy findings, and laboratory results were studied retrospectively. RESULTS: The grade of liver fibrosis in all 31 patients was evaluated according to the METAVIR scoring system, which showed that 4 cases were in group F2, 20 in group F3 and 7 in group F4. There were 24 non-cirrhosis cases (F2-F3) and 7 cirrhosis cases (F4). In groups F2, F3 and F4, the mean LSM was 9.10 ± 3.30 kPa, 11.02 ± 3.31 kPa and 22.86 ± 12.43 kPa, respectively. LSM was statistically different between groups F2 and F4 (P = 0.002), and between groups F3 and F4 (P = 0.000), however, there was no statistical difference between groups F2 and F3 (P = 0.593). The area under the receiver operating characteristic curve of LSM for ≥ F4 was 0.866. The cut-off value of LSM was 15.15 kPa for ≥ F4, with a sensitivity, specificity, positive predictive value and negative predictive value of 0.857, 0.917, 0.750 and 0.957, respectively. CONCLUSION: Fibroscan can be used as a non-invasive technique to assess liver fibrosis in children with BA. The cut-off value of LSM (15.15 kPa) can distinguish cirrhotic patients from non-cirrhotic patients.

Anterior perineal fistulectomy in repairing H-type rectovestibular fistula with a normal anus in female children.

The purpose of this study was to review a 7-year operative experience of anterior perineal fistulectomy in repairing H-type rectovestibular fistula with a normal anus in female children. From January 2007 to December 2013, 105 female children with H-type rectovestibular fistula and a normal anus underwent anterior perineal fistulectomy in the Department of General Surgery, Beijing Children's Hospital, Capital Medical University. Clinical data were retrospectively analyzed. Patients' age ranged from 6 months to 13 years 10 months, with an average age 5 years 2 months. The clinical feature of this disease was a definite history of vestibular infection at about 30 days after birth and leakage of gas or loose stool through vestibular fistular orifice after the infection was controlled. All patients had a normal anus. Follow-up through telephone or outpatient visits from 6 months to 7 years (median, 5.2 years), showed that all the patients were continent and had regular bowel movements. Among the 105 patients, 97 (92.4%) gained primary healing with a satisfactory appearance; 8 (7.6%) patients relapsed, among which 5 healed after hip bathing treatment within 3 weeks, 2 required another anterior perineal fistulectomy 7 months later and were cured, and the last one had another anterior perineal fistulectomy 1 year 10 months later but relapsed again, which healed after hip bathing treatment within 3weeks after the second surgery. Anterior perineal fistulectomy is a simple, safe and reliable surgical method to treat H-type rectovestibular fistula with a normal anus.

Rectovestibular fistula with normal anus: a simple resection or an extensive perineal dissection?

PURPOSE: The purpose of the study was to review a 10-year experience of operative procedures for rectovestibular fistula with normal anus (RVFNA) at one institution. MATERIALS AND METHODS: From January 1999 to December 2008, 182 female patients of RVFNA were treated surgically in the Department of Surgery, Beijing Children's Hospital, Capital Medical University, China. The patients' age ranged from 4 months to 15 years, with a mean age of 3.4 years. Fourteen children had a failed previous operation in other institutions. One hundred fifty-six patients (85.71%) had a definite history of vulvar inflammation within 3 months after birth and fecal leakage thereafter. In 61 of the 156 patients, a perineal abscess appeared after an episode of diarrhea. Four cases (2.20%) had associated anomalies of the VACTERL type. One hundred seventy-three patients (95.05%) had 1 external opening, whereas 9 others (4.95%) had 2 external openings separated by a skin bridge. In all cases, the internal orifice opened above the dentate line. The fistula in 135 cases (74.18%) presented as an opening with a small diameter (<5 mm). In our series, vestibular-rectal pull-through procedure was performed in 98 cases, transanal procedure in 69 cases, and anterior perineal anorectoplasty in 15 cases. None had a diverting colostomy. RESULTS: Recurrence of fistula occurred in 5 to 10 days after operation in 21 cases (11.54%). Eight of them healed spontaneously after daily sitz bath with 3% boric acid, whereas other 13 patients (7.14%) required reoperation. Follow-up was obtained by telephone or by personal visit in the outpatient department, from 3 months to 10 years (median, 5.7 years). All the patients were continent and had regular bowel movement. CONCLUSIONS: Most RVFNA was acquired after infection. Procedure requiring extensive perineal dissection and diverting colostomy are unnecessary in most RVFNA cases. We performed simple resection in most patients with satisfactory results. In the cases with a large external opening and abnormal perineal appearance, a more extensive perineal dissection might be necessary.

Space-occupying benign lesions in spleen: experiences in a single institute.

PURPOSE: To introduce a practical reference for the diagnosis and treatment of space-occupying benign lesions in children's spleens. METHOD: Ten cases were collected from 1988 to 2007, and analysis of some related literature were included with special attention to the criteria of the diagnosis and the indications of splenectomy, particularly to the age for operation. RESULTS: The age of patients on admission ranged from 5 to 15 years, with the mean age of 10 years. All chose the operation to mitigate the fear of either accidental rupturing or malignancy. The clinical diagnosis depended on ultrasonographic or other imaging findings, while the final diagnosis was based on pathological study of operative specimens. The final diagnosis of the five patients undergoing operation was benign tumors (angioma group), and the other five were cystic (mainly congenital). Eight children underwent primary splenectomy, and the other two underwent partial or secondary splenectomy, all with satisfactory results. Seven were followed up for more than 2 years and recovered uneventfully. CONCLUSION: Preoperative imaging examinations and postoperative pathology mutually confirmed space-occupying benign lesions in the spleen. The age of the children, size and character of the lesion, as well as the risk of rupturing and malignancy are reasonable indications of surgery. Splenectomy, unless with contra-indications, is a feasible choice of treatment.

Recto-vestibular disruption defect resulted from the malpractice in the treatment of the acquired recto-vestibular fistula in infants.

AIM: To explore the pathogenesis of the rectovestibular disruption (RVD) defect and to recommend a successful repair, and prevention of it. METHODS: Clinical records of 15 girls, age ranged from 3 to 15 (median, 7.5) years, with acquired rectovestibular fistula (RVF) mistreated before were retrospectively reviewed. All of them presented an abnormal appearance of perineum and were suffering from some degree of fecal incontinence, and those were graded III to IV by Li Zheng's Score. Repair of anal sphincters and reconstruction of perineum body and skin by anterior perineal rectoanoplasty were performed in all cases. RESULTS: Operation in all cases was successful. The perineum looked practically normal and fecal continence score rose up to VI by Li Zheng's Score. CONCLUSION: The conventional treatment for anal fistula, lay-open or string-treatment, should be considered as malpractice of RVF, and certainly leads to the RVD defect, and the anterior perineal rectoanoplasty could cure it satisfactorily.