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

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

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

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

Rectal intussusception and external rectal prolapse are common at proctography in patients with mucus discharge.

OBJECTIVES: Although various pelvic floor abnormalities are recognized to cause mucus discharge (MD), little is known about the exact distribution and frequency of diseases causing MD in evacuatory disorders. This study aimed to identify the most common diseases at evacuation proctography in patients with MD. METHODS: Patients seen with symptoms of evacuatory disorder underwent proctography. Data for patients with MD who were not associated with fecal incontinence (FI) were prospectively entered into a database and analyzed retrospectively. The degree of MD was documented using FI Severity Index. RESULTS: Sixty-two patients were included for analysis. Forty-nine (79%) had rectal intussusception (RI) or external rectal prolapse (ERP). Of those with RI, MD was observed more in patients with recto-anal intussusception (n = 22) than those with recto-rectal intussusception (n = 8). Of the 39 patients who were not associated with hemorrhoids or mucosal prolapse, 31 (79%) had RI or ERP. Meanwhile, of 582 patients who underwent proctography, 301 had RI and 96 had ERP. MD without FI was present in 13% (40/301) patients with RI and 9% (9/96) with ERP. Surgery was performed in 21 patients, and MD was cured in 20 (95%) postoperatively. CONCLUSIONS: RI and ERP were common at proctography in patients with MD.

Rectoanal intussusception is very common in patients with fecal incontinence.

OBJECTIVES: Fecal incontinence (FI) is a multifactorial disorder, the etiology of which is not fully understood. Recent data have shown the significance of rectoanal intussusception (RAI) in the evaluation of FI. The present study aimed to determine the incidence of RAI in patients with FI. METHODS: Between June 2010 and February 2016, 74 patients, who were evaluated using evacuation proctography, anorectal manometry, ultrasound, and incontinence scores, were included in this study. RAI was diagnosed when the apex of the rectal intussusception (RI) impinged on the internal anal orifice or was intra-anal, based on the images taken during maximal straining defecation at evacuation proctography. The characteristics of RAI patients were further analyzed. RESULTS: There were 59 women (80%) and 15 men, with a median age of 74 (52-93) years. Sixty patients (81%) had RI, and 56 (76%) showed RAI. The incidence of RAI among the 32 patients with FI alone and the 42 patients with FI and symptoms of obstructed defecation (OD) was 72% (23/32) and 79% (33/42), respectively. The incidence of RAI was not significantly different between the patients with normal manometry (maximum resting pressure [MRP] ≥55 cmH2O and maximum squeeze pressure [MSP] ≥150 cmH2O, n=26) and those with subnormal manometry (MRP <55 cmH2O and/or MSP <150 cmH2O, n=48). CONCLUSION: RAI is common in patients with FI. Evacuation proctography should be taken into account as a part of the regular study of FI patients.

Triphosphate Ion-Selective Electrode Based on Zr-Porphyrin Complex.

Ion-selective electrode using zirconium(IV) complex with octaethylporphin (H2oep) as a carrier showed high selectivity to triphosphate (TP, H5tp) against other hydrophilic anions including diphosphate and phosphate. The electroactive species was identified to be [(Zr4(oep)4(Htp)2] (TP/Zr ratio of 0.5) of the unique structure; triphosphates are recognized by one Zr atom through three O atoms on three different P atoms and by another Zr atom through two O atoms on two terminal P atoms and are also involved in complementary intermolecular hydrogen bonding to be surrounded by four porphyrin complexes. In contrast, Zr(IV) in the other complex with tetraphenylporphin has the higher Lewis acidity, due to the electron-withdrawing property of phenyl rings and, at the higher TP concentration, forms a species having a TP/Zr ratio of unity, which precipitates to lose the electroactivity. The electrode was successfully applied to monitor hydrolysis of TP that provides diphosphate and phosphate.

Proctographic findings and symptoms in patients with anterior rectoanal intussusception.

OBJECTIVES: Rectoanal intussusception (RAI) is a common finding on defecography in patients with defecation disorders. This study aimed to compare the proctographic findings and symptoms between patients with anterior RAI and those with circular RAI. METHODS: We included 208 patients who were diagnosed as having RAI on defecography. Anorectal function was evaluated using Constipation Scoring System (CSS) and Fecal Incontinence Severity Index (FISI). RESULTS: Twenty-four patients had anterior RAI and 184 had circular RAI. While the anterior intussusception descent or pelvic floor descent was significantly smaller in patients with anterior RAI than those with circular RAI [14.3 vs. 18.5 mm, p=0.004; 12.4 vs. 21.6 mm, p=0.005], there were no significant differences in incidences of obstructed defecation (OD) and fecal incontinence (FI) between the groups. Sixteen patients with anterior RAI and 137 patients with circular RAI had OD. There was no significant difference in the CSS scores between the groups. Twelve patients with anterior RAI and 108 patients with circular RAI had FI. No significant difference in the FISI scores between the groups. CONCLUSIONS: Approximately one tenth of the whole RAI was anterior in location, and symptoms in patients with anterior RAI were similar to those with circular RAI.