Case report: Hepatectomy with Rex bypass for a child with hepatoblastoma and portal vein thrombosis.

Pediatric liver tumors with portal vein obstruction are often candidates for liver transplantation. However, lifelong use of immunosuppressants and invasiveness to healthy donors in the case of living-donor liver transplantation is inevitable. Moreover, when lung metastasis is involved, the lung recurrence rate after liver transplantation is still high. Therefore, transplantation should be avoided as much as possible. In cases of tumors in the right lobe of the liver, complete resection of the portal vein trunk may be possible by creating a Rex bypass, but with the original method, end-to-side anastomosis to the umbilical portal vein is difficult in small children. We report a case of a 2-year-old girl with hepatoblastoma in whom a Rex shunt was created by end-to-end anastomosing the recanalized umbilical vein to the portal vein stump with interposing a vein graft, and the right lobe was successfully resected along with the tumor.

Infantile hepatic hemangioma and hepatic mesenchymal hamartoma in an infant associated with placental mesenchymal dysplasia: a case report.

BACKGROUND: Although infantile hepatic hemangioma and hepatic mesenchymal hamartoma are relatively common in benign pediatric liver tumors, coexistence of the two tumors is rare. Placental mesenchymal dysplasia is also a rare disorder. We report the case of a baby girl born after a pregnancy complicated by placental mesenchymal dysplasia, who developed both infantile hepatic hemangioma and hepatic mesenchymal hamartoma. CASE PRESENTATION: The patient was born at 32 weeks and 5 days of gestation for impending placental abruption, weighing 1450 g. Liver tumors, composed of both hypervascular solid and large cystic lesions, were detected after birth and markedly increased to create abdominal distention within 9 months. Diagnostic imaging suspected the coexistence of infantile hepatic hemangioma and cystic hepatic mesenchymal hamartoma. Following propranolol therapy for infantile hepatic hemangioma and needle puncture of a large cyst, the cystic lesions and adjacent hypervascular lesions were partially resected via laparotomy. Pathological findings confirmed the coexistence of hepatic mesenchymal hamartoma and infantile hepatic hemangioma, which had no association with androgenetic/biparental mosaicism. The postoperative course was uneventful, and the tumor had not regrown after 3 years. CONCLUSIONS: Although the coexistence of infantile hepatic hemangioma and hepatic mesenchymal hamartoma associated with placental mesenchymal dysplasia is extremely rare, the pathological and pathogenetic similarities between these disorders suggest that they could have derived from similar embryologic origins rather than being a mere coincidence. Further follow-up is required, with careful attention to the potential for malignant hepatic mesenchymal hamartoma transformation.

Characteristics and treatment of congenital esophageal stenosis: A retrospective collaborative study from three Japanese children's hospitals.

BACKGROUND/PURPOSE: There is no consensus on treatment strategy of congenital esophageal stenosis (CES). This study aimed to assess appropriateness of the treatment we have provided to patients with CES over the past four decades. METHODS: We carried out a retrospective chart review of 83 CES patients treated at three children's hospitals between 1973 and 2015. Each patient underwent an initial treatment with either surgery or a series of dilation that was followed by surgery if dilation failed to improve esophageal transit. Demographic data, course of treatment, outcomes, and complications were analyzed. RESULTS: During this initial treatment, 19 and 64 patients underwent surgery and dilation, respectively. Out of the 64 patients who underwent dilations as an initial treatment, 26 patients eventually required surgery. Out of all patients who required surgery (19 initial treatments + 26 failed dilations), 29 had tracheobronchial remnants and 16 had fibromuscular hypertrophy. Six patients experienced esophageal perforation during dilation and ten experienced anastomotic leakage after surgery. No patients had swallowing difficulties at the latest follow up, 141(9-324) months. CONCLUSIONS: Dilation is recommended as an initial therapy, especially if histological diagnosis of CES is uncertain. Persistent swallowing difficulties after 2 series of dilation may be an indication for surgery. LEVELS OF EVIDENCE: level IV.

A novel method for isolating lymphatic endothelial cells from lymphatic malformations and detecting PIK3CA somatic mutation in these isolated cells.

PURPOSE: Tissue disaggregation and the cell sorting technique by surface markers has played an important role in isolating lymphatic endothelial cells (LECs) from lymphatic malformation (LM). However, this technique may have the drawback of impurities or result in isolation failure because it is dependent on surface marker expressions, the heterogeneity of which has been found in the lymphatic system. We developed a novel method for isolating LM-LECs without using whole tissue disaggregation. METHODS: Seven LM surgical specimens were collected from seven patients with LMs. LM-LECs were detached from the LM cyst wall by "lumen digestion" and irrigating the cystic cavity with trypsin, and maintained in culture. RESULTS: The cells formed a monolayer with a cobblestone-like appearance. Immunohistochemistry and quantitative RT-PCR of these cells revealed high expression of lymphatic-specific genes, confirming their identity as LM-LECs. The whole-exome sequencing and PIK3CA sequencing of these cells revealed somatic mutations in PIK3CA in all cases. CONCLUSIONS: We established a novel technique for isolating LM-LECs from LM tissue by "lumen digestion" without whole-tissue disaggregation. The limited incorporation of non-LM LECs in the isolate in our method could make it an important tool for investigating the heterogeneity of gene expression as well as mutations in LM-LECs.

Lymphatic malformations compromising the upper airway in children: ultrasound-guided intralesional focal sclerotherapy with bleomycin targeting culprit lesions.

PURPOSE: Lymphatic malformations (LMs) compromising the upper airway is a life-threatening and intractable disease. Here, we establish a novel method to perform intralesional focal sclerotherapy targeting the culprit for airway stenosis. METHODS: Between July 2015 and February 2020, 11 patients with airway-compromising LMs were enrolled. To yield maximal effects on the compromised airway with minimal adverse effects, ultrasound-guided intralesional bleomycin sclerotherapy assisted by balloon was performed, aimed at the most responsible lesion around the airway. A retrospective analysis was performed. RESULTS: Ten patients presented with respiratory symptoms, eight of whom required airway support. The last asymptomatic patient showed airway compression on magnetic resonance imaging. The dose of bleomycin injected ranged from 1.3-9 mg per patient per course. A median of one course was required for withdrawal from airway support, and the median time was 15 days. A median of two courses was required to eliminate the lesion adjacent to the airway, which would have potential risk of airway stenosis. No complications were observed. CONCLUSIONS: Our intralesional focal sclerotherapy technique with bleomycin targeting the culprit lesion is dose-sparing, safe, and effective in achieving rapid shrinkage of LMs compromising the upper airway in children, thereby avoiding tracheostomy.

Laparoscopic partial circular myectomy for congenital esophageal stenosis due to tracheobronchial remnant.

Congenital esophageal stenosis (CES) is a rare anomaly, and its appropriate management is not well established. This may be the first report describing laparoscopic partial circular myectomy of the esophageal wall in an infant with CES caused by tracheobronchial remnant (TBR). The narrow segment of the esophagus was laparoscopically detected 1 cm above the esophagogastric junction because the segment was whiter and narrower than the other parts of the esophagus. The narrow segment was approximately 1.5 cm in length. Partial anterior 270° circular muscle layers were dissected using hook electrocautery. Small cartilage-like tissues were identified during the procedure. The muscular layers of both ends were interruptedly sutured. Intraoperative complications were not detected. After undergoing balloon dilation for slight residual stenosis, the patient could eat solid foods without vomiting. Laparoscopic partial circular myectomy is safe and effective for short-segment lower esophageal stenosis in patients with CES caused by TBR.

Continuous transanal decompression for infants with long- and total-type Hirschsprung's diseases as a bridge to curative surgery: a single-center experience.

BACKGROUND: The purpose of this study is to assess the usefulness of continuous bowel decompression using an indwelling transanal tube (ITT) for preoperative management in infants with long-segment (L)- or total (T)-type Hirschsprung's disease (HD). CASE PRESENTATION: Between 2012 and 2015, seven patients with L- or T-type HD underwent preoperative bowel management by continuous bowel decompression using an ITT during waiting period for curative surgery. Continuous bowel decompression was done using an ITT, a 10-12F flexible dual lumen tube placed through the rectum up to the dilated colon under fluoroscopic guidance and secured to the bilateral buttocks. The ITT tips were located at least in a dilated colon or the cecum if there was no radiographic transitional zone. The ITT was left open for continuous drainage, and its patency was checked by regular suction until the curative operation. The patient status and complications of this preoperative management were reviewed retrospectively. RESULTS: The median duration of decompression management was 65 (17-137) days. During decompression period, neither abdominal distention, enterocolitis, nor other complications occurred and six patients could stay at home until the curative operation. The weight-for-age Z-score at curative surgery was the same as or higher than that at birth in five patients. ITT replacement was needed three times per patient on an average for accidental ITT removal, ITT stenosis, or ITT hardening. CONCLUSIONS: Bowel management by continuous bowel decompression using an ITT is easy, safe, and effective for preoperative management in patients with L- or T-type HD and may permit single-stage surgery rendering colostomy or enterostomy unnecessary.

Timing and outcome of stoma closure in very low birth weight infants with surgical intestinal disorders.

PURPOSE: Very low birth weight infants (VLBWIs) are at risk of surgical intestinal disorders including necrotizing enterocolitis (NEC), focal intestinal perforation (FIP), and meconium-related ileus (MRI). We conducted this study to verify whether the timing of stoma closure and that of enteral nutrition establishment after stoma closure in VLBWIs differ among the most common disorders. METHODS: A retrospective multicenter study was conducted at 11 institutes. We reviewed the timing of stoma closure and enteral nutrition establishment in VLBWIs who underwent stoma creation for intestinal disorders. RESULTS: We reviewed the medical records of 73 infants: 21 with NEC, 24 with FIP, and 25 with MRI. The postnatal age at stoma closure was 107 (28-359) days for NEC, 97 (25-302) days for FIP, and 101 (15-264) days for MRI (p = 0.793), and the postnatal age at establishment of enteral nutrition was 129 (42-381) days for NEC, 117 (41-325) days for FIP, and 128 (25-308) days for MRI (p = 0.855). The body weights at stoma closure were 1768 (620-3869) g for NEC, 1669 (1100-3040) g for FIP, and 1632 (940-3776) g (p = 0.614) for MRI. There were no significant differences among the three groups. CONCLUSIONS: The present study revealed that the time and body weights at stoma closure and the postoperative restoration of bowel function in VLBWIs did not differ among the three diseases.

Usefulness of a recanalized umbilical vein for vascular reconstruction in pediatric hepatic surgery.

PURPOSE: Pediatric surgeons currently engage in various abdominal vascular surgeries, which sometimes require vascular conduits or grafts. Herein, we report our experience with patients undergoing vascular reconstruction using a recanalized umbilical vein (rUV) and their long-term outcome. METHOD: Five patients with extrahepatic portal vein obstruction (EHPVO) underwent mesenterico-/porto-left portal vein (PV) bypass surgery using a short rUV conduit with an interposition vein graft. A sixth neonate with a huge hepatic tumor underwent PV reconstruction with anastomosis of rUV to the proximal PV stump following right hepatectomy with partial PV resection. A seventh patient underwent living donor liver transplantation for recurrent hepatoblastoma. The hepatic inferior vena cava (IVC) was resected because of tumor involvement and reconstructed by transposition of the infrahepatic IVC and interposition of rUV obtained from the donor liver graft. RESULTS: Sufficient flow through rUV was achieved and maintained in all patients without any complications during follow-up (0.7-6.9 years). Esophageal varices, splenomegaly, and other laboratory test abnormalities because of portal hypertension disappeared after surgery in patients with EHPVO. CONCLUSION: Our experience confirmed the usefulness and long-term patency of rUV as an entry to the intrahepatic PV and as a free vascular graft to reconstruct PV or IVC.

Endoscopic repair of laryngotracheoesophageal clefts.

BACKGROUND/PURPOSE: In Japan, surgical repair of a laryngotracheoesophageal cleft (LTEC) typically consists of the anterior approach, with the lateral approach as an alternative. Endoscopic surgery to repair the tracheoesophageal septum has been reported, and this study reviewed our experience treating several cases of LTEC endoscopically. METHODS: Endoscopic repair of LTEC was performed in 7 patients (3 boys, 4 girls; age range 4 months to 2 years 10 months; mean age 11 months; mean weight at surgery 7.23 kg; weight range 3.85-12.24 kg) between 2009 and 2014. LTEC was type I in 5 patients and types II and IV in 1 patient each. The patient with type IV was first operated on by the lateral approach, and the remaining cleft, which level was type III, was repaired endoscopically. Postoperative outcomes were retrospectively studied. RESULTS: Endoscopic surgery was successful in all patients. All 6 patients with types I and II LTEC were extubated easily, while in the patient with type IV LTEC, it was difficult to remove the tracheostomy cannula because of tracheomalacia. Postoperatively, tracheostomy cannulation became more stable, and the patient is gradually being weaned off the ventilator. All patients could be fed orally without difficulty postoperatively. CONCLUSIONS: Endoscopic surgery provides a view from the cephalic aspect permitting the surgeon to form a normal larynx with only minimal risk of complications.

Impact of an external lengthening procedure on the outcome of long-gap esophageal atresia at our hospitals.

PURPOSE: To demonstrate the outcome of external lengthening for long-gap esophageal atresia (LGEA) at our hospitals. METHODS: Five patients with LGEA underwent external lengthening between 2010 and 2014 (group A), and 11 patients with LGEA underwent other lengthening techniques between 1990 and 2011 (group B). We compared the procedure and outcome between these two groups. RESULTS: The mean birth weight was 2001 g in group A and 2485 g in group B (p = 0.06). The mean age at esophageal reconstruction was 28 days in group A and 227 days in group B (p = 0.03). Although primary esophageal anastomosis without myotomy was feasible in all patients in group A, a myotomy was needed for primary esophageal anastomosis in half of the patients in group B. Anastomotic leakage occurred in none in group A and in six patients in group B (p = 0.03). The mean age at the establishment of full oral feeding was 76 days in group A and 686 days in group B (p = 0.009). CONCLUSION: External traction for LGEA can effectively lengthen the esophagus to enable primary anastomosis at an earlier age. This may facilitate oral intake.

Navigation using indocyanine green fluorescence imaging for hepatoblastoma pulmonary metastases surgery.

To achieve precise and sensitive detection of chemotherapy-resistant hepatoblastoma pulmonary metastases, we performed surgery using indocyanine green (ICG) fluorescence imaging navigation. Lung metastasectomies were performed in 10 patients aged from 1 to 11 years. ICG (0.5 mg/kg) was injected intravenously 24 h before the operation. After a thoracotomy had been performed, a 760-nm infrared ray was applied to the lung using a generator and the 830-nm evoked fluorescence was collected and visualized on a real-time display. In total, 250 fluorescence-positive lesions were extirpated in 37 operations. All of the pathologically positive lesions were clearly fluorescence positive. The diameter of the smallest detectable lesion was 0.062 mm. In two patients, there were 29 extirpated lesions that were pathologically proven not to be hepatoblastoma metastases. Although a problem of false positive remains, this method is very useful for the detection of small pulmonary metastases.

Type IV laryngotracheoesophageal cleft repair by a new combination of lateral thoraco-cervical and laryngoscopic approaches.

Type IV laryngotracheoesophageal cleft (LTEC) is a rare congenital anomaly that is associated with high morbidity and mortality despite various forms of surgical repair. This article presents our strategy for surgical management of type IV LTECs using a combination of lateral thoraco-cervical and laryngoscopic approaches.

Expression of alpha smooth muscle actin in living donor liver transplant recipients.

Recently, there have been reports from liver biopsies that showed the progression of liver fibrosis in liver transplant patients after the cessation of immunosuppression. Herein, we focused on activated hepatic stellate cells expressing alpha smooth muscle actin (α-SMA) to understand the correlation between immunosuppressant medication and liver fibrosis. The study enrolled two pediatric patients who underwent living donor liver transplantation and ceased immunosuppressant therapy. The number of α-SMA-positive cells in the specimens obtained by liver biopsy from these two patients showed a three-fold increase compared with the number from four transplanted pediatric patients who were continuing immunosuppressant therapy. In addition, the α-SMA-positive area evaluated using the WinRooF image processing software program continued to increase over time in three adult transplanted patients with liver fibrosis, and the α-SMA-positive area was increasing even during the pre-fibrotic stage in these adult cases, according to a retrospective review. Therefore, α-SMA could be a useful marker for the detection of early stage fibrosis.

Pancreatic head resection preserving the main pancreatic duct for congenital hyperinsulinism of infancy.

Surgical intervention for congenital hyperinsulinism with an unclear focal lesion in the pancreatic head sometimes require the resection of most of the pancreas head and pancreaticojejunotomy. This report presents the case of a patient that underwent pancreatic head resection preserving the main pancreatic duct to avoid pancreaticojejunostomy.

A modified Foker's technique for long gap esophageal atresia.

External traction using the Foker's technique enables elongation in the esophageal segments within days, and allows the primary repair of the long gap. This article presents our modified Foker's technique which was easily applicable for long-gap esophageal atresia.

Yolk sac tumor of the vulva: a case report with recurrence after long-term follow-up.

A yolk sac tumor (YST) of the vulva is extremely rare and highly malignant with recurrence frequently occurring within a year. This report presents the 13th known case of vulvar YST, with recurrence occurring after the longest known follow-up period so far reported in the literature.

The usefulness of a high-speed 3D-image analysis system in pediatric living donor liver transplantation.

BACKGROUND: Since March 2010, we have used a high-speed 3D-image analysis system (SYNAPSE VINCENT) to calculate the graft volume in living donor liver transplantation (LDLT) to replace CT volumetry. The SYNAPSE VINCENT is capable of extracting each vessel territory in the liver and displaying 3D images simply, quickly, and accurately. Therefore, we report here the usefulness of the SYNAPSE VINCENT in pediatric LDLTs in overcoming issues with perfusion area of hepatic venous tributaries in monosegmental grafts. MATERIAL/METHODS: The SYNAPSE VINCENT was used in three pediatric patients. In two of these cases, the possibility of monosegmental grafts was assessed when calculating graft volumetry of segment III. RESULTS: The graft recipient weight ratio (GRWR) with graft volumetry measurements of the left lateral segment were 1.8-5.6%. GRWR of segment III were 2.3 and 2.0%. Since donor V2, venous branch to segment II and V3, venous branch to segment III were independently branching in one case, the monosegmental graft could be evaluated preoperatively according to the venous perfusion. CONCLUSIONS: Graft volumetry using the SYNAPSE VINCENT was useful for planning the LDLT operative procedures, especially in infants possibly in need of monosegmental graft.

A gallstone in the Roux-en Y limb during pregnancy in a female patient with biliary atresia.

A 28-year-old female developed liver dysfunction at the 13th gestation week of her second pregnancy. A postpartum radiological examination revealed a stone in the Roux-en Y (RY) limb. She underwent surgical removal of the gallstone in the RY limb. Bile congestion during pregnancy led to the development of a gallstone.

Partial internal biliary diversion for patients with progressive familial intrahepatic cholestasis type 1.

We herein report a case of progressive familial intrahepatic cholestasis with partial internal biliary diversion (PIBD). Although by using PIBD an external stoma can be avoided, exposure of the ileocecal junction to bile reflux as well as the effects of the direct bile flow on the colonic mucosa require further investigation.