Efficacy and Safety of Endoscopic Retrograde Cholangiopancreatography in Children of Pancreaticobiliary Maljunction Without Obvious Biliary Dilatation.

PURPOSE: There is no standard surgical approach for pancreaticobiliary maljunction (PBM) without congenital biliary dilatation (CBD). This study aimed to compare outcomes between therapeutic endoscopic retrograde cholangiopancreatography (ERCP) and laparoscopic hepaticojejunostomy (LH) for pediatric patients of PBM without obvious biliary dilatation (PBM-nonOBD). METHODS: We retrospectively reviewed demographic and clinical data of pediatric patients with PBM-nonOBD from 2015 to 2021. There were 33 patients in ERCP group and 35 patients in LH group. Primary outcomes included treatment efficiency, postoperative recovery, and postoperative complications. Univariate analysis was further used to explore prognostic factors for ERCP. RESULTS: The mean diameter of the common bile duct in LH group was larger than that in ERCP group (8.6 ± 1.3 mm vs. 6.9 ± 2.1 mm, p = 0.003), while there were no significant differences between the two groups in age, gender, clinical manifestations, complications, and other imaging findings. Compared with LH group, ERCP group had a shorter operation time and postoperative recovery time. The treatment effective rate of ERCP was inferior to that of LH (45.4 % vs. 85.7 %, p＜0.001). For postoperative adverse events, post-ERCP pancreatitis (15.1 %) was most common in the ERCP group. 30.3 % of patients eventually required LH. Intestinal obstruction (5.7 %), recurrent cholangitis (5.7 %), gastrointestinal bleeding (2.8 %), and anastomotic stenosis (2.8 %) were observed in LH group and 8.6 % of patients required a reoperation. A long common channel may be associated with poor prognosis after ERCP. CONCLUSIONS: ERCP is associated with less surgical trauma, shorter recovery time, and fewer serious complications than LH, while the treatment effective rate of ERCP is inferior to LH. The indications for endoscopic sphincterotomy and the timing of radical surgery need to be further explored. LEVEL OF EVIDENCE: Ⅲ STUDY TYPE: Retrospective Comparative Study.

Clinical Characteristics and Management of Colorectal Vascular Malformation in Children: A Retrospective Study of 23 Cases.

INTRODUCTION: The objective of this study is to summarize the clinical characteristics and management of rare diseases of colorectal vascular malformation (CRVM) in children. METHODS: We retrospectively analyzed the clinical data of CRVM patients admitted to the Children's Hospital of Fudan University from 2004 to 2019. RESULTS: A total of 23 cases (16 males, 7 females) were enrolled. The median age of symptom onset was 1.4 years. Hematochezia and anemia were cardinal symptoms. Fourteen patients (60.9%) were misdiagnosed as anal fissures (n = 4), internal hemorrhoids (n = 3), rectal polyps (n = 2), inflammatory bowel disease (n = 2), portal hypertension (n = 2), and Meckel's diverticulum (n = 1), respectively. The average time from symptom onset to diagnosis was 4.5 ± 4.4 years. Other vascular malformations were detected in eight patients (34.8%). All patients showed a positive anomalous vascular image on contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI). The sensitivity of colonoscopy in the diagnosis of CRVM was 82.6% (19/23). A total of 21 patients underwent a modified Soave procedure. The lesions were mostly restricted to the colorectum and showed transmural diffuse distribution, with an average length of 20 ± 5.4 cm. Two patients (9.5%) experienced surgical complications. Bloody stools reappeared in two patients (9.5%), and colonoscopy showed abnormal angiogenesis at the anastomotic site, which were cured by sclerotherapy and/or electrocautery. The median follow-up time was 78 months. Bloody stools were absent at the last time of follow-up, and hemoglobin was in the normal range for all patients. CONCLUSION: The identification of CRVM in children often is delayed. Colonoscopy, CT, and MRI are essential in making the correct diagnosis. The modified Soave procedure is safe and feasible to treat CRVM in children. Endoscopic sclerotherapy and/or electrocautery can be used for residual lesions.

Effect of Adjuvant Steroid Therapy in Type 3 Biliary Atresia: A Single-Center, Open-Label, Randomized Controlled Trial.

OBJECTIVE: To evaluate the efficacy and side effects of additional postoperative steroid therapy for type 3 BA versus the current routine care. SUMMARY BACKGROUND DATA: Whether steroid therapy post-Kasai portoen-terostomy improves the outcomes of BA remains controversial. Clinical evidence from 2 randomized trials in the UK and USA do not support the routine use of steroid in the treatment of BA. METHODS: In this open-label randomized controlled trial, patients with type 3 BA were randomized to routine postoperative treatment with or without 10 to 12 weeks of adjuvant steroid treatment. The primary outcome was the postoperative jaundice clearance rate with native liver at 6 months. The secondary outcomes included postoperative jaundice clearance rate at 3, 12, and 24 months, survival with native liver at 12 and 24 months, and SAEs within 3 months. RESULTS: Overall, 200 participants were randomized and allocated into either steroid or control group (n = 100/group). The proportion of participants that are jaundice free without liver transplantation was significantly higher in the steroid group than in the control group at 6 months (54.1% vs 31.0%, P = 0.0015). The native liver survival rate was higher postoperatively in the steroid group than in the control group at 12 (66.3% vs 50.0%, P = 0.02) and 24 (57.1% vs 40.0%, P = 0.02) months. The survival time with native liver was significantly longer in the steroid group than in the control group (median survival, steroid vs control: not reached vs 1.21 years, P = 0.02). There were no significant differences between the 2 groups in the mean occurrence of SAEs within 3 months (steroid vs control: 0.63 vs 0.45, P = 0.20). CONCLUSIONS: Postoperative adjuvant steroid intervention improved bile drainage and survival with native liver in type 3 BA patients, without increasing early-stage SAEs.

PHOX2B as a Reliable Marker for Neuroblastoma in Tissue and Cytology Specimens.

To investigate the diagnostic utility of immunohistochemistry for paired-like homeobox 2B (PHOX2B) expression in neuroblastomas (NBs) and tumors that mimic them, tissue samples (n = 229) from 157 cases of NB, 210 central nervous system tumors, and 170 extracranial non-NB solid tumors (n = 170) were immunostained for PHOX2B. Additionally, PHOX2B expression in 67 body fluid cytology specimens was analyzed. In tissue specimens, PHOX2B expression was positive in NBs, pheochromocytomas, and paragangliomas but negative in all of the other tumors evaluated. PHOX2B was detected by immunohistochemistry in 5 NB cytology specimens; all of the others were negative. These results suggest that PHOX2B may be a sensitive and specific immunohistochemical marker for the pathological diagnosis and differential diagnosis of NB in both tissue and cytology specimens.

Single-Cell Characterization of Malignant Phenotypes and Developmental Trajectories of Adrenal Neuroblastoma.

Neuroblastoma (NB), which is a subtype of neural-crest-derived malignancy, is the most common extracranial solid tumor occurring in childhood. Despite extensive research, the underlying developmental origin of NB remains unclear. Using single-cell RNA sequencing, we generate transcriptomes of adrenal NB from 160,910 cells of 16 patients and transcriptomes of putative developmental cells of origin of NB from 12,103 cells of early human embryos and fetal adrenal glands at relatively late development stages. We find that most adrenal NB tumor cells transcriptionally mirror noradrenergic chromaffin cells. Malignant states also recapitulate the proliferation/differentiation status of chromaffin cells in the process of normal development. Our findings provide insight into developmental trajectories and cellular states underlying human initiation and progression of NB.

Long-term surgical outcomes of apple-peel atresia.

PURPOSE: The purpose of this study was to investigate (i) postoperative course of apple-peel atresia (APA), (ii) long-term follow-up of APA children, and (iii) risk factors for poor prognosis. METHODS: We conducted a retrospective review of 39 APA neonates treated at our institution between 2008 and 2017. Patient characteristics, operative details, postoperative course, long-term outcomes, and prognostic factors were analyzed. RESULTS: Of the 39 APA neonates, 30 (76.9%) were born preterm, and 20 (51.3%) were diagnosed prenatally. All patients underwent primary anastomosis within the first week after birth: 10 laparoscopic-assisted (25.6%) and 29 open (74.4%). Postoperative complications occurred in 28 patients (71.8%), of which 20 (71.4%) developed cholestasis. Survival at hospital discharge was 94.9%. Median parenteral nutrition period was 59 days. Reoperation was required in 7 children (17.9%) owing to anastomotic obstruction (n = 3) and adhesive intestinal obstruction (n = 4). 32 children (82.1%) were followed up for an average of 5.7 years, of which 23 children (71.9%) showed normal growth and development. APA patients with low birth weight and associated anomalies had significantly worse outcomes. CONCLUSION: Most of the patients with apple-peel atresia have excellent long-term outcomes, though initial postoperative complications are common. Low birth weight and the presence of associated anomalies are independent prognostic factors in APA. TYPE OF STUDY: Prognosis study (case series). LEVEL OF EVIDENCE: Level IV.

CD4+T Cell Subset Profiling in Biliary Atresia Reveals ICOS- Regulatory T Cells as a Favorable Prognostic Factor.

Biliary atresia (BA) is a destructive pediatric liver disease and CD4+T cell activation is demonstrated to play an important role in BA. However, a comprehensive scenario regarding the involvement of CD4+T cell subsets to the development of BA remains unclear. Here, we aim to explore the infiltration of CD4+T cell subsets and their clinical significance in BA. In the present study, thirty BA liver samples were collected during surgery and were divided into good (BA1, n = 16) and poor prognosis (BA2, n = 14), with samples from choledochal cyst patients (n = 8) as control. By using multiplex immunohistochemistry, we evaluated the infiltration level of CD4+T cell subsets in the portal areas. RT-qPCR and flow cytometry were further applied to explore detailed features of Treg subsets. We revealed that hepatic infiltrating Th1, Th2, Th17, and ICOS+Treg cells were significantly increased in BA patients compared to controls and were negatively associated with prognosis, while high infiltrating ICOS-Tregs showed a favorable outcome. Phenotypic analysis indicated that, in contrast to ICOS+Tregs, ICOS-Tregs were mainly CD45RAhiCD45ROlow, and preferentially expressed more CD73. Besides, RT-qPCR revealed elevated expression of CD25, CD73, TGF-ß, and BCL-2 genes in ICOS-Tregs. Finally, functional assay confirmed that ICOS-Tregs had a higher suppressive capacity to cytokine secretion and were more resistant to apoptosis in vitro. Collectively, we demonstrate that a mixed immune response is involved in BA pathogenesis, and the globally enhanced effector CD4+T cell response is associated with unfavorable prognosis, highly suppressive ICOS-Tregs is a protective factor and may serve an important reference to predict prognosis.

Long-term prognosis of low-risk neuroblastoma treated by surgery alone: an experience from a single institution of China.

BACKGROUND: Low-risk neuroblastomas have favorable biologic characteristics. Children Oncology Group (COG) proposed that surgical resection of the primary tumor was sufficient. We evaluated the long-term prognosis of surgery alone for patients with low-risk neuroblastoma in China. METHODS: A total of 34 patients with low-risk neuroblastoma were treated in our center between Jan 2009 and Dec 2013. The medical records of these patients were reviewed. RESULTS: The primary lesion was located in the adrenal gland in 19 patients, the retroperitoneum in 5, the posterior mediastinum in 9 and the neck in 1. The tumor diameters and volumes were 1.80-10.0 cm (average 5.5 ± 2.3 cm) and 1.28-424.10 cm3 (average 58.81 ± 92.00 cm3), respectively. The stages of the patients were as follows: stage I in 25, stage II in 7, and stage IVs in 2. All patients were in the low-risk group according to COG risk stratification criteria. No patients showed MYCN amplification. The primary tumors of all patients were completely resected. Nine adrenal tumors were completely resected by laparoscopy. All patients were successfully followed for 66-115 (average 89.71 ± 16.17) months. Recurrence was observed in 4 patients. In addition to one local recurrence, another three recurrences were metastases. The lesions were effectively controlled in all patients with recurrences. All patients survived, including 28 cases of tumor-free survival; the 4-year overall and event-free survival rates were both 100%. CONCLUSIONS: Surgery alone is a safe and effective treatment strategy for low-risk neuroblastoma. Recurrent lesions may be controlled and treated by rescue chemotherapy and surgery.

Clinical features, treatment, and outcomes of bilateral Wilms' tumor: A systematic review and meta-analysis.

BACKGROUND: Wilms' tumor(WT) is the most common malignant renal tumor of childhood. Despite the good prognosis of WT, bilateral Wilms' tumor (BWT) still has a poor outcome. We systematically reviewed the literature on BWT, aiming to define its clinical features, treatment, and outcomes. METHODS: PubMed, OVID EMbase, Web of Science, and Cochrane Library were systematically searched for studies published from 1980 to 2017. Case series and comparative studies reported clinical data of BWT patients were included. RESULTS: A total of 32 studies comprising 1457 patients were retained for primary outcome. Hemihypertrophy, cryptorchidism, and Beckwith-Wiedemann syndrome(BWS) are the most common congenital anomalies and syndrome. 86% of patients had favorable histology (FH). Patients with local stage I or II accounted for 64%, and 12.6% had metastasis at diagnosis. Bilateral nephron-sparing surgery (NSS) was achieved in 33.8%. Recurrence and renal failure occurred in 20% and 8%. The overall survival (OS) was 73%. In comparative studies, OS of patients undergoing bilateral NSS was similar to that of other operation types. CONCLUSION: Prognosis of BWT has been improved but is significantly poorer than WT. Bilateral NSS was recommended by most centers to preserve more renal volume. However, finding a balance between retaining renal function and avoiding recurrence remains a question. TYPE OF STUDY: Systematic review. LEVEL OF EVIDENCE: Level IV.

The surgical choice for unilateral thyroid carcinoma in pediatrics: Lobectomy or total thyroidectomy?

BACKGROUND: Total thyroidectomy is no doubt the standard procedure for patients with bilateral thyroid carcinoma in pediatrics. However, for lesions confined in unilateral thyroid gland, lobectomy or radical total thyroidectomy is still controversial in pediatrics. METHODS: Thirty-five cases of pediatric thyroid carcinoma which were confirmed by pathology were retrospectively analyzed in our hospital from 2005 to 2016. In 13 cases of unilateral lesions, 6 received lobectomy, while others underwent total thyroidectomy. Recurrence and complications were compared between 2 groups. Also, there were 20 cases of primary total thyroidectomy and 4 cases of supplementary resection of the residual gland. The short-term complications were compared. RESULTS: There were no differences between groups of lobectomy and total thyroidectomy in size, extrathyroid extension, stage, cervical lymph nodes metastases, and distant metastases. They were the same in incidence of postoperative hypocalcemia, calcium supplement, recurrent laryngeal nerve damage, and recurrence rate and mortality, except that the length of stay in hospital was shorter in lobectomy group. Patients in supplementary resection were more likely to present the symptoms of postoperative hypocalcemia and hypokalemia, and it was difficult to identify the parathyroid gland during the operation by comparing with patients of primary total thyroidectomy. CONCLUSIONS: Thyroid carcinoma in pediatrics is inclined to be multifocal, and even in a single focus, the volume is large. Total thyroidectomy does not bring more complications. In contrast, the morbidity increases in staged resection. Endocrine hormone supplement can satisfy the need for growth and development. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III.

Neuroblastomas in Eastern China: a retrospective series study of 275 cases in a regional center.

PURPOSE: Most studies on neuroblastoma (NB) have been conducted in Western countries or Japan. The objective of our study was to analyze clinical and pathological features, MYCN status, surgical methods, and prognosis in Chinese NB patients. METHODS: A retrospective, single-center case series study of 275 NBs was implemented. Clinical manifestations, pathological features, MYCN status, and surgical treatment were analyzed. Log-rank test and Cox hazards models were used to assess overall survivals (OSs). RESULTS: The cohort consisted of 105 females and 170 males, with an age range of five days to 15 years. MYCN amplification was detected in 21.5% of all cases. The median OS was 15.0 months for MYCN amplified group. The five-year OS rates were 70.8% and 18.3% for MYCN unamplified and amplified groups, respectively, and the comparison of Kaplan-Meier curves for these two groups showed statistical significance (P < .001 by log-rank test). Gross total resection (GTR, n = 111) and subtotal resection (STR, n = 58) were administered in 169 patients at stages 3 and 4 who received chemotherapy and the comparison of Kaplan-Meier curves for different groups in these patients had statistical significance (STR vs. GTR, P = .009; MYCN unamplified vs. amplified, P < .001 by log-rank test, respectively).The multivariate survival analyses showed statistical significance (STR vs. GTR, P = .047; MYCN unamplified vs. amplified, P = .001 by Cox regression model). CONCLUSIONS: MYCN amplification is an independently adverse prognostic factor in Chinese NB patients at stages 3 and 4 and GTR is associated with improved OS compared with STR in these patients.

Comparison of long-term prognosis of laparoscopic and open adrenalectomy for local adrenal neuroblastoma in children.

OBJECTIVE: To investigate and compare long-term outcomes in children undergoing laparoscopic or open adrenalectomy for local adrenal neuroblastoma. METHODS: A retrospective review was conducted of 37 children with local adrenal neuroblastoma treated between January 2005 and December 2013 in our hospital. These patients met inclusion criteria for having adrenal neuroblastoma and undergoing operative resection. All patients were successfully followed up until December 2017. RESULTS: The local adrenal neuroblastoma cases included 25 males and 12 females with an average age of 37.24 ± 37.55 months (range from 5 days to 158 months). Left adrenal lesions were present in 13 cases, the right in 24 cases. According to the INSS staging system, 27 patients were classified as stage I and 10 as stage II. Open adrenalectomy was performed in 24 patients. Laparoscopic adrenalectomy was performed in the other 13 patients, 2 of whom were converted to open surgery because of adhesions to renal vessels and diaphragmatic rupture. Significant differences were observed between the laparoscopic surgery and open surgery groups regarding tumor size (P = 0.005). There were two recurrence cases in open surgery, but there was no recurrence in laparoscopic surgery. The average follow-up time was 86.78 ± 24.52 months. The overall 5-year survival rate of open and laparoscopic surgery were 86.2 and 100% (P = 0.316). CONCLUSIONS: Laparoscopic adrenalectomy for neuroblastoma is feasible and can be performed with equivalent recurrence and mortality rates with open resection. For small tumor size and absence of vascular encasement, the adrenal neuroblastoma may be preferred laparoscopic surgery.

Ectopic nephrogenic rests in children: A series of 13 cases in a single institution.

PURPOSE: Ectopic nephrogenic rests (ENR) are extremely rare, and their diagnosis and treatment have not yet been standardized. Our study aimed to analyze the clinical and pathological features of ENR in children and explore the optimal methods for diagnosis and treatment. METHODS: A retrospective, single-center, case series study of 13 children with ENR was performed. Clinical manifestations, work-up, pathological features, and treatment methods were analyzed, and the postoperative status was evaluated at follow-up. RESULTS: The study cohort included seven males and six females aged 1/4-19 months, who were divided into two subgroups: group 1 (pure ENR, cases 1-3) and group 2 (ENR associated with teratoma, cases 4-13). The patients commonly presented with a painless mass or an incidental finding in surgery. The ENR were detected in the lumbosacral and sacrococcygeal region (five cases), retroperitoneal area (five cases), testis (one case), gubernaculum testis (one case), and stomach (one case). Radiological findings showed a mixed-density mass or a mixed-signal-intensity lesion in group 2. All the patients underwent surgical resection, and all except two were event-free in a follow-up period of 2-154 months. Case 8 showed recurrence, and extended surgery was performed. Case 7 developed metastases and received chemotherapy. CONCLUSIONS: ENR are rare among children and have no specific clinical manifestations, lab tests, and radiological features. Different treatment strategies may be adopted based on the diagnosis. Complete resection can generally be achieved, and the prognosis is excellent in most cases. However, relapse or metastasis may occur in a small subset of patients. Close follow-up is mandatory to detect recurrence and metastasis early.

Separation of pygopagus, omphalopagus, and ischiopagus with the aid of three-dimensional models.

BACKGROUND: The three-dimensional (3D) technique provides with accurate anatomical information. We present the separation surgeries for three different kinds of conjoined twins with the aid of three-dimensional techniques. METHOD: For the pygopagus twins, a pelvic and lower vertebral model was made. For the omphalopagus and ischiopagus, their enhanced computed tomography (CT) scan images were transferred to the Computer-Assisted Surgery Planning System (CASP) (Hisense, Qingdao, China) to generate the 3D models. RESULT: In the case of the pygopagus twins, the 3D model clearly showed that their coccyges were joined at a 120°angle from each other horizontally which suggested that the blind-end orifice on their back was a pilonidal sinus, which separated the normal sphincter into two halves. In the omphalopagus, the 3D model revealed one of the branches of each twin's hepatic vein was connected with the other's. The 3D model of the ischiopagus twins revealed that both of the twins had duplicated bladders and each baby's duplicated bladders united with one of the bladders of the other baby and a single rectum passing through the middle of the four bladders. CONCLUSION: 3D techniques could provide more detailed anatomical information, which is helpful in planning procedures for such complicated separation surgery. LEVELS OF EVIDENCE: Level IV.

Diagnosis and management of pyriform sinus cyst in neonates: 16-year experience at a single center.

BACKGROUND/PURPOSE: The purpose of this study was to review our experience in diagnosing and managing neonatal pyriform sinus cyst (PSC) and to assess its outcomes. METHODS: We retrospectively reviewed 38 cases of neonatal PSC from 2001 to 2016. Clinical features, PSC diagnosis, treatment, and outcomes were analyzed. RESULTS: Cervical masses were detected prenatally in 8 patients (21.1%). Neck cyst and respiratory distress were frequent symptoms among these patients. Preoperative PSC diagnosis was made in 15 cases (39.5%) by esophagography. CT or MRI images showed an air-filled cyst in 22 patients (57.9%). Cyst drainage was performed in 8 infected cases (21.1%). Cyst and fistula excision was performed in 30 cases (78.9%). Fistula excision assisted by endoscopy was required in 13 PSC patients (34.2%). Fistula detection failed in 4 cases (10.5%). Patients aged between 0 to 7days presented with low infection and high cyst removal rate. No fistula recurrence occurred in cases with cyst resection. CONCLUSIONS: CT or MRI images with an air-filled cyst are significant indications in diagnosing PSC. Surgical options for PSC depend on levels of cervical infection. The optimal timing for surgical intervention is between 0 to 7days after birth. Our surgical approaches were effective in treating neonatal PSC. LEVELS OF EVIDENCE: Treatment study, level IV (retrospective study).

In utero repair of fetal rat myelomeningocele affects neuromuscular development in the bladder.

Fetal repair of myelomeningocele (MMC) has been proven to be beneficial for the central nervous system development; however, the effect of fetal MMC repair on bladder function remains controversial. The objective of the present study was to establish an early timepoint for in utero MMC repair using a rat model, and to investigate the changes in bladder development subsequent to that intervention. Sprague Dawley rats were divided into the MMC, MMC repair and control groups. MMC rat fetus models were created by treating pregnant rats with all-trans retinoic acid. The MMC defect was then repaired in utero at embryonic day 17 (E17) using a chitosan-gelatin membrane patch. Fetal rat bladders were removed at E19 and E21 in each group, as well as at stage E17 in the MMC and control groups. Differential expression of ß-III-tubulin, α-smooth muscle actin (α-SMA), nerve growth factor (NGF) and acetylcholinesterase (AChE) mRNA, and ß-III-tubulin and α-SMA protein in the bladder following fetal repair was measured and compared among the three groups. In addition, the expression of NGF mRNA was significantly elevated at E21 in the MMC group compared with that of the control group, however, the level decreased in the repair group at stage E21. The expression of α-SMA mRNA significantly increased at E19 and then decreased at E21 in the repair group compared with that of the MMC group; however, there were no significant changes in α-SMA protein following the repair. Furthermore, the repair enhanced ß-III-tubulin mRNA expression at E19, but ameliorated the decrease of ß-III-tubulin protein at E21. The expression of AChE mRNA increased in the MMC group at E19 and E21 compared with that of the control group, although it was not significantly altered following repair as compared with that of the MMC group. In conclusion, in the current study, abnormal neuromuscular development was observed in the MMC bladder, which enabled a certain degree of improvement in the in utero MMC repair.

Reoperation After Cyst Excision with Hepaticojejunostomy for Choledochal Cysts: Our Experience in 18 Cases.

BACKGROUND Complete cyst excision with biliary reconstruction is the treatment of choice for choledochal cyst (CC). The aim of this article is to review our experience in patients who underwent reoperation between January 1995 and December 2014. MATERIAL AND METHODS The records of 18 patients (female/male, 15/3) were retrospectively analyzed including age, sex, cyst type, initial procedure, lab and imaging findings, indications for reoperation, intraoperative findings, and results of reoperation. The median follow-up period was 6 years. RESULTS The rate of reoperation in this study was about 6.8%. Eighteen patients (7 type Ia, 2Ic, 9 IV-A) developed severe postoperative complications and required surgical intervention. The median time interval from primary surgery to reoperation was 19.5 months (range, 3 days to 8 years). Two early complications required surgery due to anastomotic bile leakage and intussusception. Sixteen late complications occurred, including 3 intrahepatic bile duct stenosis with calculi, 5 anastomotic strictures with/without stones, 4 intrapancreatic cyst remnants, 3 adhesive bowel obstructions, and 1 internal hernia. For patients with persistent dilatation of the intrahepatic bile duct or anastomotic stricture, removal of stones and revision of hepaticojejunostomy were performed, with additional hepatic ductoplasty when necessary. Radical excision of the dilated cystic remnant in the head of pancreas was performed in 4 patients, with 1 needing additional pancreaticojejunostomy procedure. No deaths occurred. Sixteen patients recovered uneventfully after reoperation, and 2 wound infections developed. CONCLUSIONS A wide hepaticojejunostomy with/without ductoplasty is essential to prevent cholangitis, anastomotic stricture, and calculi formation. Complete cyst excision, including the intrapancreatic portion, should be meticulously pursued.

Usefulness of posterior sagittal anorectoplasty for redo pull-through in complicated and recurrent Hirschsprung disease: Experience with a single surgical group.

AIM: To retrospectively examine 12 patients with Hirschsprung disease (HD) who underwent posterior sagittal anorectoplasty (PSARP) for various complications. METHODS: This study included patients with HD who underwent redo pull-through (PT) via PSARP at our institute between 2005 and 2014. The type of initial procedure, clinical presentations, indications, and functional results were analyzed. Postoperative excretory function was assessed using the Krickenbeck classification. RESULTS: The study group comprised 9 boys and 3 girls (total, 12). Five patients were diagnosed with rectosigmoid aganglionosis, 5 with long segment aganglionosis, and 2 with total colonic aganglionosis. The primary operations performed on these patients included the Soave, Duhamel, Swenson, Rehbein, and Ikeda-Soper procedures. The interval between the primary operations and reoperation ranged from 5months to 8years (median, 3years). The indications for PSARP were rectocutaneous fistulae (6 cases), frozen pelvis (5 cases), severe anastomotic stricture (3 cases), rectovaginal fistulae (2 cases), and hemorrhagic proctitis with an inflammatory polyp (1 case). All fistulae were repaired using PSARP; only one rectocutaneous fistula recurred and required two additional surgeries. Stricture and hemorrhagic proctitis were cured in all involved cases. Nine patients were followed up for 8months to 10years after PSARP surgery (average, 5.1years). All 9 patients had voluntary bowel movements within 6months after the last PSARP and stoma closure: 3 had normal bowel movement, while 6 had varying degrees of soiling, depending on the length of residual colon. None complained of constipation. CONCLUSION: PSARP is useful for treating severe complications of failed PT in HD. Complex and recurrent rectocutaneous fistulae and frozen pelvis are the main indications for PSARP, while soiling is the most common surgical complication.