Pre-operative Serum Albumin Predicts Native Liver Survival in Biliary Atresia.

BACKGROUND: To predict native liver survival (NLS) after Kasai portoenterostomy (KP) for biliary atresia (BA) using pre-operative clinical data. METHODS: Pre-operative data were collected from 29 patients with BA who underwent KP at our department between 1989 and 2017 and were analysed including serum albumin, bilirubin, prothrombin time-international normalised ratio, body height, body weight, age at KP, paediatric end-stage liver disease score calculated using the pre-operative data and the period of NLS. RESULTS: The 10-year NLS rate of all patients was 51%. A multivariate analysis revealed that among all factors, the pre-KP serum albumin level was the only independent predictor of NLS (P = 0.04, hazard ratio = 0.269, 95% confidence interval = 0.077-0.934). The area under the receiver operating characteristic curve for NLS, determined using pre-KP serum albumin was 0.760 and 3.75 mg/dl was selected as the cut-off value. There was a significant difference in NLS between patients with high (≥3.8 mg/dl) and low (≤3.7 mg/dl) pre-KP serum albumin (90.0% vs. 31.5%, P < 0.01). CONCLUSIONS: Decreased pre-KP serum albumin may reflect not only functional impairment of the liver, but also the inflammatory process, which is hypothesized to occur during its advancement. The pre-KP serum albumin level may be a good prognostic factor for NLS in post-KP BA patients.

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.

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.

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".

Laparoscopic Duodenal-Caudal Detachment Method: Early Experience of a Novel Technique for Malrotation with Volvulus in Neonates.

Purpose: A laparoscopic approach for malrotation is feasible and safe in hemodynamically stable neonates without intestinal necrosis; however, volvulus is associated with recurrence and conversion. We developed a novel approach using a laparoscopic duodenal-caudal detachment method to perform the Ladd procedure for neonates with volvulus under the limited view of laparoscopy. This study presents the results, effectiveness, and details of the method. Materials and Methods: In the laparoscopic duodenal-caudal detachment method, we first detached the adhesions around the duodenum, including the Ladd's band. After the adhesions were completely removed, the duodenum was freely drawn caudally, leading to the release of torsion. We retrospectively reviewed the medical records of patients who underwent surgery for malrotation of the volvulus at 30 days of age between January 2014 and September 2021. Results: Seven neonates underwent the laparoscopic duodenal-caudal detachment method and 13 underwent the open Ladd procedure. The new technique was performed in all 7 patients, and there were no conversions or recurrences. The operation time was significantly longer in the laparoscopic procedure group (55 minutes versus 111 minutes; P < .01). Conclusions: Our detorsion method, involving an initial incision of the Ladd's band, is safe and effective for neonates and may lead to an improvement in the conversion rates.

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 internal intestinal drainage of bile lakes in a patient with recurrence of jaundice after laparoscopic revision of Kasai portoenterostomy for biliary atresia: A case report.

Bile lake, of the postoperative complications after Kasai portoenterostomy (PE) for biliary atresia, causes cholangitis that may induce progressive fibrosis of the liver. Standard treatment for bile lakes has not yet been established, but there are reports that surgical internal intestinal drainage for bile lakes effectively prevents cholangitis and maintains jaundice-free status. In this case, insertion of the percutaneous transhepatic biliary drainage into the bile lake allowed continuous drainage of large volumes of bile juice. However, reoperation following laparotomy increases the surgical risk of subsequent liver transplantation due to postoperative adhesion. Laparoscopic surgery was selected for the patient who was likely to require liver transplantation in the future. In this case, laparoscopic internal intestinal drainage of bile lakes was performed safely by a Cavitron ultrasonic surgical aspirator for the recurrence of jaundice after laparoscopic revision of PE. Cholangitis and jaundice were rapidly resolved after this surgical procedure.

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.

Preoperative imaging contributes to pathologically complete resection of the urachal remnant by determining an appropriate surgical approach without unnecessary and excessive surgical invasion: a retrospective study.

BACKGROUND: The urachus is an embryonic structure that connects the bladder to the allantois during early embryonic development. Occasionally, it fails to disappear at birth, leading to a case of urachal remnant (UR). This study aimed to determine whether our policy for selecting an appropriate UR resection approach is valid. We performed preoperative imaging to examine whether UR continued toward the bladder apex. If so, the UR and bladder apex were excised using the trans-umbilical approach, in addition to laparoscopy, if necessary. If preoperative imaging indicated that the UR ended near the umbilicus, the UR from the umbilicus to the duct end was resected. Pathological evaluations were performed to determine the appropriateness of the surgical approach indicated by preoperative imaging. METHODS: We retrospectively reviewed pediatric patients with UR who underwent surgery between 2015 and 2021. Their background characteristics and surgical outcomes were evaluated. RESULTS: Twenty patients with UR were included (median age, 7 [interquartile range, 2-10.25] years). UR continued toward the bladder apex in 10 patients and ended near the umbilicus in 10 patients. Urachus tissue at the bladder site was observed when the UR and bladder apex were excised. When UR was resected from the umbilicus to the duct end, urachus tissue was not pathologically detected at the resection margin. CONCLUSION: Our policy results in complete resection without excessive surgical invasion.

Combined negative pressure wound therapy with irrigation and dwell time and artificial dermis prevents infection and promotes granulation formation in a ruptured giant omphalocele: a case report.

BACKGROUND: Omphalocele is a congenital abdominal wall defect of the umbilical cord insertion site. A giant omphalocele, with a fascial defect > 5 cm in diameter and/or containing > 50% of the liver within the hernia sac, can be challenging for pediatric surgeons. Recently, negative pressure wound therapy has been reported as an effective management for giant omphaloceles; however, it is not recommended for an infected wound with necrotic tissue as it may exacerbate infection. We adopted negative pressure wound therapy with irrigation and dwell time (NPWTi-d) for a case of a ruptured giant omphalocele. Artificial membranes, followed by artificial dermis, were used to promote fibrous capsule formation, and then NPWTi-d was used to promote granulation while controlling infection. However, studies have not been conducted regarding NPWTi-d for ruptured giant omphaloceles; hence, we present our treatment experience with NPWTi-d for a giant omphalocele. CASE PRESENTATION: The patient was a boy born at 38 weeks and 3 days of gestation, weighing 1896 g. He was diagnosed with a ruptured giant omphalocele with a total liver and intestine defect hole of 10 cm × 10 cm. The patient underwent silo placement using an artificial mesh, followed by plicating the artificial mesh at 4 days of age. The herniated viscera were gradually reduced into the abdominal cavity; however, the defect size was still large. Hence, a collagen-based artificial dermis was patched on the defect hole. After creating a fresh and smooth granulated tissue, NPWTi-d was applied at 33 days of age to promote granulation and control infection. We used the 3 M™ V.A.C.® Ulta Therapy Unit with 3 M™ VeraFlo™ therapy. NPWTi-d was stopped at 60 days of age when the granulation tissue was well formed including at the artificial dermis site. The wound was managed with prostandin ointment and appropriate debridement, resulting in complete epithelialization at 5 months of age. CONCLUSIONS: Artificial membranes followed by artificial dermis were used to promote a fibrous capsule and artificial dermis granulation, which protects against organ damage. NPWTi-d achieved better control of infection and promoted wound healing. NPWTi-d combined with artificial dermis can effectively treat ruptured giant omphaloceles.

Case report: A giant bilateral inguinal hernia requiring artificial mesh and multi-stage surgery in infancy; hernioplasty with silo placement to prevent acute compartment syndrome.

Hernioplasty for giant inguinal hernias can cause abdominal compartment syndrome (ACS) in adults but rarely does in infants. We encountered a case of a giant bilateral inguinal hernia in infancy complicated by ACS after hernioplasty. Silo placement via a skin incision effectively treated ACS, after which the abdominal wall was safely closed. Hernioplasty performed early in the clinical course can help expand the abdominal cavity and avoid ACS. Thus, hernioplasty should be performed earlier if the hernia size in the flank space gradually increases.

Refractory tracheoesophageal fistula treated using multi-stage surgery: A case report.

A tracheoesophageal fistula (TEF) recurs in approximately 2%-13% of cases of esophageal atresia with TEF that are treated surgically. Currently, there is no consensus on the most effective treatment to prevent recurrent TEF (RTEF). Herein, we present a patient with type C esophageal atresia who underwent thoracoscopic esophago-esophageal anastomosis and TEF repair at 2 days old. However, RTEFs were observed at ages 3, 6, and 11 months, and thoracoscopic TEF repairs using a pleural patch, fascia lata graft, and pectoralis major myocutaneous (PMMC) flap were performed, respectively. A fourth recurrence led to mediastinitis, shock liver, disseminated intravascular coagulopathy, and a compromised respiratory status. Hence, laparoscopic esophageal transection was first performed to improve the respiratory condition by preventing the regurgitation of gastric contents. Once the patient was stable, a subtotal esophageal resection with TEF closure followed by gastric tube reconstruction was performed. In conclusion, we encountered a case of refractory RTEF that was repaired four times using various techniques, including a fascia lata graft and PMMC flap. However, TEF still recurred after these four operations. The final surgical strategy involved an esophageal transection as a palliative therapy, which improved the respiratory condition, followed by closure of the TEF and subtotal esophageal resection. Finally, esophageal reconstruction using a gastric tube after the complete remission of inflammation was effective. This multi-stage surgery was considered the only choice to rescue the patient and effectively prevent another recurrence.

Laparoscopic restorative proctocolectomy with ileal-J-pouch anal canal anastomosis without diverting ileostomy for total colonic and extensive aganglionosis is safe and feasible with combined Lugol's iodine staining technique and indocyanine green fluorescence angiography.

Background: We present the surgical technique and outcomes of reduced-port laparoscopic restorative proctocolectomy with ileal-J-pouch anal canal anastomosis (IPACA) without diverting ileostomy for total colonic and extensive aganglionosis (TCA+). Methods: We retrospectively reviewed TCA+ cases between 2014 and 2022. Preoperative ileostomy was performed when transanal bowel irrigation was ineffective. Radical surgery for TCA+ was performed at approximately 6 kg. The surgery was performed using laparoscopy through a multi-channel trocar with or without an additional 3-mm trocar and IPACA reconstruction with indocyanine green fluorescence angiography (ICG) to assess anastomotic perfusion and Lugol's iodine staining to visualize the surgical anal canal. Results: Ten patients with TCA+ were included. Ileostomy was performed in seven cases. The median operation time and blood loss were 274.5 min and 20 ml, respectively. No significant postoperative complications were found. All patients experienced frequent liquid stools and perianal excoriation in the early postoperative period, requiring anti-flatulence or codeine. The median follow-up period was 3.5 years. Three patients required irrigation management 1 year postoperatively, and the others defecated a median of 3.5 times per day. The median Kelly's clinical score was 5 in 5 patients aged >4 years. Conclusion: Reduced-port surgery, combined with Lugol's iodine staining and ICG, was safe, feasible, and had cosmetically and clinically acceptable mid-term outcomes.

Single-incision laparoscopic full-thickness anterior abdominal wall repair of a Morgagni hernia using a suture-assisting needle in a child: A case report.

Single-incision laparoscopic repair of a congenital Morgagni diaphragmatic hernia using a suture-assisting needle was performed in a 1-year-old boy. Three ports were inserted through a single umbilical incision to repair the 2.5 × 2.3-cm defect. The full-thickness muscle layer of the anterior abdominal wall and the posterior rim of the defect were penetrated with the suture-assisting needle holding a thread, which was then released. The needle tip was pulled back over the muscle layer, shifted laterally, and again passed through the muscle layer and the posterior rim. The thread was then captured by the needle and pulled out through the anterior abdominal wall. Five mattress sutures were placed in this way and tied subcutaneously. The postoperative course was uneventful, and the cosmetic outcome was favorable. A suture-assisting needle is useful for completing full-thickness anterior abdominal wall repair, which is important for preventing the recurrence of a congenital Morgagni diaphragmatic hernia.

Combined Fundal and Segmental Adenomyomatosis of the Gallbladder in a Child: A Rare Case Report.

Adenomyomatosis of the gallbladder (AMG) is characterized by mucosal hyperplasia leading to invagination through the thickened muscle layer, which is relatively common in adults, but is rare in childhood. We report a 12-year-old boy with adenomyomatosis of the gallbladder combined segmental and fundal type. This combined type is rare in adults and is first reported here in childhood. Although initial imaging with computed tomography (CT) suggested the presence of a circular solid mass-like lesion because of its rare morphology, repeated ultrasonography (US) was useful for leading to a correct diagnosis.

A single institution experience of Eppikajutsuto for the treatment of lymphatic malformations in children.

BACKGROUND: Eppikajutsuto (TJ-28) is an herbal medicine recently reported to be effective in treating lymphatic malformations (LMs). We report our experience concerning the clinical efficacy of TJ-28 for LMs. METHODS: Medical records of 10 LM cases treated with TJ-28 between 2016 and 2018 were reviewed. TJ-28 was given at 0.3 g/kg/day and then increased to 0.5 if no improvement was noted after the first three months of treatment. Their clinical data were collected, and LM volume indices (depth×width×height) were measured with the first (LMVI-F) and latest (LMVI-L) imaging studies. The response rates were calculated as 1-LMVI-L / LMVI-F (%). RESULTS: The median age at the diagnosis and treatment period was 1.5 years and 17.5 months, respectively. LMs were located in the neck (six), mesenterium or retroperitoneum (three), and inguinal region (one). The median response rate was 83%, including 100% in three cases and the apparent improvement of obstructive airway symptoms in one case. One case underwent surgery for insufficient improvement, and another that showed no effect is being considered for surgery. Most of the satisfactory outcomes were demonstrated in the first six months of treatment. CONCLUSION: TJ-28 seems to be effective in treating LMs in children, especially early in treatment. LEVEL OF EVIDENCE: IV.

Complete Genome Sequence of Thermus thermophilus TMY, Isolated from a Geothermal Power Plant.

Thermus thermophilus TMY (JCM 10668) was isolated from silica scale formed at a geothermal power plant in Japan. Here, we report the complete genome sequence for this strain, which contains a chromosomal DNA of 2,121,526 bp with 2,500 predicted genes and a pTMY plasmid of 19,139 bp, with 28 predicted genes.