Early and late outcomes of congenital biliary dilatation in pediatric patients.

BACKGROUND: This study aimed to reveal the early and late postoperative complications and outcomes after surgery for congenital biliary dilatation (CBD) by reviewing cases over the past 40 years. METHODS: We retrospectively evaluated 59 patients with CBD who underwent radical surgery for complications and outcomes, based on medical records. Early complications were defined as those requiring treatment within 5 years of the initial operation. Late complications were defined as those treated more than 5 years later. RESULTS: The median age at the first surgery was 37 months. Regarding biliary reconstruction, 54 of the 59 patients (91.5%) underwent hepaticojejunostomy. Although three patients underwent cholecystoduodenostomy and one patient underwent hepaticoduodenostomy, all were converted to hepaticojejunostomy after a median of 12.5 years. One patient developed synchronous biliary carcinoma and underwent pancreaticoduodenectomy. Early complications occurred in seven patients with 10 events (surgical site infection, n = 3 bile leakage, n = 3; ileus, n = 3; bile duct obstruction, n = 1 and intussusception, n = 1). Late complications occurred in nine patients with 12 events (ileus, n = 3; anastomotic stricture, n = 3; hepatolithiasis, n = 3; asynchronous biliary carcinoma, n = 2; pancreatolithiasis, n = 1). Two of the three patients with hepatolithiasis underwent hepatectomy refractory to the endoscopic approach. Two patients developed asynchronous biliary carcinoma at 34 and 13 years after last operation; both ultimately died of the carcinoma. Only 35 patients (61.4%) underwent a follow-up examination. A total of 11 female patients (45.8%) eventually married, and all successfully gave birth. CONCLUSION: Although the long-term prognosis is excellent with complete cyst excision and hepaticojejunostomy, we emphasize the importance of long-term follow-up.

A rare case of pyosalpinx in adolescent girl with Hirschsprung's disease who underwent transvaginal ultrasound-guided drainage.

BACKGROUND: Hydrosalpinx and pyosalpinx are rare gynecologic problems during adolescence, especially in girls without a history of sexual activity. They are even rarer in women with Hirschsprung's disease (HD). We herein report a case of pyosalpinx in an adolescent girl with HD treated by transvaginal ultrasound-guided drainage. CASE PRESENTATION: The present patient was a 12-year-old girl (weight 83 kg; height 159 cm; body mass index 32.8 kg/m2). She had undergone five laparotomies for long-segment HD by 2 years. Her menarche had occurred at 10 years. She was admitted with lower abdominal and anal pain. Computed tomography (CT), magnetic resonance imaging (MRI), and transvaginal ultrasound showed left pyosalpinx and abdominal abscess. Surgical drainage was necessary; however, she had a history of polysurgery and was severely obese, so laparotomy was considered to carry a high risk. Transvaginal ultrasound was deemed more likely to reach the abscess safely. Therefore, she was treated with transvaginal ultrasound-guided drainage by a gynecologist skilled in the procedure. She was discharged home after 52 days. One year and nine months after discharge, there was no reformation of either the abscess or pyosalpinx. CONCLUSIONS: Adolescent girls with HD are at risk of developing hydrosalpinx. Depending on the defecation status, pyosalpinx may also develop. As a less-invasive surgical treatment, transvaginal ultrasound-guided drainage can avoid laparotomy. Collaboration with a gynecologist is essential for the diagnosis and treatment of this clinical condition. Pediatric surgeons should communicate with gynecologists for such cases beginning around puberty for continuous follow-up.

A rare case of eosinophilic gastrointestinal disorders with short bowel syndrome after strangulated bowel obstruction.

BACKGROUND: Short bowel syndrome (SBS) is a rare yet costly disease with an incidence rate of 3 per million people. Herein, we report a rare case of eosinophilic gastrointestinal disorders (EGIDs) with SBS after strangulated bowel obstruction. CASE PRESENTATION: A 5-year-old male had a necrotic intestine of 340 cm resected due to strangulated bowel obstruction caused by an intestinal mesenteric hiatal hernia. The length of the residual intestine was 51 cm. Bloody stools appeared 19 days postoperatively. Colonoscopy showed diffuse redness of the colonic mucosa, and pathological findings showed moderate chronic inflammatory cellular infiltration. On blood examination, the eosinophil count was > 30%. EGIDs with short bowel syndrome (SBS) were suspected. Because his symptoms did not improve with initial nutrition therapy, he was transferred to our hospital 5 months after the operation. Prednisolone was administrated at an initial dose of 1.4 mg/kg/day, 6 days after his transfer. Bloody stools disappeared after prednisolone administration. Seven months after discharge, he had no bloody stool recurrence. CONCLUSION: The risk of developing secondary EGIDs in children with SBS should be considered, and postoperative management should include attention to abdominal symptoms and elevated eosinophil counts on blood examination.

The usefulness of OK-432 for the treatment of postoperative chylothorax in a low-birth-weight infant with trisomy 18.

Chylothorax is a rare but life-threatening condition in neonates. We herein report the successful use of OK-432 for a low-birth-weight infant with trisomy 18 who developed refractory chylothorax after thoracic surgery. Increasing the concentration of OK-432 seems useful in cases with a lot of pleural effusion.

Usefulness of Living Donor Liver Transplantation for Patients After Undergoing the Kasai Operation for Biliary Atresia.

BACKGROUND: Living donor liver transplantation (LDLT) has been actively performed for patients with poor clearance of jaundice after the Kasai operation for biliary atresia (BA). The present study clarified the usefulness of LDLT for BA. MATERIALS AND METHODS: Between 2000 and 2020, 24 patients (late group) underwent radical surgery for BA in our institute. The overall survival rate, native liver survival rate, and proportion of LDLT in the late group were retrospectively compared with those of 47 patients treated before 1999 (early group). P values <.05 was considered statistically significant. RESULTS: The overall survival rates at 5, 10, and 15 years were 57%, 54%, and 49%, respectively, in the early group and 100%, 100%, and 100% in the late group (P < .001). The native liver survival rates at 5, 10, and 15 years were 57%, 52%, and 39%, respectively, in the early group and 57%, 49%, and 42% (P = .993) in the late group. In the early group, LDLT was performed in 7 of 47 patients (15%), and the overall survival rate after LDLT was 71%. In the late group, LDLT was performed in 11 of 24 patients (46%), and the overall survival rate after LDLT was 100%. CONCLUSIONS: The long-term outcomes after the Kasai operation for BA have improved in recent years. There were no marked differences in long-term native liver survival before and after 2000. LDLT was actively introduced for patients with poor clearance of jaundice after the Kasai operation, and the survival rate significantly improved.

Management of refractory chylothorax in the neonatal intensive care unit: A 22-year experience.

BACKGROUND: The aim was to assess the therapeutic strategy of patients with chylothorax in a neonatal intensive care unit. METHODS: Twenty-eight infants with chylothorax were included in this study. Their clinical characteristics and outcomes were reviewed retrospectively. RESULTS: The male-to-female ratio was 1:1. The mean gestational age and birthweight were 35.1 ± 3.5 weeks and 2,692 ± 791 g, respectively. Eighteen patients were diagnosed with congenital chylothorax; chylothorax occurred postoperatively in 10 patients. Chromosomal anomalies were diagnosed in 8 patients. Six patients received surgical therapy, such as pleurodesis, thoracic duct ligation, or lymphaticovenous anastomosis. Two patients required surgery due to resistance to pleurodesis. In surgically managed patients, the daily maximum amount of pleural effusion (mL)/bodyweight (kg) ratio was significantly larger than in non-surgically managed patients: 229.0 ± 180.5 versus 59.7 ± 49.2 mL/kg. In the receiver operating characteristic analysis of the daily maximum amount of pleural effusion/bodyweight ratio, the area under the curve was 0.889 when the cut-off value was 101 mL/kg, and the sensitivity was 0.8333 and the specificity was 0.8095 (P = 0.0059). CONCLUSIONS: Pleurodesis using OK432 could become a surgical first-line therapy for chylothorax even for neonates. It was important to initiate pleurodesis for refractory chylothorax at an earlier stage. A daily chylous effusion/bodyweight ratio of >101 mL/kg was a good predictor and seemed to be a useful parameter for prompt surgical intervention.

Usefulness of the Monti-Malone procedure as a reconstruction of the antegrade continence enema procedure: a case report.

BACKGROUND: The antegrade continence enema (ACE) procedure is effective for severe constipation in patients with spina bifida and can improve quality of life (QOL). The Monti-Malone procedure (MM), which is a method of creating an enema tract from the colon, has been reported as an alternative to the ACE procedure when the appendix cannot be used. We report the usefulness of MM as a reconstruction of the antegrade continence enema procedure. CASE PRESENTATION: Our patient was a 22-year-old man with congenital spina bifida and hydrocephalus. Ventriculoperitoneal (VP) shunt surgery was performed immediately after birth, and preventative appendectomy was carried out during VP shunt repair when 4 months old. At 5 years of age, the ACE procedure using a balloon-button gastrostomy tube was performed for intractable chronic constipation. Simple management was expected, but after 17 years of age, he experienced increased stool leakage around the gastrostomy tube and his QOL declined due to difficulty in managing the ACE. Therefore, reconstruction of the ACE procedure by MM was performed. After reconstruction, the ACE performed well without any complications. The patient is currently satisfied because management of the ACE is easier than before, and his QOL has markedly improved without stool leakage and dermatitis. CONCLUSIONS: MM is less likely to cause complications and is useful as a reconstruction of the ACE procedure.

Staged laparotomies based on the damage control principle to treat hemodynamically unstable grade IV blunt hepatic injury in an eight-year-old girl.

BACKGROUND: Severe blunt hepatic injury is a major cause of morbidity and mortality in pediatric patients. Damage control (DC) surgery has been reported to be useful in severely compromised children with hepatic injury. We applied such a technique in the treatment of a case of hemodynamically unstable grade IV blunt hepatic injury in an eight-year-old girl. This case is the first to use multimodal approaches including perihepatic packing, temporary closure of the abdominal wall with a plastic sheet, transarterial embolization (TAE), and planned delayed anatomical hepatic resection in a child. CASE PRESENTATION: An eight-year-old girl was run over by a motor vehicle and transferred to the emergency department of the local hospital. Her diagnoses were severe blunt hepatic injury (grade IV) with left femoral trochanteric fracture. No other organ injuries were observed. Because her hemodynamic state was stable under aggressive fluid resuscitation, she was transferred to our hospital for surgical management. On arrival at our institution about 4 h after the injury, her hemodynamic condition became unstable. Abdominal compartment syndrome also became apparent. Because her condition had deteriorated and the lethal triad of low BT, coagulopathy, and acidosis was observed, a DC treatment strategy was selected. First, emergent laparotomy was performed for gauze-packing hemostasis to control intractable bleeding from the liver bed, and the abdomen was temporarily closed with a plastic sheet with continuous negative pressure aspiration. Transarterial embolization of the posterior branch of the right hepatic artery was then carried out immediately after the operation. The lacerated right lobe of the liver was safely resected in a stable hemodynamic condition 2 days after the initial operation. Bleeding from the liver bed ceased without further need of hemostasis. She was transferred to the local hospital without any surgical complications on day 42 after admission. She had returned to her normal life by 3 months after the injury. CONCLUSION: The DC strategy was found to be effective even in a pediatric patient with hemodynamically unstable severe blunt hepatic injury. The presence of the deadly triad (hypothermia, coagulopathy, and acidosis) and abdominal compartment syndrome was an indication for DC surgery.

The area and attachment abnormalities of the gubernaculum in patients with undescended testes in comparison with those with retractile testes.

PURPOSE: In order to evaluate the gubernaculum (GN) abnormalities quantitatively in patients with undescended testes (UDT), the area and attachment site of the gubernaculum were evaluated. PATIENTS AND METHODS: Sixty-seven testes from 61 patients with an undescended testis treated in the past 11 years at our institution were examined. Using intraoperative photographs or DVDs, the area of the GN inside the processus vaginalis was measured, and the ratio to that of the testis was determined. When the GN was attached to the vas deferens, the GN distance from the testis was also measured, and the ratio to that of the transverse length of the testis (deviation index) was calculated. Reference values were obtained from 23 testes from 15 patients with mobile testes. RESULTS: In cases with mobile testes, the GN attached to the bottom of the testis, and involved the lower pole of the epididymis. Even though the GN was attached to the bottom of the testis in 43 testes in the UDT patients (64 %), the GN was found to be elongated. The mean GN area ratio was 1.58 (1SD, 0.6) in the UDT cases, in comparison to 0.47 (0.2) in the cases with mobile testes. The GN was attached to the vas deferens in 24 testes (36 %). The deviation index was 1.34 (1.0), but the GN area ratio of these cases was 1.56 (0.7), which was similar to that of the GN attached to the bottom of the testis. CONCLUSION: The present study revealed that an increase in the GN area ratio was the most common imaging abnormality in cases with UDT.

Risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts.

BACKGROUND AND AIM: The aim of this study was to elucidate the risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts. METHODS: A retrospective analysis of 94 patients who had undergone cyst excision for congenital choledochal cysts was conducted. The median age at the time of cyst excision and median follow-up time after cyst excision were 7 years and 181 months, respectively. RESULTS: Biliary tract cancer developed in four patients at 13, 15, 23, and 32 years after cyst excision. The cumulative incidences of biliary tract cancer at 15, 20, and 25 years after cyst excision were 1.6%, 3.9%, and 11.3%, respectively. The sites of biliary tract cancer were the intrahepatic (n = 2), hilar (n = 1), and intrapancreatic (n = 1) bile ducts. Of the four patients with biliary tract cancer after cyst excision, three patients underwent surgical resection and one patient received chemo-radiotherapy. The overall cumulative survival rates after treatment in the four patients with biliary tract cancer were 50% at 2 years and 25% at 3 years, with a median survival time of 15 months. CONCLUSIONS: The risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts seems to be relatively high in the long-term. The risk of biliary malignancy in the remnant bile duct increases more than 15 years after cyst excision. Despite an aggressive treatment approach for this condition, subsequent biliary malignancy following cyst excision for congenital choledochal cysts shows an unfavorable outcome.

Tracheoplasty using a metallic fusiform centrally-doubled coiled stent for a major tracheal defect in rabbits.

BACKGROUND/PURPOSE: The long-term survival rate of rabbits treated with a fusiform metallic coil for a large tracheal wall defect is 60%. In the present study, the central half of a simple coil was covered by a double coil to prevent the ingrowth of the surrounding connective tissue and to provide a sutureless fixation of the coil to obtain a further increase in the survival rate. STUDY DESIGN: The anterior half of the tracheal wall was removed for a longitudinal length of 6 tracheal rings to make a large tracheal defect. Metallic coils were placed into the tracheal lumen through the wall defect. The doubly-coiled portion was designed to fit the length of the defect to eliminate the need for suture fixation. The survival at two months after the operation, endoscopic findings and histological changes were evaluated. RESULTS: All 6 rabbits treated with a centrally-doubled coiled stent survived without major respiratory trouble for at least 2 months. Endoscopic examinations performed 1 month after the operation revealed an irregular coating of reddish granulation tissue inside the coil, and a wet portion was aspirated. The tracheal defect was replaced with fibrous tissue, but neither a complete epithelialization nor cartilage formation was observed. CONCLUSION: These results suggest that the metallic coil was useful to obtain an improvement in the survival of rabbits with a large tracheal wall defect. Therefore, this new coil might be indicated for the treatment of tracheal wall defects.