Donor-dominant one-way matching of human leukocyte antigen-A/B/DR alleles predicts graft-versus-host disease following living donor liver transplantation.

AIM: Graft-versus-host disease (GVHD) following liver transplantation is rare but fatal. Therefore, it is important to identify possible risk factors before transplantation. Although it has been suggested that donor-dominant one-way human leukocyte antigen (HLA) matching of three loci (HLA-A/B/DR) is associated with the occurrence of GVHD, the precise significance of HLA matching including HLA-C/DQ/DP remains unclear. METHODS: We retrospectively analyzed the impact of donor-dominant one-way HLA matching at six HLA loci at the allele level on GVHD using clinical registry data from 1759 cases who underwent living donor liver transplantation between June 1990 and June 2019. We extracted cases with donor-dominant one-way HLA matching at the antigen level and reconfirmed them at the allele level using preserved DNA samples. RESULTS: Three of four cases (75%) who developed GVHD showed donor-dominant one-way HLA matching at three HLA-A/B/DR loci. These cases also showed donor-dominant one-way HLA matching at HLA-C/DQ/DP. Three of six cases (50%) with donor-dominant one-way HLA matching at three loci of HLA-A/B/DR developed GVHD. Notably, none of the cases with donor-dominant one-way HLA matching at one or two HLA-A/B/DR loci developed GVHD, irrespective of matching status at HLA-C/DQ/DP. The HLA matching status at the antigen level was revised in 22 of 56 cases, following reconfirmation at the allele level. CONCLUSIONS: Pairing of donors and recipients with donor-dominant one-way HLA matching at three HLA-A/B/DR loci should be avoided to prevent GVHD. No impact of HLA-C/DQ/DP on GVHD was identified. For liver transplantation, HLA genotypes should be determined at the allele level.

A Comparison of Laparoscopic Redo Fundoplications for Failed Toupet and Nissen Fundoplications in Children.

PURPOSE: We compared laparoscopic redo fundoplications performed for failed laparoscopic Toupet fundoplication (LTF) and failed laparoscopic Nissen fundoplications (LNFs). METHODS: Redo LTF (R-LTF; n = 4) and redo LNF (R-LNF; n = 6) performed between 2007 and 2014 were assessed retrospectively for severity of intraperitoneal adhesions on a scale of 0-3, identification/preservation of the anterior/posterior/hepatic branches of the vagus nerve (VN), complications, and outcome. RESULTS: Redos were performed after a mean of 34 months in R-LTF and 32 months in R-LNF (P = ns) indicated for sliding hernia (n = 3; 2 with partial wrap dehiscence) and partial wrap dehiscence (n = 1) in R-LTF and sliding hernia (n = 6; 4 with partial wrap dehiscence) in R-LNF. The mean adhesion severity score was 1.5 in R-LTF and 2.5 in R-LNF (P < 0.05). The mean number of VN branches identified/preserved was 2.0 in R-LTF and 0.8 in R-LNF (P < 0.05). Mean operative times and mean blood loss were similar. Intraoperative complications were accidental local trauma (n = 1 in R-LTF and n = 3 in R-LNF, one requiring conversion to open repair) (P = ns). Gastric outlet obstruction developed in two R-LNF cases; both were managed conservatively. There have been no further recurrences to date. CONCLUSION: Although our series is small, adhesions were less, and identification/preservation of VN was easier during R-LTF.

Low Titers of Antidonor ABO Antibodies After ABO-Incompatible Living Donor Liver Transplantation: A Long-Term Follow-Up Study.

BACKGROUND: The ABO blood-type barrier in kidney and liver transplantation has been overcome by aggressive treatments such as B cell depletion using rituximab. However, the long-term effects of ABO-incompatible liver transplantation (ABO-I LTx) on immunological status have not previously been studied. Here, we assessed whether long-term immune hyporesponsiveness against ABO blood-group antigens was retained. METHODS: We recruited 81 patients, 75 patients who had survived ABO-I LTx without retransplantation and 6 patients who had survived after retransplantation using blood type-compatible grafts. The time between ABO-I LTx and outpatient visits for blood sampling for this study ranged from 1.1 to 16.8 years. We also evaluated patients' backgrounds and postoperative therapies. RESULTS: Overall, antidonor ABO antibody titers in the 75 patients without retransplantation decreased during long-term follow-up. In the subset of 40 patients with blood type O, anti-nondonor ABO antibody titers did not decrease and were significantly higher than antidonor ABO antibody titers. In addition, long-term antidonor ABO antibody titers were significantly lower in pediatric patients than in adult patients. In the 6 patients who were retransplanted with blood type-compatible grafts, antidonor ABO antibody immunoglobulin G titers remained low, but IgM titers increased slightly long after removal of the ABO-incompatible graft. CONCLUSIONS: These findings suggest that donor-specific hyporesponsiveness remains after ABO-I LTx, particularly in pediatric patients. Long-term persistence of blood antigens may contribute to this donor-specific hyporesponsiveness.

Comparison of laparoscopic Toupet and laparoscopic Nissen fundoplications in neurologically normal children.

INTRODUCTION: We compared laparoscopic Toupet fundoplication (LTF) and laparoscopic Nissen fundoplication (LNF) in neurologically normal children. METHODS: Forty neurologically normal children who were followed up for more than 3 years after LTF (n = 22) or LNF (n = 18) were reviewed retrospectively. LTF and LNF were performed between 2006 and 2012. RESULTS: There were no significant differences in gender (LTF, 15 male and 7 female patients; LNF:, 12 male and 6 female patients), mean age at surgery (LTF vs LNF: 2.5 vs 2.3 years), mean weight at surgery (LTF vs LNF: 9.6 vs 8.9 kg), preoperative symptoms, preoperative pH monitoring (pH <4) (LTF vs LNF: 26.7% vs 21.8%), mean operative time (LTF vs LNF: 117 vs 126 min), postoperative recommencement of enteral feeding (LTF vs LNF: 3.7 vs 3.8 days), or duration of hospitalization (LTF vs LNF: 5.5 vs 6.3 days). Intraoperative complications were esophageal trauma (LTF; n = 1; 4.5%) and liver trauma (LNF; n = 1; 5.6%) (P = 0.70). Post-LTF complications were wrap stenosis (n = 1; 4.5%), and post-LNF complications were wrap stenosis (n = 1; 5.5%) and gastric outlet obstruction (n = 1; 5.5%) (P = 0.43); all were managed conservatively. No case required conversion to open repair. There was no recurrence after LTF, but there were three cases (16.7%) after LNF (P = 0.08). Reoperation was performed at 4, 11, and 13 months, respectively. CONCLUSION: Despite LTF and LNF appearing to be equally effective, three LNF cases required reoperation.

Comparison of laparoscopic hepaticojejunostomy and open hepaticojejunostomy. Can stenosis of the hilar hepatic duct affect postoperative outcome?

INTRODUCTION: The aim of this study was to compare laparoscopic hepaticojejunostomy (LHJ) and open hepaticojejunostomy (OHJ) for choledochal cyst associated with hilar hepatic duct stenosis (HHDS). METHODS: Data collection was prospective for LHJ cases from 2009 and retrospective for OHJ cases from 2003 to 2008. Data were compared with respect to HHDS. HHDS was incised longitudinally as required during hilar hepatic ductoplasty. RESULTS: Fifty-eight subjects were studied (LHJ: n = 27, 4 boys, 23 girls; OHJ: n = 31; 6 boys, 25 girls). HHDS was present in 10 LHJ cases and 10 OHJ cases. Todani classification of choledochal cyst in LHJ patients was type 1A (n = 16) and type 4A (n = 11), and in OHJ patients, it was type 1A (n = 19) and type 4A (n = 12). There were significant differences between LHJ and OHJ with regard to mean operative time (386 vs 341 min), mean blood loss (5.9 vs 18.4 mL), recommencement of enteral feeding (3.9 vs 6.4 days), and hospital stay (11.7 vs 15.5 days) (all P < 0.05). Hepatic ductoplasty was performed in 23 LHJ patients and in 21 OHJ patients. There were no intraoperative complications and no conversions to OHJ. There were no significant differences between LHJ and OHJ, regardless of the presence of HHDS, for postoperative complications, which included minor bile leakage, anastomotic stricture, and intestinal obstruction. CONCLUSION: LHJ is as effective as OHJ for the treatment of choledochal cysts irrespective of the presence of HHDS and the need for hepatic ductoplasty.

Surgical Management of Hiatal Hernia in Children with Asplenia Syndrome.

Purpose Patients with asplenia syndrome (AS) are likely to have upper gastrointestinal tract malformations such as hiatal hernia. This report discusses the treatment of such conditions. Methods Seventy-five patients with AS underwent initial palliation in our institution between 1997 and 2013. Of these, 10 patients had hiatal hernia. Of the patients with hiatal hernia, 6 had brachyesophagus and 7 had microgastria. Results Of the 10 patients with hiatal hernia, 9 underwent surgery in infancy (7 before Glenn operation, 2 after Glenn operation). Two underwent typical Toupet fundoplication, and the other 7 underwent atypical repair including reduction of the stomach. Two patients with atypical repair showed recurrence of hernia and required reoperation. Three patients required reoperation due to duodenal obstruction. Duodenal obstruction occurred due to preduodenal portal vein or abnormal vessels compressing the duodenum. Obstructive symptoms were not seen in any cases preoperatively. Conclusions In patients with hiatal hernia, typical fundoplication is often difficult because most have concomitant brachyesophagus, microgastria, and hypoplasia of the esophageal hiatus. However, we should at least reduce the stomach to the abdominal cavity as early as possible to increase thoracic cavity volume and allow good feeding. Increasing the volume of the thoracic cavity thus makes Glenn and Fontan circulations more stable. Duodenal obstruction secondary to vascular anomalies is also common, so the anatomy in the area near the duodenum should be evaluated pre- and intraoperatively.

A Case of Juxtaglomerular Cell Tumor, or Reninoma, of the Kidney Treated by Retroperitoneoscopy-Assisted Nephron-Sparing Partial Nephrectomy Through a Small Pararectal Incision.

A 15-year-old girl was found to be hypertensive (230-270/140-170 mm Hg) without any subjective symptoms. Magnetic resonance imaging confirmed the presence of a well-defined 22 mm hypodense lesion in the lower pole of the left kidney, located close to the renal hilum. Plasma rennin activity was elevated (75 ng/mL/h), and reninoma was diagnosed. Retroperitoneoscopy-assisted nephron-sparing surgery was planned. The retroperitoneum was accessed through a 4 cm left pararectal upper abdominal incision. Following blunt dissection, the abdominal wall was elevated with a lifting bar and lifting retractor, inserted below the 12th rib in the anterior axillary line to create sufficient working space in the retroperitoneal cavity without the need for pneumoperitoneum. Three 5 mm trocars were introduced above the superior iliac crest for the camera and the assistant. Gerota's fascia was opened and the kidney exposed. The surgeon dissected the left kidney through the minilaparotomy incision under both direct vision and using the magnified view on the monitor, which was particularly effective for the lateral and posterior sides of the kidney. The posterior peritoneum was incised intentionally next to the diaphragm to allow further mobilization of the kidney. Diathermy was used to remove the tumor and a layer of surrounding normal parenchymal tissue at least 0.5 cm thick. The histopathologic diagnosis was reninoma. Ischemia time was 14 minutes. Postoperatively, both plasma rennin activity and blood pressure were normal (1.9 ng/mL/h and 90-110/70-80 mm Hg, respectively). After follow-up of 12 months, there is no evidence of recurrence.

Laparoscopic Heller Myotomy for Non-Dilated Esophageal Achalasia in Children with Intraoperative Stepped Dilation Under Image Guidance: Attempting Complete Myotomy.

This study presents a modified surgical approach to laparoscopic myotomy for achalasia using stepped dilation with a Rigiflex balloon and contrast medium under image guidance. A 10-year-old boy with persistent dysphagia and vomiting had ingested only liquids for 3 months, losing >10 kg in body weight. Barium swallow and esophageal manometry diagnosed esophageal achalasia with mild esophageal dilatation. After failed pneumatic dilatation, laparoscopic Heller myotomy with Dor fundoplication was performed. Prior to surgery, a Rigiflex balloon dilator was placed within the esophagus near the diaphragmatic hiatus. A four-port technique was used, and mobilization of the esophagus was limited to the anterior aspect. A 5-cm Heller myotomy was performed, extending another 2 cm onto the anterior gastric wall. During myotomy, the Rigiflex balloon was serially dilated from 30 to 50 mL, and filled with contrast medium under fluoroscopic image guidance in order to maintain appropriate tension on the esophagus to facilitate myotomy, and to confirm adequate myotomy with sufficient release of lower esophageal sphincter by resecting residual circular muscle fibers. Residual circular muscle fibers can be simultaneously visualized under both fluoroscopic image guidance and direct observation through the laparoscope, and they were cut precisely until the residual notch fully disappeared. Dor fundoplication was completed. The operative time was 180 minutes, and oral intake was started after esophagography on postoperative day 1. As of the 12-month follow-up, the patient has not shown any symptoms, and his postoperative course appeared satisfactory.

Comparison of percutaneous extraperitoneal closure (LPEC) and open repair for pediatric inguinal hernia: experience of a single institution with over 1000 cases.

BACKGROUND: Recently, laparoscopic percutaneous extraperitoneal closure (LPEC) for pediatric inguinal hernia has become more popular. The aim of this study was to compare LPEC with open repair (OR) performed in one institution. METHODS: In total, 1050 patients underwent OR from July 2003 to June 2008, and 1017 patients underwent LPEC from July 2008 to June 2013. The mean follow-up period was 100 months in OR and 40 months in LPEC (p < 0.01). Given the difference in the follow-up periods, the log-rank test was used for the analysis of the long-term results. The mean age at operation in OR and LPEC was 3.72 and 3.75 years, respectively (p = 0.81). The mean body weight was 14.73 and 14.72 kg, respectively (p = 0.98). The male/female ratio was 617/433 and 561/456, respectively (p = 0.10). In the LPEC procedure, the asymptomatic contralateral internal ring was routinely observed, and when a patent processus vaginalis (PPV) was confirmed, prophylactic surgery was performed. RESULTS: The mean operative time for unilateral surgery in OR and LPEC was 28.5 and 21.2 min, respectively (p < 0.01). The mean operative time for bilateral surgery was 52.3 and 25.4 min, respectively (p < 0.01). Recurrence was confirmed in 0.52% in OR and in 0.27% in LPEC (p = 0.53). In the LPEC group, 41.7% of patients with clinically unilateral inguinal hernia were confirmed to have a contralateral PPV and underwent prophylactic LPEC. Contralateral metachronous inguinal hernia (CMIH) was seen in 6.48% in OR and in 0.33% in LPEC (p < 0.01). Two patients showed postoperative testicular atrophy, and two had iatrogenic postoperative cryptorchism after OR, while no postoperative testicular complications were seen after LPEC. CONCLUSION: Both OR and LPEC obtained satisfactory results from the perspective of recurrence rate and complications. Prophylactic contralateral LPEC is useful for preventing CMIH without prolonging operative time compared with OR. The midterm safety and efficacy of LPEC are proven.

Laparoscopic suture repair of idiopathic gastric perforation in Duchenne muscular dystrophy.

We report herein an adolescent case of Duchenne muscular dystrophy (DMD) with idiopathic gastric perforation, in which emergency surgical repair was performed laparoscopically. A 14-year-old nonambulatory boy with DMD was brought to our emergency department with sudden onset of severe abdominal pain and distention. Plain radiograph and computed tomography confirmed the presence of free intraperitoneal air and intrapelvic effusion. The patient elected to undergo laparoscopic inspection with 4 trocars, revealing a focal perforation, 3-4 cm in diameter, on the upper gastric body near the diaphragm. The stomach was also found to have a thin wall without evidence of peptic ulcer disease or other abnormalities. An interrupted suture was placed using 4-0 PDS. The abdomen was extensively irrigated, and multiple J-Vac drains were left in situ. Total operation time was 90 min, and no intraoperative complications were encountered. Enteral feeding through a nasogastric tube was started on postoperative day 7. The postoperative course has been uneventful as of the 12-month follow-up. Pediatric surgeons should be aware of the increased risk of gastric perforation associated with DMD, and that laparoscopic repair can be safely performed even in emergency settings.

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.

Laparoscopic Toupet fundoplication for gastroesophageal reflux: a series of 131 neurologically impaired pediatric cases at a single children's hospital.

PURPOSE: To present the medium to long-term outcome of the largest pediatric series of laparoscopic Toupet fundoplications (LTF) performed at a single institution. PATIENTS AND METHODS: Subjects were 131 neurologically impaired children (81 M, 50 F) who underwent LTF between 2003 and 2013. Our LTF involves full dissection of the crus of the diaphragm to allow the intraabdominal esophagus to be mobilized at least 3-4 cm. RESULTS: Preoperative mean fraction time for pH <4 was 14.6 %. Mean age at LTF was 6.7 years (3 months-18 years). Mean duration of follow-up was 5.7 years (range 1.2-12.1 years). One case required conversion to open surgery. Intra-operative complications were all injuries to the esophagus/gastric wall (n = 4; 3.0 %) including full-thickness perforation (n = 1; 0.8 %). Postoperative complications included pyloric stenosis (n = 4; 3.0 %), dysphagia (n = 1; 0.8 %), incisional hernia (n = 1; 0.8 %), hemorrhage requiring transfusion (n = 1; 0.8 %), recurrence (n = 3; 2.3 % at 11, 13, and 48 months, respectively), and gastrostomy site infection (n = 7; 5.3 %). Mean operative time decreased significantly with experience from 180.8 min for the first quarter of subjects to 150.6 (2nd quarter), 128.6 (3rd) and 109.2 min (4th). CONCLUSIONS: Our LTF would appear to be safe for treating GERD in children because of reliable outcome and low recurrence.

Laparoscopic Toupet Fundoplication using an Air Seal Intelligent Flow System and Anchor Port in a 1.8-kg infant: A Technical Report.

INTRODUCTION: We report a case of a 1.8-kg infant who had laparoscopic Toupet fundoplication (LTF) using the AirSeal Intelligent Flow System and Anchor Port (AP). MATERIALS AND SURGICAL TECHNIQUE: Our case had severe gastroesophageal reflux in association with genetic and cardiac anomalies. Despite the patient being continuously fed, persistent vomiting caused failure to thrive, and LTF was performed at 4 months of age when he weighed 1.8 kg. The AirSeal Intelligent Flow System is a novel laparoscopic CO2 insufflation system that improves the visual field by constantly evacuating smoke and providing a more stable pneumoperitoneum. The AP is a recently developed, stretchable, elastomeric, low-profile cannula. Three 5-mm AP were inserted: one subumbilically for the scope and one in both the right and left upper abdomen for the surgeon. A 5-mm AirSeal trocar was inserted in the left lower abdomen for the assistant. The gastrosplenic ligament was dissected free, and the intra-abdominal esophagus was prepared. A posterior hiatoplasty was performed, followed by the 270° fundoplication. During the fundoplication, the esophagus was fixed to the crus and then the right and left wraps were fixed to the esophagus. Pneumoperitoneum was maintained stably throughout the LTF procedure, with optimal operative field. Total operating time for LTF was 90 min. Body temperature dropped from 37.4°C to 35.7°C during pneumoperitoneum but resolved once pneumoperitoneum was ceased. Postoperative progress was uneventful, and an upper gastrointestinal study on postoperative day 2 showed no residual gastroesophageal reflux. DISCUSSION: We believe the AirSeal Intelligent Flow System and AP contributed to the successful completion of LTF in a 1.8-kg infant.

Hepatic ductoplasty and hepaticojejunostomy to treat narrow common hepatic duct during laparoscopic surgery for choledochal cyst.

We describe a novel technique for laparoscopic hepatic ductoplasty and hepaticojejunostomy in choledochal cyst with a narrow common hepatic duct. After complete cyst excision, a longitudinal incision was made in the anterior wall of the narrow common hepatic duct to enlarge the anastomotic opening. Next, the anterior wall was everted, then anchored to the hepatic hilum by suturing. Anchoring of the anterior wall provided a good field of view for creating the anastomosis, and the lumen of the anastomotic region was also secured. We believe that our technique is feasible and useful when the duct size is small.

Diaphragmatic eventration in children: laparoscopy versus thoracoscopic plication.

AIM: To determine the best way to perform diaphragmatic plication for diaphragmatic eventration (DE) using minimally invasive surgery. MATERIALS AND METHODS: We conducted a retrospective review of pediatric cases of DE treated between 2007 and 2012. Thoracoscopic plication (TP) is performed using single-lung ventilation with three 5-mm ports; laparoscopic plication (LP) is performed using three or four 5-mm ports. The choice of technique was determined preferentially by the treating surgeon. RESULTS: There were 20 subjects (13 treated by LP and 7 treated by TP). Etiology of DE was phrenic nerve injury (LP, n=11; TP, n=1) and muscular deficiency (LP, n=2; TP, n=6). Mean age (LP, 18 months; TP, 25 months) and weight (LP, 8.0 kg; TP, 9.7 kg) at surgery were not significantly different. Mean operating time was 155.6 minutes in LP and 167.0 minutes in TP (P=not significant). Mean intraoperative end-tidal CO2 was 41.9 mm Hg (range, 35-52 mm Hg) in LP and 36.9 mm Hg (range, 33-41 mm Hg) in TP (P=.01). Mean duration of postoperative ventilation was 1.2 days in LP and 1.3 days in TP (P=not significant). Mean time taken to recommence feeding postoperatively was 1.6 days in both groups (P=not significant). Complications were one conversion to thoracotomy in TP, 1 case of atelectasis in each group (P=not significant), and 6 cases of recurrence in LP versus none in TP (P=.04). CONCLUSIONS: Both TP and LP are beneficial for treating small children with DE. However, there is a higher incidence of recurrence after LP, and the role of TP in cardiac patients requiring subsequent surgery is debatable.

Usefulness of laparoscopic cholecystostomy in children with complicated choledochal cyst.

INTRODUCTION: In children with choledochal cysts (CC), obstruction of the discharge of bile or pancreatic juice is frequently observed, and biliary perforation and severe pancreatitis may occur. In such cases, temporary drainage is necessary to stabilize these patients. We employed laparoscopic cholecystostomy followed by laparoscopic cyst excision in cases with complicated CC. METHODS: Between 2009 and 2013, emergency laparoscopic cholecystostomy for bile drainage was performed in seven girls with complicated CC in whom conservative treatment failed to ameliorate abdominal pain, vomiting, and jaundice; among these seven patients included two with biliary perforation, one with a huge cyst, and four with peribiliary edema suggestive of impending biliary rupture. Protein plugs in the common channel or the terminal portion of the cyst were present in six patients. RESULTS: Amelioration of symptoms, such as abdominal pain and jaundice, was achieved in all patients after laparoscopic cholecystostomy. Saline irrigation of the bile duct via cholecystostomy was commenced 2 days postoperatively. All patients underwent laparoscopic cyst resection after improvement of their general condition and detailed evaluation of the intrahepatic and extrahepatic bile ducts and pancreaticobiliary maljunction. Impacted protein plugs or gallstones were eliminated in all patients by the time of laparoscopic cyst excision. CONCLUSION: Laparoscopic cholecystostomy followed by laparoscopic cyst excision is a useful and safe procedure for the treatment of complicated CC. This technique is also favorable from a cosmetic viewpoint because the resultant wound can be reused as the trocar insertion site at the time of laparoscopic cyst excision.

Unilateral pulmonary agenesis associated with oesophageal atresia and tracheoesophageal fistula: A case report with prenatal diagnosis.

We describe herein a case of unilateral pulmonary agenesis (PA) with oesophageal atresia (EA)/tracheoesophageal fistula (TEF) that was diagnosed prenatally and repaired by esophagoesophagostomy with stable postoperative course. The patient was born at 34 weeks gestation, after ultrasonography at 22 weeks gestation showed possible right-sided diaphragmatic eventration or PA and EA was subsequently suspected due to hydramnios. The initial X-ray showed mediastinal shift to the right, and coil up sign of the nasogastric tube, without intracardiac anomaly. Immediately after the diagnosis of EA/TEF and unilateral PA on day 0, the patient was intubated in the operating room, and a gastrostomy tube was placed. After pulmonary status stabilized, at 4 days old, EA/TEF was repaired through a thoracotomy in the right 4 th intercostal space. The right main bronchus was noted to continue into the distal oesophagus; this fistula was ligated and divided, and a single-layer esophagoesophagostomy was performed under mild tension with one vertebral gap. The neonate was maintained on mechanical ventilation and gradually weaned to extubation at 7 days old. The postoperative course was uneventful, with the exception of prolonged jaundice that emerged at 3 months old. Laparoscopic cholangiography at that time excluded biliary atresia, and jaundice resolved spontaneously. The patient has not shown any respiratory symptoms or feeding difficulties as of the 12-month follow-up.

Laparoscopic repair of malrotation: what are the indications in neonates and children?

AIM: To define the role of laparoscopy for treating malrotation in children. MATERIALS AND METHODS: The Ladd procedure (9 laparoscopic [lap-Ladd], 17 open [open-Ladd]; n=26) was performed in children up to and including 30 days of age (neonatal [Group N]) and older (Group C). These groups were compared retrospectively. RESULTS: Group N (n=14) comprised 3 lap-Ladd and 11 open-Ladd patients. Group C (n=12) comprised 6 lap-Ladd and 6 open-Ladd patients. No case had ischemic bowel preoperatively. Intestinal volvulus was confirmed in 3 of 3 lap-Ladd and 9 of 11 open-Ladd patients in Group N, compared with 5 of 6 lap-Ladd and 6 of 6 open-Ladd patients in Group C (P=not significant). Mean operating times were significantly longer for lap-Ladd patients (130.7 minutes versus 81.1 minutes in Group N and 119.2 minutes versus 74.2 minutes in Group C). Conversion to an open-Ladd procedure was necessary in 1 of 3 patients in Group N and 1 of 6 patients in Group C (P=not significant). Complications arose in open-Ladd patients, bowel obstruction in Group N (1 of 11), and mesenteric chylorrhea in Group C (1 of 6). There was recurrence in 1 of 3 lap-Ladd patients in Group N. Mean time to recommence feeding was earlier for lap-Ladd patients (P=not significant). Length of hospitalization was similar in Group N but was shorter for lap-Ladd patients in Group C (P=not significant). CONCLUSIONS: Although lap-Ladd appears to be a safe procedure, it cannot be recommended for the treatment of malrotation in neonates.

Thoracoscopic versus open repair of esophageal atresia with tracheoesophageal fistula at a single institution.

PURPOSE: The potential benefits of thoracoscopic repair (TR) of esophageal atresia and tracheoesophageal fistula (EA/TEF) in newborns are still unclear. Our aim was to define the criteria, perioperative outcome after undergoing TR versus open repair (OR) for EA/TEF. PATIENTS AND METHODS: A retrospective chart review was conducted of 36 consecutive neonates who underwent EA/TEF repair between 2001 and 2012 in Shizuoka Children's Hospital. Patients in this study were birth weight >2,000 g, and did not have severe cardiac malformations or chromosomal aberrations. Of the 26 newborns who met the selection criteria, 11 patients underwent attempts at TR compared to 15 patients who underwent OR. All cases were followed 1 year after operation at least. RESULTS: All 11 TR were successfully completed. There were no significant differences between intra- and perioperative complications in the two groups. Intraoperative EtCO2 and arterial blood gases were not significantly different between the two groups. We did not found eating disorder, respiratory disorder, and failure of growth in all cases. CONCLUSION: In our study, the thoracoscopic approach appeared to be favorable and safe for EA/TEF repair in carefully selected patients.

Solid pseudopapillary tumor of the pancreas in children: surgical intervention strategies based on pathological findings.

PURPOSE: The appropriate surgical intervention strategies for solid pseudopapillary tumor (SPT) of the pancreas in children are still controversial. The aim of this study was to establish surgical intervention strategies based on the pathological findings. METHODS: We retrospectively reviewed the medical records of patients who underwent surgery for SPT between January 1994 and April 2013. RESULTS: Five patients with SPT were identified. Tumors were located in the pancreatic head (n = 1), body (n = 2) and tail (n = 2). One patient showed spleen, liver and lung metastases. All five patients underwent pancreatectomy. Enucleation was not performed. One patient with metastases underwent partial hepatectomy. Four tumors had infiltration into the adjacent pancreatic parenchyma. The main pancreatic duct was incorporated into the tumor capsule wall in two patients. Surgical margins were negative in all patients without metastases. The median follow-up period was 37.8 months (range 7-74 months). One patient with metastases died at 60 months after surgery. All other patients were alive and showed no evidence of recurrence. CONCLUSION: Radical resection with negative margins should be performed for SPT. From the perspective of curability, we suggest that pancreatectomy is the first choice for SPT in children.