Massive Colostomy Prolapse with an Intrastomal Ileal Hernia: A Case Report.

A 79-year-old man underwent sigmoid colostomy about 50 years previously and sought surgical reconstruction of the colostomy. He presented with 30 cm of prolapsed stoma accompanying an intrastomal hernia which contained ileum. The prolapsed stoma which led to the intrastomal hernia was made from the distal sigmoid colon, and the everted colon wall constituted the hernia sac. A computed tomography scan was useful to demonstrate the contents of the intrastomal hernia. Reconstruction with relocation of the colostomy was considered appropriate for the presented patient. The thickened and stretched distal sigmoid colon was resected with the stoma. A new end colostomy using the descending colon was seated in the left upper quadrant. The lateral pararectus muscles which formed the 8-cm hernia orifice were closed using tension-reducing incisions. The postoperative course was uneventful.

Outcomes After Living Donor Liver Transplantation in Pediatric Patients with Inherited Metabolic Diseases.

BACKGROUND There is no consensus about the long-term prognosis of pediatric patients with a variety of rare liver diseases but with inherited metabolic diseases (IMDs). We retrospectively reviewed the developmental outcomes of patients with IMDs undergoing living donor liver transplantation (LDLT). MATERIAL AND METHODS Between May 2001 and December 2020, of 314 pediatric patients who underwent LDLT, 44 (14%) had IMDs. The median age at LDLT was 3.0 years old (range 0-15.0 years). Associations between the post-transplant complications and graft survival rate in patients with IMDs and biliary atresia (BA) were calculated. We evaluated the safety of LDLT from heterozygous carrier donors, the prognosis of patients with IMDs who have metabolic defects expressed in other organs, and developmental outcomes of patients with IMDs. RESULTS The 10-year graft survival rates in patients with IMDs and BA were 87% and 94%, respectively (P=0.041), and the causes of graft failure included pneumocystis pneumonia, acute lung failure, hemophagocytic syndrome, hepatic vein thrombosis, portal vein thrombosis, and sepsis. The rate of post-transplant cytomegalovirus viremia in patients with IMDs was higher than that of patients with BA (P=0.039). Of 39 patients with IMDs, 15 patients (38%) had severe motor and intellectual disabilities in 4 patients, intellectual developmental disorders including epilepsy in 2, and attention-deficit hyperactivity disorder in 2. Of 28 patients with IMDs, 13 (46%) needed special education. CONCLUSIONS The long-term outcomes of LDLT in patients with IMDs are good. However, further long-term social and educational follow-up regarding intellectual developmental disorders is needed.

Laparoscopic Totally Extraperitoneal (TEP) Groin Hernia Repair in Patients After Contralateral TEP Groin Hernia Repair.

BACKGROUND: The totally extraperitoneal (TEP) repair for groin hernia is considered difficult in patients with a healed surgical scar in the lower abdomen. We reported the feasibility of repair in those patients and found that the most frequent previous procedure in patients for whom the procedure was changed intraoperatively was a contralateral TEP after TEP repair. We now report an expanded patient cohort with a suggested unified treatment strategy. MATERIALS AND METHODS: From 2006 to 2020, 443 patients underwent laparoscopic TEP groin hernia repair. A contralateral TEP after TEP repair was performed in 35 patients. The conversion rate after TEP was compared with that after other operations. Patients were divided into completed contralateral TEP after TEP repair (N=28) and changed procedure groups (N=7). Clinical characteristics were compared including age, body mass index, location and type of hernia, and interval after previous surgery. Multivariate analysis was performed to evaluate risk factors for conversion of the TEP procedure. RESULTS: Patients undergoing contralateral TEP after TEP repair were significantly overrepresented among patients for whom the procedure was changed compared with other previous operations (P<0.01), with an odds ratio of 19.91. Comparing completed TEP after TEP repair and changed procedure groups, there were no significant differences regarding age (mean: 67 vs. 69 y old), body mass index (22.4 vs. 22.5 kg/m2), type of hernia (indirect or direct), or duration after previous TEP repair (median: 642 vs. 470 d) and identified no significant risk factors. CONCLUSIONS: The contralateral TEP after TEP repair for groin hernia is feasible. However, dense adhesions may be present if balloon dissection was performed at the previous TEP repair, and it is necessary to carefully dissect being ready to convert to other procedures such as TAPP repair or an anterior approach.

Ultrasonographically guided percutaneous transhepatic liver biopsy after pediatric liver transplantation.

BACKGROUND: Complications associated with ultrasonographically guided percutaneous transhepatic liver biopsy (PTLB) after liver transplantation (LT) have been rarely reported, and there is no consensus about its safety. We retrospectively reviewed the safety and outcomes of PTLB after pediatric LT. METHODS: Between January 2008 and December 2019, 8/1122 (0.71%) pediatric patients who underwent ultrasonographically guided PTLB after LT developed complications. The median age at PTLB was 7.8 years (range 0.1-17.9). Grafts included left lobe/left lateral segment in 1050 patients and others in 72. PTLB was performed using local anesthesia±sedation in 1028 patients and general anesthesia in 94. RESULTS: Complications after PTLB included acute cholangitis in 3 patients, sepsis in 2, respiratory failure due to over-sedation in 1, subcapsular hematoma in 1, and intrahepatic arterioportal fistula in 1. The incidence of complications of PTLB in patients with biopsy alone and those with simultaneous interventions was 0.49% and 3.19%, respectively (p = .023). Patients who developed acute cholangitis, respiratory failure, subcapsular hematoma, and arterioportal fistula improved with non-operative management. Of two patients with sepsis, one underwent PTLB and percutaneous transhepatic portal vein balloon dilatation and developed fever and seizures the following day. Sepsis was treated with antibiotic therapy. Another patient who underwent PTLB and exchange of percutaneous transhepatic biliary drainage catheter developed fever and impaired consciousness immediately. Sepsis was treated with antibiotic therapy, mechanical ventilation, and continuous hemofiltration. CONCLUSIONS: Percutaneous transhepatic liver biopsy after pediatric LT is safe. However, combining liver biopsy with simultaneous procedures for vascular and biliary complications is associated with an increased risk of complications.

Peliosis Hepatis in a Child with X-Linked Myotubular Myopathy Treated with Living-Donor Liver Transplant: A Case Report.

BACKGROUND: Myotubular myopathy is a rare disease sometimes accompanied by peliosis hepatis, a leading cause of fatal liver hemorrhage. CASE REPORT: We present a case of a 2-year-old boy with myotubular myopathy who developed liver hemorrhage because of peliosis hepatis and was successfully treated with living-donor liver transplant. The patient initially presented with fever, anemia, and liver dysfunction. A computed tomographic scan revealed hemorrhages in the liver, and the patient underwent hepatic artery embolization twice. After the second embolization, multiple peliosis hepatis cavities appeared in the left lobe of the liver that had increased in size. Therefore, the patient underwent ABO-incompatible living-donor liver transplant using a lateral segment graft from his father. The patient developed severe septic shock with an unknown focus on postoperative day 18, which resolved with antibiotic therapy. On postoperative day 62, he was discharged. Fourteen months after undergoing living-donor liver transplant, the patient showed no recurrence of peliosis hepatis. CONCLUSIONS: Although the long-term prognosis of peliosis hepatis due to myotubular myopathy after living-donor liver transplant remains unclear, liver transplant may be a curative treatment for patients with myotubular myopathy who have uncontrollable peliosis hepatis.

Liver Transplant for Posthepatectomy Liver Failure in Hepatoblastoma.

OBJECTIVES: Predicting the risk of posthepatectomy liver failure is important when performing extended hepatectomy. However, there is no established method to evaluate liver function and improve preoperative liver function in pediatric patients. MATERIALS AND METHODS: We show the clinical features of pediatric patients who underwent living donor liver transplant for posthepatectomy liver failure in hepatoblastoma. The subjects were 4 patients with hepatoblastoma who were classified as Pretreatment Extent of Disease III, 2 of whom had distal metastasis (chest wall and lung). RESULTS: Hepatic right trisegmentectomy was performed in 3 patients and extended left hepatectomy in 1 patient. The median alpha-fetoprotein level at the diagnosis of hepatoblastoma was 986300 ng/mL (range, 22500-2726350 ng/mL), and the median alpha-fetoprotein level before hepatectomy was 8489 ng/mL (range, 23-22500 ng/mL). The remnant liver volume after hepatectomy was 33.3% (range, 20% to 34.9%). Four patients had cholangitis after hepatectomy and progressed to posthepatectomy liver failure. The peak serum total bilirubin after hepatectomy was 11.4 mg/dL (range, 8.7-14.6 mg/dL). Living donor liver transplant was performed for these 4 patients with posthepatectomy liver failure, and they did not have a recurrence. CONCLUSIONS: When the predictive remnant liver volume by computed tomography-volumetry before extended hepatectomy for patients with hepatoblastoma is less than 40%, the possibility of posthepatectomy liver failure should be recognized.

Antithrombin III treatment for portal vein thrombosis after living donor liver transplantation: a case report.

BACKGROUND: There have been no reports on the effectiveness of the administration of antithrombin III (AT III) for post-transplant portal vein thrombosis (PVT). We herein report a case of post-transplant PVT that was resolved by AT III treatment after living donor liver transplantation (LDLT). CASE PRESENTATION: The patient was a 57-year-old man who had been diagnosed with decompensate liver cirrhosis by hepatitis C virus infection. He presented with repeated hepatic coma and refractory ascites. Computed tomography (CT) revealed PVT of Yerdel classification grade II before LDLT. He underwent ABO-identical LDLT using a right lobe graft. A liver function test revealed elevated liver enzyme levels on post-operative day (POD) 14. The CT examination on POD 15 revealed PVT in the left side of the main portal vein at the side of left gastric vein ligation. AT III treatment from POD 15 to POD 24 was performed. Magnetic resonance imaging revealed that the PVT had decreased 10% on POD 27. Furthermore, AT III treatment from POD 28 to POD 32 was performed. The CT examination demonstrated the disappearance of PVT on POD 69 and thereafter, he had no recurrence of PVT on 10 post-operative month (POM). CONCLUSIONS: The present case suggests that the administration of AT III is safe and suitable for the treatment of post-transplant PVT.

Pregnancy Outcomes Following Pediatric Liver Transplantation: A Single-Center Experience in Japan.

BACKGROUND The number of pregnancies after liver transplantation (LT) is increasing; however, the safety and incidence of complications associated with these pregnancies are still unclear. In this report, we retrospectively assessed the influences and problems associated with post-transplant pregnancy on allografts, recipients, and fetuses. MATERIAL AND METHODS A total of 14 pregnancies were identified in 8 female recipients between 2005 and 2018. The original disease was biliary atresia in all recipients. We provide a basic guide for the management of planned pregnancies in female recipients. RESULTS Of the 7 planned pregnancies, no recipients took mycophenolate mofetil (MMF) or had allograft liver dysfunction. Among the 7 unplanned conceptions, we judged that the pregnancy was inadequate to continue in 4 recipients due to taking MMF and 2 recipients due to allograft liver dysfunction at conception. However, 4 recipients who immediately stopped taking MMF continued with their pregnancies. Ten pregnancies resulted in live 11 births. Among obstetric complications or fetal and neonatal complications, gestational diabetes mellitus in 3 recipients was the most common. There were 3 miscarriages and 1 planned termination because of MMF medication and liver dysfunction. CONCLUSIONS Planned pregnancies in LT recipients can lead to the birth of a healthy baby and no influence on either the allograft or the recipient. However, unplanned pregnancies in LT recipients, such as recipients who take MMF or have allograft liver dysfunction, may have an adverse influence on the fetus.