Duodenal duplication cyst at the second part of the duodenum with congenital duodenal position anomaly completely resected by laparoscopic partial duodenectomy: a case report.

BACKGROUND: Duodenal duplication cysts (DDC) are rare duplications of the alimentary tract. Their treatment depends on their size and location. A radical treatment is total resection, if possible. However, partial excision, puncture, and marsupialization can be selected to prevent surgical injury to the pancreaticobiliary tract despite the risk of recurrence. There are some reports of pancreaticoduodenectomy for DDC because of the risk of recurrent symptoms and malignancy. However, this is considered excessively invasive for DDC, particularly in pediatric cases, because of its extremely low rate of malignancy and high morbidity and mortality rates. We encountered a case of DDC with a congenital duodenal position anomaly occurring in the second part of the duodenum. Taking advantage of the congenital duodenal position anomaly, the DDC was completely resected without injuring the pancreaticobiliary duct. CASE PRESENTATION: A 6-year-old boy was diagnosed with a duodenal duplication cyst with obstruction. There was a congenital duodenal position anomaly. The distal second part of the duodenum was the dorsal side of the proximal second part of the duodenum and ascended upward from the proximal second part of the duodenum. The third and fourth parts of the duodenum ran downward to the left and posterior parts of the portal vein, forming the ligament of Treitz. Complete laparoscopic resection of the duodenal duplication cyst and the second to fourth parts of the duodenum, and duodenojejunostomy with retrocolic reconstruction was performed because the duodenum was easily mobilized to the ligament of Treitz owing to the duodenal position anomaly. The duodenojejunostomy with retrocolic reconstruction achieved a more physiologically normal appearance compared to what would have been achieved with a Roux-en-Y reconstruction. The patient was discharged on postoperative day 12 without any complications. CONCLUSIONS: The procedure used in this case might not be easily applied in all laparoscopy cases. However, it could be an option for duodenal duplication cysts with congenital duodenal position anomalies.

Laparoscopic Fundoplication in Patients with Ventriculoperitoneal Shunts: A Systematic Review and Our Experience.

Background: Data on the outcomes of laparoscopic fundoplication (LF) in patients with ventriculoperitoneal (VP) shunts are limited. Materials and Methods: We retrospectively evaluated the demographic characteristics and outcomes of patients who underwent LF at our institutions between 2014 and 2022. Then, we systematically reviewed articles in MEDILINE/PubMed, Cochrane Library, and Web of Science. Results: There was no significant difference in terms of the outcomes between patients with VP shunt (n = 10) and those without (n = 96) at our institutions. None of the patients presented with shunt trouble after LF. The meta-analysis included four retrospective studies and our institutional data. In total, 605 patients (55 with VP shunt) underwent LF. Furthermore, 2 (3.6%) of 55 patients (1 with infection and 1 with occlusion) had shunt troubles. The conversion and complication rates, operative time, and length of hospital stay did not significantly differ between patients with VP shunt and those without. Conclusions: LF can be safely performed on children with VP shunts and is associated with a low risk of shunt troubles. The Clinical Trial Registration number is 2022-387.

Update on aortopexy and posterior tracheopexy for tracheomalacia in patients with esophageal atresia.

Despite improving the survival after repair of esophageal atresia (EA), the morbidity of EA repair remains high. Specifically, tracheomalacia (TM) is one of the most frequent complications of EA repair. Continuous positive airway pressure is generally applied for the treatment of TM. However, surgical intervention is required against an apparent life-threatening event or inability to perform extubation for a long period. According to our review, most cases of TM showed symptom improvement after aortopexy. The ratio of the trachea's lateral and anterior-posterior diameter at the brachiocephalic artery crossing the trachea, which reflects the compression of the trachea by the brachiocephalic artery, is a good indicator of aortopexy. Our finding suggests that most TM cases associated with EA may not be caused by tracheal fragility alone, but may involve blood vessel compression. Posterior tracheopexy (PT) is also an effective treatment for TM. Recently, open or thoracoscopic PT was able to be performed simultaneously with EA repair. In many cases, aortopexy or PT is a safe and effective surgical treatment for TM with EA. Other surgical procedures, such as external stenting, should be considered for patients with diffuse-type TM for whom aortopexy and PT appear relatively ineffective.

A new criterion including the aspartate aminotransferase-to-platelet ratio index and liver and spleen stiffness to rule out varices needing treatment in children with biliary atresia: Modification of the Baveno VII criteria.

AIMS: Biliary atresia (BA) is a congestive biliary disease that develops in the neonatal period or early infancy. It may present with portal hypertension and varices needing treatment (VNT) even after successful Kasai portoenterostomy. This study aimed to stratify the risk of VNT in children and adolescents with BA. METHODS: In this prospective cross-sectional study, we measured liver stiffness (LS) and spleen stiffness (SS) by two-dimensional shear wave elastography and checked for VNT endoscopically in 53 patients with BA who attended for follow-up between July 2018 and September 2022. Varices needing treatment were defined as large esophageal varices, esophageal varices of any size with red color signs, and/or gastric varices along the cardia. RESULTS: Twenty-five patients (aged 0-18 years) had VNT. Eighteen patients met the Baveno VI criteria (LS <20 kPa; platelet count >150 000/L) and were deemed to be at low risk of VNT (spared endoscopies) while three had missed VNT (16.7%). Applying the Baveno VII criteria, which combines the SS cut-off value of 40 kPa with the Baveno VI criteria, resulted in five missed VNTs among 22 spared endoscopies (22.7%). A modification of the Baveno VII criteria using the aspartate aminotransferase-to-platelet ratio index (APRI) instead of the platelet count with cut-off values of 25 kPa, 30 kPa, and 1.04 for LS, SS, and APRI, respectively, missed only one VNT (5.0%) among 20 spared endoscopies. CONCLUSIONS: A novel diagnostic criterion that combines LS, SS, and APRI reduced the risk of missing VNT to 5% in children and adolescents with BA.

Surgical Strategies for Neonates with Prenatally Diagnosed Congenital Biliary Dilatation.

BACKGROUND: This study aimed to develop a postnatal treatment strategy for infants with prenatally diagnosed congenital biliary dilatation. METHODS: We performed a retrospective study of patients with prenatal diagnosed congenital biliary dilatation (CBD), aged <1 year who underwent surgery at our hospital between 2013 and 2023. We classified the patients into two groups, the "early group," consisting of patients who could not wait for growth, and required early surgery, and the "scheduled group," consisting of patients who were asymptomatic and could undergo scheduled surgery, and compared them. The parameters for early surgical prediction were AST, ALT, TB, DB, and CRP levels at birth, 1 week, 2 weeks, 1 month, 2 months, and 3 months after birth, and immediately before surgery, as well as the cyst diameter, presence of intrahepatic bile duct dilation, and presence of debris in the common bile duct. RESULTS: During the study period, 15 patients were diagnosed prenatally. The cyst diameter was significantly larger at all points in the early group. Patients with a cyst diameter of >30 mm at birth, intrahepatic bile duct dilatation at birth, and postnatal enlargement of the common bile duct to >30 mm are more likely to develop symptoms early. Blood biochemistry tests showed no significant differences between the two groups. CONCLUSIONS: Patients with a cyst diameter >30 mm in the early postnatal period require careful postnatal management and parents should be counseled regarding the high likelihood of their child needing surgery within the first 3 months of life. LEVEL OF EVIDENCE: Level IV.

Preoperative management comprising tube irrigation using a trans-anal indwelling tube for infants with hirschsprung disease can allow single-stage radical surgery.

BACKGROUND: Preoperative management of Hirschsprung's disease (HD) is currently being conducted with the goal of performing single-stage radical surgery without ileostomy. METHODS: We retrospectively reviewed HD cases between 2013 and 2022, as well as their outcomes related to preoperative management. RESULTS: Thirty-nine patients with HD were included in this study, including short-segment HD (30 cases), long-segment HD (4 cases), and total colonic aganglionosis (5 cases). Among these 39 patients, 95% (37 of 39 patients) underwent single-stage radical surgery after management with glycerin enema use (n = 13), irrigation with tube insertion each time irrigation was performed (n = 13), and irrigation using a tube placed in the bowel (n = 11). CONCLUSIONS: Preoperative management of patients with HD allowed for single-stage surgery of long-segment HD and total colonic aganglionosis. Cases that could be managed without performing an emergency enterostomy during the neonatal period were managed with irrigation until radical surgery was performed.

Safe thoracoscopic repair of recurrent congenital diaphragmatic hernia after initial open abdominal repair.

PURPOSE: The optimal surgical approach for recurrent congenital diaphragmatic hernia (CDH) remains controversial. We compared the surgical outcomes of a thoracoscopic approach versus an open abdominal approach for recurrent CDH after initial abdominal open repair. METHOD: The subjects of this comparative study were patients who underwent open abdominal or thoracoscopic surgery for recurrent CDH following an initial open abdominal repair. RESULTS: Among 166 patients with Bochdalek-type CDH, 15 underwent reoperation for recurrent CDH following an open abdominal repair. Seven patients underwent open abdominal surgery (group O) and eight underwent thoracoscopic surgery (group T). The operative duration was similar for the two groups, with less blood loss (17.2 ml/kg vs. 1 ml/kg, P = 0.001) and fewer intraoperative complications in the T group (n = 6 vs. n = 0 cases, P = 0.001). There was no significant difference in the number of postoperative complications (n = 1 vs. n = 1, P = 1.0) or in the number of patients with a second CDH recurrence (n = 2 vs. n = 1, P = 0.569) between the two groups. CONCLUSION: Thoracoscopic surgery is preferable to the open surgical approach for recurrent CDH following an initial abdominal open repair.

Refractory esophageal anastomotic stricture after esophageal atresia surgery improved with retrograde balloon dilatation through gastrostomy followed by laparoscopic fundoplication: a case report.

BACKGROUND: An esophageal anastomotic stricture (EAS) after an esophageal atresia surgery occurs in approximately 4-60% of the cases, and its first-line therapy includes balloon dilatation. Oral balloon dilatation cannot be performed in some EAS cases; conversely, even if dilatation is possible, these strictures recur in some cases, necessitating a surgical procedure for repairing the stenosis. However, these procedures are invasive and have short- and long-term complications. If an EAS recurs repeatedly after multiple balloon dilations, gastroesophageal reflux disease (GERD) may be the underlying cause. A fundoplication procedure may be effective for treating a refractory EAS, as in the present case. CASE PRESENTATION: A neonatal patient with type D esophageal atresia underwent thoracoscopic esophago-esophageal anastomosis at the age of 1 day, and her postoperative course was uneventful. Thereafter, the patient underwent gastrostomy for poor oral intake at the age of 3 months. After gastrostomy, the patient presented with a complete obstructive EAS. Balloon dilatation via the oral route was attempted; however, a guidewire could not be inserted into the EAS site. Hence, retrograde balloon dilatation via gastrostomy was performed successfully. However, the EAS recurred easily thereafter, and laparoscopic anti-reflux surgery was performed to prevent GERD. The anti-reflux surgery cured the otherwise refractory EAS and prevented its recurrence. CONCLUSIONS: Retrograde balloon dilatation is another treatment option for an EAS. When an EAS recurs soon after dilatation, the patient must be evaluated for GERD; if severe GERD is observed, an appropriate anti-reflux surgery is required before dilating the EAS.

Robotic versus laparoscopic radical surgery for pediatric congenital biliary dilatation: a comparison of surgical outcomes of a single surgeon's initial experience.

PURPOSE: This study aimed to clarify the validity of robot-assisted surgery (RAS) for pediatric patients with congenital biliary dilatation (CBD). METHODS: We retrospectively compared RAS and laparoscopic surgery (LS) for pediatric CBD performed by the same certified surgeon between 2016 and 2022. RESULTS: We included 6 RAS and 12 LS cases in this study. One case of RAS with laparotomy was excluded from the analysis. The patients in the two groups had comparable ages and body weights. The median surgery duration, the suture time per stitch, and the time to drain removal were 385 min, 145 s, and 5 days in the RAS group and 370 min (p = 0.28), 177 s (p = 0.03), and 6 days (p = 0.03) in the LS group, respectively. The time to create the Roux-en-Y limb was significantly longer in the RAS group. Postoperative complications occurred in one RAS case and in four LS cases. CONCLUSIONS: Less anastomotic time per stitch and less time to drain removal suggest that RAS may contribute to accurate suturing and fine intra-pancreatic bile duct dissection. In addition, RAS requiring large movements of forceps in a large surgical field, such as Roux-en-Y creation, is inferior to LS.

IgG4-related disease of duodenal obstruction due to multiple ulcers in a 12-year-old girl.

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease and affected individuals typically present with an increased infiltration of IgG4-positive plasma cells in the pancreas, hepatobiliary tract, and liver but rarely in the gastrointestinal tract. CASE PRESENTATION: A 12-year-old girl presented with vomiting and poor weight gain. Gastroscopy revealed duodenal stenosis and ulceration. Computed tomography revealed edematous duodenal wall thickening and air-fluid levels on the right side of the duodenum, which suggested duodenal perforation or penetration. She underwent pancreaticoduodenectomy, and IgG4-RD was diagnosed via histopathology. CONCLUSIONS: This is the first pediatric case of isolated duodenal IgG4-RD resulting in duodenal obstruction after multiple ulcers. Gastrointestinal IgG4-RD should be among the differential diagnoses of unexplained gastrointestinal obstruction or ulceration even in children.

Case Report: Retropancreatic fascia hernia protruding into the thoracic cavity through a Bochdalek hernia.

Retropancreatic fascia hernia is a novel internal hernia originating from the retropancreatic fascial defect, which subsequently expands toward the dorsal aspect of the pancreatic body and migrates into the retroperitoneal space. We encountered a rare case of concomitant retropancreatic fascia and Bochdalek hernias. Here, we describe the imaging characteristics of this hernia type and its surgical strategies.

Case report: Laparoscopic gastrojejunostomy for duodenal atresia with situs inversus and preduodenal portal vein: a report of two cases.

Congenital duodenal atresia with situs inversus is occasionally accompanied by a preduodenal portal vein (PDPV), which is incidentally diagnosed during surgery. Duodenoduodenostomy is the most common and effective treatment. However, some patients require other anastomoses. Here, we present two cases of laparoscopic gastrojejunostomy for congenital duodenal atresia with situs inversus and PDPV and describe the reason for selecting gastrojejunostomy. The optimal surgical strategy is patient specific and should be determined based on the patient's general and physical condition.

Preoperative Contrast Examinations Help Determine the Appropriate Cervical Approach for Congenital Gross Type C Esophageal Atresia: A Report of Two Cases.

BACKGROUND Kluth demonstrated that esophageal atresia/tracheoesophageal fistula (EA/TEF) has several anatomical variations and thus requires a preoperative imaging study to determine the surgical strategy. We routinely perform a contrast examination with iodixanol to assess the location of the TEF and the upper end of the esophageal pouch to determine the most appropriate approach. We herein present two cases of type C EA/TEF who successfully underwent radical surgery by a cervical approach based on the information from the contrast examination. CASE REPORT Case 1 was a Japanese boy suspected of type C EA/TEF after birth. A contrast examination with iodixanol showed that a TEF was at the second thoracic vertebra (Th2), as was the upper end of the esophageal pouch. Thus, the patient underwent esophago-esophageal anastomosis and TEF ligation using a cervical approach; the postoperative course was uneventful. Case 2 was also a Japanese boy suspected of type C EA/TEF. A contrast examination showed that the TEF was at Th1-2, as was the upper end of the esophageal pouch. Thus, the patient underwent esophago-esophageal anastomosis and TEF ligation using a cervical approach. The patient suffered from congenital tracheal stenosis and required tracheoplasty. However, there were no apparent complications after the surgery. CONCLUSIONS Here, we used the imaging information to adopt the cervical approach in type C EA/TEF cases and concluded that routine preoperative contrast examinations helped assess the TEF location and upper end of the esophageal pouch without significant complications.

Anastomotic time was associated with postoperative complications: a cumulative sum analysis of thoracoscopic repair of tracheoesophageal fistula in a single surgeon's experience.

PURPOSE: This study aimed to evaluate the learning curve of thoracoscopic repair of tracheoesophageal fistula (TEF) by a single surgeon using a cumulative sum (CUSUM) analysis. METHODS: Prospective clinical data of consecutive Gross type-C TEF repairs performed by a pediatric surgeon from 2010 to 2020 were recorded. CUSUM charts for anastomosis and operating times were generated. The learning curves were compared with the effect of accumulation based on case experience. RESULTS: For 33 consecutive cases, the mean operative and anastomosis times were 139 ± 39 min and 3137 ± 1110 s, respectively. Significant transitions beyond the learning phase for total operating and anastomosis times were observed at cases 13 and 17. Both the total operating time and anastomosis time were significantly faster in the proficiency improvement phase than in the initial learning phase. Postoperative complications significantly decreased after the initial anastomosis learning phase but not after the initial total operating learning phase. CONCLUSIONS: Thoracoscopic repair of TEF is considered safe and feasible after 13 cases, where the surgeon can improve their proficiency with the total operation procedure, and 17 cases, which will enable the surgeon to achieve proficiency in anastomosis. Postoperative complications significantly decreased after gaining familiarity with the anastomosis procedure through the learning phase.

A new type of retropancreatic fascia hernia in the supramesocolic space preoperatively misdiagnosed as a diaphragmatic hernia: report of two cases.

BACKGROUND: We encountered two cases of a new type of retroperitoneal hernia. We herein report the unique features of these cases. CASE PRESENTATION: Case 1: A Japanese girl was born at a gestational age of 37 weeks, weighing 2550 g. She underwent laparotomic left diaphragmatic hernia repair for a left Bochdalek hernia at the age of one day. The postoperative course was uneventful; however, chest radiography at the age of 35 days revealed bowel gas in the mediastinum, while computed tomography exhibited intestinal prolapses from the medial side of the mesh into the thoracic cavity. Reoperation was performed at the age of 77 days, showing that the defect hole was not at the diaphragm but in the absence of retropancreatic fascia, which was connected to the posterior mediastinum from the supramesocolic space. The mediastinum space was closed with a suturing spine and artificial mesh, and the defect hole in the pancreatic body was sutured. Case 2: A Japanese boy was born at a gestational age of 40 weeks, weighing 3502 g. He was diagnosed with a left diaphragmatic hernia at birth and underwent laparotomy at the age of two days. Operative findings showed no defect hole in the diaphragm, and no intestine was observed in the abdominal cavity. After close observation of the abdominal cavity, the intestine was found around the pancreatic body, and manual reduction of the intestine was performed. The defect hole existed in the absence of the retropancreatic fascia, which was connected to the extra-pleural space. The defect hole in the pancreatic body was sutured and closed with a non-absorbable thread. CONCLUSIONS: We assumed that our cases were a new type of retroperitoneal hernia, which we named "retropancreatic fascia hernia".

The presence of high-risk varices after sclerotherapy in biliary atresia.

BACKGROUND: Esophagogastric varices (EGVs) may develop as a result of portal hypertension in children with biliary atresia (BA). Although endoscopic injection sclerotherapy (EIS) with ethanolamine oleate (EO) is reported useful for children, risk factors associated with the presence of high-risk EGVs after treatment remain unknown. METHODS: The subjects were BA patients under 15 years of age who underwent EO-EIS. We retrospectively reviewed a total of 28 treatment sessions of EGVs with red signs and those larger than F2, which were considered to be at high risk of bleeding. Survival analysis was performed for the presence of high-risk EGVs at the time of follow-up endoscopy as the occurrence of an event. RESULTS: Univariate analysis showed a significantly increased risk of the presence of high-risk EGVs post-EO-EIS in patients with increased liver stiffness (LS) and Mac-2 binding protein glycan isomer (M2BPGi), with hazard ratios of 1.48 and 1.15, respectively. The median presence-free period was significantly shorter in the LS ≥ 2.8 m/s patients than in those with LS <2.8 m/s (189 vs. 266 days). Similarly, the median presence-free period was significantly shorter in patients with M2BPGi ≥ 4.0 than in those with M2BPGi < 4.0 (182 vs. 203 days). The results of multivariate analysis revealed that the risk of the presence of high-risk EGVs was significantly higher only in the high-LS group, with a hazard ratio of 2.76. CONCLUSIONS: Increased LS is associated with risk of the presence of high-risk EGVs following EO-EIS in children with BA.

Hemi-circumferential mucosal resection and anastomosis procedure for rectal prolapse following anorectoplasty for anorectal malformations.

PURPOSE: Rectal prolapse after radical surgery for anorectal malformations (ARMs) is a common postoperative complication that causes bleeding and stenosis, and sometimes requires surgical treatment. Different surgical techniques have been reported, but most are associated with high recurrence rates and a long postoperative stay. We performed the hemi-circumferential mucosal resection and anastomosis procedure (HCMR) to preserve anal muscle and anal function. Here, we report the success of our minimally invasive surgical approach to correct rectal prolapse in patients with ARMs. METHODS: The subjects of this retrospective review were patients who underwent HCMR for rectal prolapse after radical surgery for ARMs between January, 2014 and August, 2021. HCMR involves rectal mucosal resection without muscle plication. RESULTS: A collective 15 HCMR procedures were performed in 10 patients. The median age at repair was 1.8 years (range, 10 months-18 years). The median operation time and postoperative length of stay were 46 min (range, 17-85 min) and 3 days (range, 1-7 days), respectively. The median postoperative observation period was 3 years 4 months (range, 8 months-7 years 10 months) and no complications or recurrences were observed. CONCLUSIONS: HCMR is a safe and effective surgical treatment for rectal prolapse after radical operation for ARMs, which is minimally invasive and preserves rectal muscle and function.

Rare case of a right Bochdalek hernia with retroperitoneal prolapse of organs into the thoracic cavity in infancy: A case report.

We report an extremely rare case of a right Bochdalek hernia with a sac, in which the retroperitoneal and intra-abdominal organs prolapsed into the thoracic cavity at the same time. The patient was a 7-month-old female with no comorbidities. She presented with cough and fever, and chest radiography revealed a right diaphragmatic hernia. Computed tomography showed that the right kidney, intestine, colon, and liver had prolapsed into the thoracic cavity. The patient underwent thoracoscopic surgery, which showed that the abdominal and retroperitoneal organs prolapsed into the thoracic cavity through the Bochdalek hernia. The herniated organs were spontaneously reduced using thoracoscopic insufflation. The defect hole was closed with artificial mesh. We adopted a thoracoscopic approach, in terms of easy reduction of herniated organs and accurate evaluation of the hernia orifice, which was useful.

Laparoscopic closure of Roux limb perforation during double-balloon endoscopic retrograde cholangiography with laparoscopy-assisted endoscopic hepatolithectomy 30 years after congenital biliary dilatation radical surgery: A case report.

We report a case of hepatolithiasis 30 years after congenital biliary dilatation (CBD) surgery. A 31-year-old woman presented with fever and epigastric pain. She had a history of radical surgery for type I CBD at the age of 1 year and had no significant symptoms for approximately 30 years after surgery. Laboratory and imaging results showed hepatolithiasis at the common trunk of segments II and III with cholangitis. She was admitted to our hospital for antibiotics and underwent double-balloon endoscopic retrograde cholangiography (DBERC) to treat the hepatolithiasis. Roux-limb jejunum was perforated during DBERC; hence, emergent laparoscopic perforation site closure and simultaneous endoscopic lithotomy through the Roux-limb jejunum with exteriorization via umbilical incision were performed. The postoperative course was uneventful, and the patient was discharged without any complications.

Retroperitoneoscopic resection of a large paraganglioma surrounded by the right diaphragmatic crus on the dorsal side of the inferior vena cava: A pediatric case report.

Pheochromocytomas and paragangliomas are rare tumors. A 10 year-old girl was brought to the emergency room with complaints of sudden vomiting and convulsions, and was initially diagnosed with hypertensive encephalopathy. Magnetic resonance imaging and computed tomography scan showed a large mass (6 × 3 × 3 cm) on the dorsal side of the inferior vena cava, surrounded by the right diaphragmatic crus, and closely attached to the aorta. Blood noradrenaline, urinary normetanephrine, and noradrenaline levels were elevated. The final diagnosis was retroperitoneal paraganglioma, then, surgery was contemplated. The location of the liver and great vessels in front of the tumor made the commonly performed transabdominal approach complicated. Therefore, retroperitoneoscopic surgery was preferred for safer resection, with better visualization in a sufficient space with less risk of damaging the surrounding organs. The retroperitoneoscopic approach is a good indication for tumors located behind the great vessels.