Long-term (> 10 years) bowel function of anorectal malformations: a retrospective single-center study.

PURPOSE: To describe the long-term bowel function of anorectal malformation (ARM) patients and explore the potential influence factors. METHODS: ARM patients with follow-up data > 10 years were included. Cases of cloaca, Currarino syndrome, and VACTERL syndrome were excluded. Rintala score and PedsQL 4.0 were used to assess bowel function score (BFS) and quality of life (QoL). Based on the results, patients were divided into satisfactory group with BFS ≥ 17 and unsatisfactory group with it < 17. Comparisons between the groups were made. RESULTS: Among the 81 patients were 44 males and 37 females. Follow-up time was 138 (126,151) months. 16 (19.75%) patients had associated anomalies. 23 (28.40%) patients had reoperations, and fistula recurrence was the most common reason. BFS of the patients was 20 (18,20). QoL score was 100 (100,100), which correlated positively with BFS (r = 0.648, P < 0.001). The satisfactory and the unsatisfactory groups had 69 and 12 cases, and their BFS were 20 (20,20) and 11 (8,15) respectively, which had statistical difference (P < 0.001). Total QoL score and psycho-social health score of the unsatisfactory group were lower (P < 0.001). Only reoperations were statistically different between the groups (P < 0.001). CONCLUSIONS: Long-term (> 10 years) bowel function of ARM patients is good in this study. Defecation problems have negative impacts on QoL and mainly affects their psycho-social health. Primary anorectoplasty is extremely important. Reoperations, which are most commonly seen in recto-urethral fistula recurrence, adversely affect the outcome.

Management of complicated choledochal cyst in children: ultrasound-guided percutaneous external drainage and subsequent definitive operation.

OBJECTIVE: The purpose of this study was to analyze the outcomes of the combination of ultrasound (US)-guided percutaneous external drainage and subsequent definitive operation to manage complicated choledochal cyst in children. METHODS: This retrospective study included 6 children with choledochal cyst who underwent initial US-guided percutaneous external drainage and subsequent cyst excision with Roux-en-Y hepaticojejunostomy between January 2021 and September 2022. Patient characteristics, laboratory findings, imaging data, treatment details, and postoperative outcomes were evaluated. RESULTS: Mean age at presentation was 2.7 ± 2.2 (0.5-6.2) years, and 2 patients (2/6) were boys. Four patients (4/6) had a giant choledochal cyst with the widest diameter of ≥ 10 cm and underwent US-guided percutaneous biliary drainage on admission or after conservative treatments. The other 2 patients (2/6) underwent US-guided percutaneous transhepatic cholangio-drainage and percutaneous transhepatic gallbladder drainage due to coagulopathy, respectively. Five patients (5/6) recovered well after US-guided percutaneous external drainage and underwent the definitive operation, whereas 1 patient (1/6) had liver fibrosis confirmed by Fibroscan and ultimately underwent liver transplantation 2 months after external drainage. The mean time from US-guided percutaneous external drainage to the definitive operation was 12 ± 9 (3-21) days. The average length of hospital stay was 24 ± 9 (16-31) days. No related complications of US-guided percutaneous external drainage occurred during hospitalization. At 10.2 ± 6.8 (1.0-18.0) months follow-up, all patients had a normal liver function and US examination. CONCLUSIONS: Our detailed analysis of this small cohort suggests that US-guided percutaneous external drainage is technically feasible for choledochal cyst with giant cysts or coagulopathy in children, which may provide suitable conditions for subsequent definitive operation with a good prognosis. TRIAL REGISTRATION: Retrospectively registered.

Preoperative complications in children with mesenteric lymphatic malformations: Incidence, risk factors and outcomes.

Background: Preoperative complications of mesenteric lymphatic malformations (ML) in children are various and complex. We aim to analyze the incidences and risk factors of three major preoperative complications (hemorrhage of the cyst, infection of the cyst and intestinal volvulus) in ML patients, and explore their influence on the outcomes. Methods: This retrospective cohort study enrolled ML patients undergoing surgery at Beijing Children's Hospital between June 2016 and June 2022 and classified them according to different preoperative complications, preoperative hemorrhage or infection, and preoperative intestinal volvulus. The groups were examined and compared according to sex, age at admission, presenting symptoms, laboratory examinations, imaging examinations, preoperative treatments, cyst characteristics, surgical details, perioperative clinical data, and follow-up. Logistic regression analysis was performed to identify the independent risk factors for preoperative hemorrhage or infection, and preoperative intestinal volvulus. Results: Of the 104 enrolled ML patients, 27 (26.0%) had preoperative hemorrhage or infection, and 22 (21.2%) had preoperative intestinal volvulus. Univariate analysis showed that patients with preoperative hemorrhage or infection had a higher rate of ML in the mesocolon (44.4 vs. 23.4%, p < 0.038) and larger cysts (10 vs. 8 cm, p = 0.042) than patients without preoperative hemorrhage or infection. Multivariable logistic regression analysis found that the location (OR, 3.1; 95% CI, 1.1-8.6; p = 0.026) and size of the cyst (≥7.5 cm) (OR, 6.2; 95% CI, 1.6-23.4; p = 0.007) were independent risk factors for preoperative hemorrhage or infection. Preoperative intestinal volvulus was only found in ML at the intestinal mesentery. Further analysis showed that ML in the jejunal mesentery was an independent risk factor for preoperative intestinal volvulus (OR, 3.3; 95% CI, 1.1-10.0; p = 0.027). Patients with preoperative hemorrhage or infection spent more on hospitalization costs than patients without preoperative hemorrhage or infection (3,000 vs. 2,674 dollars, p = 0.038). Conclusions: ML patients should be treated as soon as possible after diagnosis. The location and size of the cyst were independent risk factors for preoperative hemorrhage or infection. ML in the jejunal mesentery was an independent risk factor for preoperative intestinal volvulus.

Caudal duplication syndrome: 10-year experiences with a comprehensive literature review.

PURPOSE: Caudal duplication syndrome (CDS) has rarely been reported. The purpose was to describe the characteristics and discuss possible pathogenesis of CDS by reviewing our experience along with a comprehensive literature review. METHODS: A total of 51 patients including 3 from our team and 48 from literature were selected in this study. General condition, clinical manifestations, type of anomalies, treatment and prognosis was analyzed and summarized. RESULTS: Among the 51 patients were 30 females and 21 males, and age at first clinical visit was from birth to 39 years old. Except 12 patients, most of the patients had no troubling clinical manifestation. Physical examination showed that 30 patients had 1 perineum, 21 patients had 2 completely independent perineums. Degree of duplication varied; colon-rectum tubular, bladders and urethras, vaginas in females and penis shafts and glans in males were found to be the most common type of alimentary system and urogenital system duplication in this study with 24/51, 41/51, 10/30 and 16/21 patients, respectively. Anorectal malformation was calculated: 18 had 2 ARMs, 14 had 1 normal anus and 1 ARM on the other side, 12 had a normal anus, 5 had 2 normal anus, the remaining 2 patients had only 1 ARM. Spinal cord anomalies were showed as meningomyeloceles and lipomas in 13 and 3 patients. Vertebral anomalies of bifid, dysplasias, scoliosis, and hemivertebra were noticed in 28 patients and accessory dysplasia lower limbs were found in 10 patients. Prognosis showed 39 of the 51 patients had normal function in urination and defecation. CONCLUSIONS: CDS is an extremely rare disease with uncertain pathogenesis. Colon-rectum tubular duplication with two ARMs, duplicated bladders and urethras, double vaginas in females and penis shafts and glans in males are the most common type. Long-term prognosis is good with multidisciplinary, individualized and staged surgical procedures.

Surgical treatment of benign splenic lesions in pediatric patients: a case series of 30 cases from a single center.

BACKGROUND: Benign splenic lesions are rarely encountered. This study aimed to review the clinical characteristics and surgical outcomes in a case series of 30 pediatric patients. METHODS: From January 1st, 2001 to December 31st, 2021, 30 pediatric patients from a single center were consecutively included. Electronic medical records were reviewed and patients were followed up. Clinical presentations, imaging features, surgical procedures, pathological diagnoses, and prognoses were summarized. The lesion locations and 7-day postoperative platelet levels were compared between total and partial splenectomy patients. RESULTS: Eighteen males and twelve females were included, with mean age at surgery 116.4 ± 43.6 months. The clinical presentations included abdominal pain (16/30), splenomegaly (6/30), skin petechia (2/30), hemolytic jaundice (1/30), and no symptoms (5/30). Pathological diagnoses included congenital epithelial cyst (CEC, 17/30), vascular malformation (8/30), sclerosing angiomatoid nodular transformation (SANT, 3/30), hamartoma (1/30), and leiomyoma (1/30). Patients undergone total splenectomy were more likely to have a lesion involving the hilum than those undergone partial splenectomy (68.4% vs 31.6%, P = 0.021). The 7-day postoperative platelet level was higher in total splenectomy patients than partial splenectomy patients (adjusted means 694.4 × 109/L vs 402.4 × 109/L, P = 0.002). CONCLUSIONS: Various clinical characteristics of pediatric benign splenic lesions are summarized. The most common pathological diagnoses are congenital epithelial cyst and vascular malformation. Partial and total splenectomy result in good prognosis with a low recurrence rate, and the former is preferred to preserve splenic function if possible.

Diagnosis and treatment of splenic torsion in children: preoperative thrombocytosis predicts splenic infarction.

BACKGROUND: Pediatric splenic torsion is a rare entity, and the most common cause is wandering spleen. This study aimed to summarize our clinical experience in the diagnosis and surgical treatment pediatric patients with splenic torsion, and to use preoperative thrombocytosis as a preoperative predictive factor for splenic infarction. METHODS: From January 1st, 2016 to December 31st, 2021, 6 children diagnosed as splenic torsion were included. All patients were surgically treated and followed up. The clinical data was collected including clinical presentations, laboratory tests, imaging results, surgical procedures, and prognosis. Clinical experience of diagnosis and surgical treatment were summarized. RESULTS: There were 4 females and 2 males, with median age at surgery 102.6 (range 9.4-170.7) months. Abdominal pain and abdominal mass were the most common presentations. The diagnosis of splenic torsion depended on imaging studies, and adjacent organ involvement (gastric and pancreas torsion) was observed on contrast CT in one patient. Five patients were diagnosed as torsion of wandering spleen, and one was torsion of wandering accessory spleen. Emergent laparoscopic or open splenectomy was performed in all patients. Pathology revealed total splenic infarction in 4 patients, partial infarction in 1 patient, and viable spleen with congestion and hemorrhage in 1 patient. Preoperative platelet counts were elevated in all 4 patients with splenic infarction, but normal in the rest 2 with viable spleen. Postoperative transient portal vein branch thromboembolism occurred in one patient. CONCLUSIONS: Imaging modalities are crucial for the diagnosis of pediatric splenic torsion and adjacent organ involvement. Preoperative thrombocytosis may predict splenic infarction. Spleen preserving surgery should be seriously considered over splenectomy in patients with a viable spleen.

Colocolic Intussusception in Children: A Case Series and Review of the Literature.

Background: Colocolic intussusception is a rare subtype of intussusception mostly caused by juvenile polyps. The treatment of colocolic intussusception caused by other pathologic lead points in children remains poorly understood. Method: A systematic literature review was performed between January 2000 and June 2021 to characterize the comprehensive treatment of colocolic intussusception in children. This report also included patients admitted to our center between January 2010 and June 2021 who were not previously reported in the literature. Results: We identified 27 patients in 20 studies in addition to 17 patients from our center for a total of 44 patients (median age, 4.4 years; 52.3% male). The lead point was identified in 40 patients (40/44, 90.9%). The most common lead point was juvenile polyps (19/44, 43.2%). A therapeutic enema was performed in 15 patients with colocolic intussusception caused by juvenile polyps and was successful in 9 patients (9/15, 60.0%). Colonoscopic polypectomy was subsequently performed in 8 patients and was successful in 7 patients (7/8, 87.5%). The other patient had undergone laparoscopic exploration, and no abnormality was found. Subsequently, the patient underwent open surgery. The patients with colocolic intussusception caused by other pathologic lead points almost underwent surgical treatment (15/21, 71.4%), including 13 open surgeries and 2 laparoscopic surgeries. Conclusion: A therapeutic enema followed by colonoscopic polypectomy is feasible to treat colocolic intussusception caused by juvenile polyps unless the patient has bowel perforation; however, surgery is sometimes needed. For patients with colocolic intussusception caused by other pathologic lead points, open surgery remains the primary treatment.

Approximate analytical model for transient transport and oxygen-limited biodegradation of vapor-phase petroleum hydrocarbon compound in soil.

Volatile organic compounds (VOCs) contamination may occur in subsurface soil due to various reasons and pose great threat to people. Petroleum hydrocarbon compound (PHC) is a typical kind of VOC, which can readily biodegrade in an aerobic environment. The biodegradation of vapor-phase PHC in the vadose zone consumes oxygen in the soil, which leads to the change in aerobic and anaerobic zones but has not been studied by the existing analytical models. In this study, a one-dimensional analytical model is developed to simulate the transient diffusion and oxygen-limited biodegradation of PHC vapor in homogeneous soil. Laplace transformation and Laplace inversion of the Talbot method are adopted to derive the solution. At any given time, the thickness of aerobic zone is determined by the dichotomy method. The analytical model is verified against numerical simulation and experimental results first and parametric study is then conducted. The transient migration of PHC vapor can be divided into three stages including the pure aerobic zone stage (Stage I), aerobic-anaerobic zones co-existence stage (Stage II), and steady-state stage (Stage III). The proposed analytical model should be adopted to accommodate scenarios where the transient effect is significant (Stage II), including high source concentration, deep contaminant source, high biodegradation capacity, and high water saturation. The applicability of this model to determine the breakthrough time for better vapor intrusion assessment is also evaluated. Lower first-order biodegradation rate, higher source concentration, and shallower source depth all lead to smaller breakthrough time.

Diagnosis of liver cirrhosis with two-dimensional shear wave elastography in biliary atresia before Kasai portoenterostomy.

PURPOSE: To evaluate the application value of two-dimensional shear wave elastography (2D-SWE) for non-invasive diagnosis of liver cirrhosis (LC) in patients with biliary atresia (BA) before Kasai portoenterostomy (KP), and the cutoff value of liver stiffness measurement (LSM) for diagnosing LC. METHODS: The clinical data of 51 patients with BA who were diagnosed via surgery and pathological results from May 2017 to December 2018 in the department of general surgery, Beijing Children's Hospital, Capital Medical University, were retrospectively analyzed. The liver tissue specimens obtained during KP were evaluated according to the METAVIR criteria. The LSM was obtained using the 2D-SWE technique before KP. RESULTS: There was a grade positive correlation between LSM and METAVIR staging, and the Spearman correlation coefficient was 0.432 (P = 0.002). The AUC for 2D-SWE diagnosing LC (METAVIR score S = 4) in patients with BA before KP was 0.843 (95% confidence interval 0.736 ~ 0.950). The best cutoff value was 16.05 kPa, and the corresponding sensitivity was 75.0%, specificity was 83.7%, positive predictive value (PPV) was 46.1%, negative predictive value (NPV) was 94.7%, and the accuracy was 82.4%. CONCLUSION: 2D-SWE can be used to noninvasively diagnose LC in patients with BA before KP, and the cutoff value is 16.05 kPa.

Development and validation of a novel nomogram and risk score for biliary atresia in patients with cholestasis.

BACKGROUND: Timely discriminating biliary atresia (BA) from other causes of cholestasis is important but challenging. AIMS: To develop a useful diagnostic nomogram and a simplified scoring system to diagnosing BA. STUDY DESIGN: All medical records of the patients who were consecutively admitted to our institution with cholestasis from March 2016 to December 2020 were retrospectively searched. The patients were allocated to the derivation cohort (n = 343) and the validation cohort (n = 246). Multivariable logistic regression models were used to construct the nomogram. The nomogram was validated in both cohorts. The simplified risk score was derived from the nomogram. RESULTS: The nomogram was constructed based on presence of clay stool, gallbladder length, gallbladder emptying index, shear wave elastography value, and gamma-glutamyl transferase level. This model showed good calibration and discrimination ability, with the C-index of 0.968 (95% CI: 0.951-0.984). The discriminating ability is most prominent in the 61-90 days group, with AUC of 0.982 (95% CI: 0.955-1.000). The simplified risk score identified most patients with very high or low risk of BA, and was capable of exempting 64.3% non-BA patients from intraoperative cholangiogram procedure. CONCLUSIONS: This novel diagnostic nomogram had good discrimination and calibration abilities. The simplified scoring system showed significant clinical utility.

Transient migration behavior of VOC vapor in layered unsaturated soils subjected to multiple time-dependent point pollution sources: Analytical study.

Predicting the migration behavior of volatile organic compounds (VOCs) vapor is essential for the remediation of subsurface contamination such as soil vapor extraction. Previous analytical prediction models of VOCs migration are mostly limited to constant-concentration nonpoint sources in homogeneous soil. Thus, this study presents a novel analytical model for two-dimensional transport of VOCs vapor subjected to multiple time-dependent point sources involving transient diffusion, sorption and degradation in layered unsaturated soils. Two representative time-dependent sources, i.e., an instantaneous source and a finite pulse source, are used to describe the short-term and long-term leakage. Results reveal that soil heterogeneity can cause pollution accumulation, especially in low-diffusivity capillary fringe. The assumption of an equivalent plane source from multiple point sources would significantly overestimate the vapor concentration and the contaminated range. The previous single point source model is no longer inapplicable when the relative distance and/or the release interval between sources is small, giving a strong interaction between multiple sources. Moreover, a faster vapor degradation rate or a higher groundwater level will reduce the area of vapor plume linearly. Hence, close attention should be paid to the time-variation characteristics of multiple sources, the vapor degradation and the groundwater level fluctuation in practice to facilitate soil remediation. The proposed model is a promising tool for addressing the above issue.

The utility of shear wave elastography and serum biomarkers for diagnosing biliary atresia and predicting clinical outcomes.

To investigate the utility of liver stiffness measurement by shear wave elastography (SWE) and several commonly used biomarkers in differentiating biliary atresia (BA) from other causes of cholestasis (non-BA) patients within 45 days and in predicting the postoperative prognosis. A consecutive series of medical records of patients presenting with cholestasis within 45 days in our institution between February 2016 and December 2020 was collected. The BA diagnosis was confirmed by intraoperative cholangiography (IOC). Other causes of cholestasis were confirmed by IOC, liver biopsy, genetic analysis, or recovery after conservative treatment. Preoperative and postoperative data were analyzed. A total of 156 patients were included, consisting of BA (n = 83) and non-BA (n = 73) cases. SWE and serum gamma-glutamyl transferase (GGT) showed better discriminative utility. The optimal cutoff values for SWE and GGT were > 7.10 kPa and > 195.4 U/L, with AUC of 0.82 (95% CI, 0.76-0.89; p < 0.0001) and 0.87 (95% CI, 0.82-0.93; p < 0.0001), respectively. Subgroup analysis showed the increased discriminative performance of SWE with age. Multivariable logistic regression analysis showed better diagnostic performance for SWE (adjusted OR, 35.03; 95% CI, 7.12-172.50) and GGT (adjusted OR, 24.70; 95% CI, 6.55-93.18) after adjusting for other confounders. The 30-day postoperative to preoperative serum direct bilirubin (DB) level, DB (post-30:pre), of > 0.3 showed the best predictive value for the need of liver transplantation, with HR of 6.15 (95% CI 1.95-19.38, P = 0.042).Conclusion: Serum GGT level and liver stiffness measurement by SWE showed the best discriminative utility. The diagnostic performance of SWE increased with age. A DB (post-30:pre) value > 0.3 was associated with the need for liver transplantation in later life. What is Known: • Liver stiffness measurement by shear wave elastography (SWE) could help discriminate biliary atresia (BA) from other causes of cholestasis, with sensitivity of 70-90%. • The postoperative total bilirubin less than 2 mg/dL within the first 3 months was a predictor of transplant-free survival. What is New: • The diagnostic performance of liver stiffness measurement by SWE increased with age. • The 30-day postoperative direct bilirubin (DB) level to preoperative DB level, DB (post-30:pre), is a predictor for short-term clinical outcomes.

Clinical features and outcomes of bowel perforation in primary pediatric gastrointestinal lymphoma.

BACKGROUND: Whether surgery can improve the prognosis of patients with primary pediatric gastrointestinal lymphoma (PPGL) who experienced bowel perforation remains controversial. This study aimed to evaluate the prognosis of such patients. METHODS: Nine patients pathologically diagnosed with PPGL who experienced perforation at our center between January 2010 and December 2020 were enrolled and divided into two groups: those with perforation during (n = 4) and before (n = 5) chemotherapy. Their medical records were reviewed, and long-term follow-up was conducted by telephone in February 2021. RESULTS: All patients with perforation during chemotherapy were diagnosed with PPGL in the outpatient department. The mean time from outpatient visit to chemotherapy was 17.3 ± 6.1 days. Two patients experienced perforation during the first chemotherapy regimen and received conservative treatment, while the others developed perforation after multiple chemotherapy regimens and underwent surgery. All of the patients received regular chemotherapy and survived for a mean follow-up time of 3.8 ± 1.9 years. No patient with perforation before chemotherapy had a definite diagnosis in the outpatient department. Among these patients, 4 experienced perforation and underwent surgery, of whom 3 developed perforation-related complications and died; the other recurred after chemotherapy. Only the patient who received conservative treatment was diagnosed with PPGL before chemotherapy, received regular chemotherapy, and survived without a recurrence for 1.0 year. CONCLUSION: Prompt diagnosis and chemotherapy improve the prognosis of PPGL. Surgery does not affect the prognosis of patients with perforation during chemotherapy but may accelerate disease progression in patients with perforation before chemotherapy.

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.

An analytical model for one-dimensional diffusion of degradable contaminant through a composite geomembrane cut-off wall.

A one-dimensional analytical model is proposed to analyze contaminant diffusion through a composite geomembrane cut-off wall (CGCW) composed of a geomembrane (GMB) and a bentonite cut-off wall (BCW). The model considers degradation process of contaminant and time-dependent inlet boundary condition which are common in engineering practices. Moreover, two limiting scenarios of the exit boundary condition (EBC) of CGCW for field conditions are taken into account, including the flushing and non-advective semi-infinite aquifer EBCs. The influence of boundary conditions and performance of CGCW are comprehensively investigated. The results show that the upper and lower limits of the mass flux of the exit face of CGCW can be obtained by the models with flushing EBC and the model with non-advective semi-infinite aquifer EBC, respectively. In addition, degradation has substantial influence on the contaminant migration, and smaller half-life in BCW results in smaller contaminant leakage. The performance of CGCW can be improved by embedding GMB at a proper location which is related to the type of contaminant and EBC. Furthermore, thickening HDPE GMB or adopting a coextruded EVOH GMB is efficient to improve the performance of CGCW. The present model can be used as an applicable tool for rational design of CGCW.

Vascular Malformation of the Colorectum in Children: Case Series of 27 Patients.

BACKGROUND: Vascular malformation of the colorectum is a rare disease that presents most commonly in early childhood. Some pull-through procedures have been performed for its treatment. However, laparotomy was routinely required. The aim of this study was to present features of this disease, as well as the outcomes of transanal endorectal pull-through (TEPT) with or without laparotomy. METHODS: A retrospective analysis was performed on consecutive patients with vascular malformation of the colorectum who underwent TEPT with or without laparotomy in our hospital between January 2010 and December 2019. Follow-up information included hematochezia, other bleeding and bowel function. Bowel function was assessed using the Rintala score. RESULTS: Twenty-seven patients were included (14 boys and 13 girls). Hematochezia and anemia were the main chief complaints. Perianal vasodilatation and prolapse of rectal mucosa with spurting blood were detected in 13 (48.1%) and 9 (33.3%), respectively. Eighteen patients (66.7%) underwent TEPT with laparotomy, and 9 patients underwent TEPT without laparotomy (including 5 TEPT under laparoscopic supervision) for lesions located beyond the distal sigmoid colon. Compared to TEPT with laparotomy, the length of resected bowel and operative time was significantly shorter in TEPT without laparotomy (p = 0.001 and p = 0.004). There was no statistical difference for intraoperative blood loss. Other vascular malformations were detected in 9 patients (33.3%); 3 were detected by laparoscopy. Three patients (11.1%) experienced postoperative complications. Follow-up was held with 24 patients (3 were lost to follow-up). The mean follow-up time was 51.9 ± 32.8 months. Four patients occasionally had bloody stools, without anemia. None had hematuria or vaginal bleeding. Median bowel function score was 20 (range 17-20). CONCLUSION: Hematochezia, anemia and hemorrhoid-like manifestations might be an indication for vascular malformation of the colorectum in children. TEPT was effective for vascular malformation of the colorectum, and TEPT without laparotomy was more suitable for the short lesions.

Transanal fistulectomy for postoperative persistent rectourethral fistula in patients with ARM: is simple resection enough?

BACKGROUND: Postoperative rectourethral fistula (RUF) in patients with congenital anorectal malformation (ARM) remains a challenge for paediatric surgeons, among them persistent fistula is the most common. Various techniques have been proposed, only a few reports based on different causes are available, and there is no consensus so far. This study is to evaluate the application, advantages and limitations of transanal fistulectomy approach in repairing persistent RUF in ARM patients. METHODS: From January 2007 to July 2019, 78 ARM patients who received revisional surgery for RUF were reviewed, 34 persistent fistulas were identified. Examination under anaesthesia included patients with fistulas that were located within 3 cm from the anus verge, good appearance of the anus and sphincter function, and no urethral and rectoanal obstruction. Three patients were excluded because of complex urologic pathologic defects. In total, thirty-one patients underwent transanal fistulectomy to repair RUF. RESULTS: All cases were approached with transanal incision and fistulectomy to repair RUF. The average operative time was 91 ± 35 min. At a minimum six-month follow-up, 29 patients healed after the first attempt, the success closure rate was 93.5%. Two patients received redo transanal fistulectomy and healed. Two patients had postoperative complications: one patient had urethral stenosis and it was managed by dilation; one patient had urethral diverticulum but it did not require revisional surgery. No patient in this study was incontinent because of the surgery. CONCLUSIONS: Transanal fistulectomy provides a simple, straightforward, and safe approach to repair persistent RUF in ARM patients, especially in those with a low-lying fistula, good anus appearance and sphincter function without obstruction in the rectum or urethra. TRIAL REGISTRATION: Retrospectively registered.

Etiology and surgical management of pediatric acute colon perforation beyond the neonatal stage.

PURPOSE: Acute colon perforation is a pediatric surgical emergency. We aimed to analyze the different etiologies and clinical characteristics of acute non-traumatic colon perforation beyond the neonatal period and to identify surgical management and outcomes. METHODS: This retrospective study included 18 patients admitted with acute colon perforation and who received surgical treatment. RESULTS: Age of patients ranged between 1 month and 15 years. Five patients swallowed foreign objects (two swallowed magnets), two had colon perforation secondary to a malignant tumor (both colorectal adenocarcinoma) and two were iatrogenic (one prior colonoscopy, one air enema for intussusception). There was one perforation due to chemotherapy and Amyand's hernia respectively. The remaining seven patients had unknown etiologies; five of them were diagnosed with colitis. Fifteen (83.3 %) patients underwent open laparotomy, among which four attempted laparoscopy first. Three (16.7 %) patients underwent laparoscopic surgery. Fourteen (77.8 %) patients received simple suture repairs and four (22.2 %) received colonic resections and anastomosis. Four (22.2 %) patients received a protective diverting colostomy and three (16.7 %) received an ileostomy. CONCLUSIONS: There is a wide range of etiology besides necrotizing enterocolitis and trauma, but a significant portion of children present with unknown etiology. Type of surgery elected should be dependent on the patient's etiology, disease severity and experience of surgeons.

Anorectal Malformations Associated With Labioscrotal Fold Malformation and Perineal Mass in Pediatric Patients: Over a Decade of Experience.

Background: The triad of anorectal malformation (ARM), labioscrotal fold malformation, and perineal mass has rarely been reported before. The purpose of this study was to review our experience in these patients, describe their characteristics, and discuss the possible pathogenesis. Methods: Seven pediatric patients diagnosed with ARM associated with both labioscrotal fold malformation and perineal mass were included in this study. Medical records of these patients were retrospectively reviewed, and follow-up was held through telephone contact or outpatient service. Results: Among the seven patients were six females and one male, and the age at surgery was between 5.2 and 12.4 months. The ratio of lateral-type to mid-perineum-type labioscrotal fold malformation was 5:2. The ARM type was all rectoperineal fistula. Operation was excision of the malformation and perineal mass at the same time of anoplasty. The pathology was lipoma (three cases), fibroma (one case), lipofibroma (one case), angiolipoma (one case), and mesenchymal hamartoma (one case). All the seven patients had no wound complication, and during the follow-up period of 7-100 months after surgery, none of the seven patients suffered perineal mass recurrence. Bowel control was satisfactory in the follow-up period. Conclusions: There is a low incidence for the triad of ARM, labioscrotal fold malformation, and perineal mass. The nature of this disease is neoplastic overgrowth of intervening mesenchymal tissue, which impedes the continuity of caudal development into normal labioscrotal fold and affects the extension of urorectal septum, leading to ARM. Prognosis is mainly dependent on the type of ARM.

Characteristics and treatments for pediatric ordinary and incarcerated inguinal hernia based on gender: 12-year experiences from a single center.

BACKGROUND: Congenital primary inguinal hernia is a common condition among children. Although much literature regarding inguinal hernia is available, large scale analysis are few, and rarely do they expand on gender difference or incarcerated hernias. METHODS: Patients with unilateral or bilateral inguinal hernia who were admitted to our hospital and received open inguinal hernia repair (OIHR) or laparoscopic inguinal hernia repair (LIHR) under general anesthesia were included. LIHR was performed using single-site laparoscopic percutaneous extraperitoneal closure (SLPEC). Medical records were retrospectively collected and analyzed. RESULTS: A total of 12,190 patients were included in this study. The ratio of male to female was 4.8:1. There was a total of 10,646 unilateral hernias (87.3%) and 1544 bilateral hernias (12.7%), with a corresponding ratio of 6.9:1. 12,444 hernia repair surgeries, 11,083 (89.1%) OIHR and 1361 (10.9%) LIHR, were held. OIHR had a shorter operative time than LIHR for all unilateral and female bilateral repair, unlike for bilateral male repair. There was no difference between OIHR and LIHR for ipsilateral recurrent hernia in males. There was a difference between OIHR and LIHR for metachronous contralateral hernia. Incarcerated inguinal hernia was associated with longer operative time, hospital stay and higher hospital costs. Females and patients under 1 year were more likely to present with incarcerated hernia. CONCLUSIONS: OIHR should be considered for male patients, especially for unilateral and complete inguinal hernia. LIHR is highly recommended for female patients. For incarcerated hernia, attention should be paid to patients under 1 year old, as they can be 60 times more susceptible, and females. Surgeons should also be aware of ovary hernias in females.