Bile acid profiles in adult patients with biliary atresia who achieve native liver survival after portoenterostomy.

Bile acids have received increasing attention as a marker of the long-term prognosis and a potential therapeutic target in patients with biliary atresia, which is a progressive disease of the hepatobiliary system. A detailed analysis of serum and urinary bile acid compositions was conducted to assess the characteristics of bile acid profiles and the correlation between bile acid profiles and liver fibrosis markers in adult patients with biliary atresia who achieved bilirubin normalization. Serum total bile acids and glucuronide-conjugated (glyco- and tauro-) cholic acids (GCA and TCA) and chenodeoxycholic acids (GCDCA and TCDCA) were significantly higher in patients with biliary atresia than in healthy controls, whereas unconjugated CA and CDCA showed no significant difference. There were no significant differences in CA to CDCA ratios and glycine-to-taurine-conjugated ratios. Urinary glycocholic acid 3-sulfate (GCA-3S) was significantly higher in patients with biliary atresia. Serum GCDCA showed a strong positive correlation with Mac-2 binding protein glycosylation isomer (M2BPGi). These results demonstrate that bile acid congestion persists into adulthood in patients with biliary atresia, even after cholestasis has completely improved after Kasai portoenterostomy. These fundamental data on bile acid profiles also suggest the potential value of investigating bile acid profiles in patients with biliary atresia.

Advantages of Urethroplasty Terminating at the Corona for Severe Hypospadias: Complications, Uroflowmetry, and Esthetics.

PURPOSE: Mid-shaft/proximal (msp) and penoscrotal/scrotal/perineal (pssp) hypospadias treated by urethroplasty (UP) terminating at the corona (UPC) or UP terminating at the tip of the glans (UPG) were compared. METHODS: UP performed at a single institute (n = 234) between 2003 and 2020 were grouped as: msp-UPC (n = 23), msp-UPG (n = 75), pssp-UPC (n = 81), and pssp-UPG (n = 55) to compare data obtained from medical records for post-UP complications (PUC; urethral stenosis, urethrocutaneous fistula, diverticulum formation, and bleeding; n = 234), post-UP uroflowmetry (PUF; average flow rate (Qave), maximum flow rate (Qmax), voiding time (VT), voided volume (VV) and urine flow curves) in 57 UP patients [msp-UPC (n = 5), msp-UPG (n = 12), pssp-UPC (n = 32), pssp-UPG (n = 8)] and 9 controls, and post-UPC esthetics (EST; n = 104). P < 0.05 was considered significant. RESULTS: Mean ages at UP (years) were: msp-UPC (3.1 ± 3.0), msp-UPG (3.3 ± 1.4), pssp-UPC (4.6 ± 2.4), and pssp-UPG (3.4 ± 1.4); p < 0.0001 by ANOVA test. Overall, there were significantly more PUC in pssp-UPG compared with pssp-UPC except for bleeding. Ages at PUF assessment were similar. Mean Qave (mL/s) for pssp-UPG (4.0 ± 1.0) was significantly less than pssp-UPC (5.9 ± 2.5; p < 0.05) and both were significantly less than controls (6.9 ± 1.8; p < 0.05, p < 0.01, respectively). Mean Qmax (mL/s) for pssp-UPC (11.4 ± 4.8) was significantly better than pssp-UPG (7.8 ± 2.3; p < 0.05) and for controls (14.9 ± 4.4) was significantly better than pssp-UPG (7.8 ± 2.3; p < 0.01). All VT (seconds) were similar to controls; all urine flow curves were normal. For EST in UPC (n = 104), 3 cases requested meatal advancement. CONCLUSIONS: UPC may be a valid option for treating pssp hypospadias because of significant differences in PUC/PUF and minimal EST issues compared with UPG. LEVEL OF EVIDENCE: Prognosis Study Level-Ⅱ.

Native liver survivors of portoenterostomy for biliary atresia with excellent outcome: redefining "successful" portoenterostomy.

PURPOSE: Native liver survivors (NLS) after portoenterostomy (PE) for biliary atresia (BA) with normal biomarkers defined as total bilirubin (T-Bil), aspartate aminotransferase (AST)/alanine aminotransferase (ALT) for liver function (LF), cholinesterase (ChE), platelet count (PC), and absence of portal hypertension (PHT) were reviewed to redefine "successful" PE. METHODS: 92 post-PE BA patients were classified as NLS-1: normal biomarkers, PHT (-); NLS-2: at least one abnormal biomarker, PHT (-); NLS-3: normal biomarkers, PHT ( +); NLS-4: abnormal biomarkers, PHT ( +) and reviewed for a maximum 32 years. RESULTS: As of June 2022, 55/92 (59.8%) had received liver transplants and 37/92 (40.2%) were NLS. NLS patients were classified as excellent outcome (EO): NLS-1 (n = 10; 27.0%) or non-EO: NLS-2: (n = 8; 21.6%), NLS-3: (n = 6; 16.2%), and NLS-4: (n = 13; 35.1%). Compared with non-EO, EO had PE earlier (50.5 versus 65 days; not significant; p = 0.08), significantly earlier onset of symptoms (13 days versus 32 days; p = 0.01) and significantly shorter jaundice-clearance (JC; 34.5 days versus 56.0 days; p < 0.001). Durations of follow-up were similar: 13 years in EO, 18.5 years in NLS-2, 20 years in NLS-3, and 15 years in NLS-4. CONCLUSION: Incidence of "successful" PE or EO is low and correlated with early onset of symptoms and quicker JC.

Does pneumoperitoneum adversely affect growth, development and liver function in biliary atresia patients after laparoscopic portoenterostomy?

PURPOSE: We assessed the effect of high partial pressure of arterial carbon dioxide (PaCO2) due to pneumoperitoneum (PP) on growth (height/weight) and development (gross/fine motor function, receptive/expressive communication, and social interaction), by comparing outcome after portoenterostomy (PE) for biliary atresia (BA) using laparoscopic PE (LPE: n = 13) and open PE (OPE: n = 13) cases performed between 2005 and 2014. METHODS: Our PE is based on Kasai's original PE. All data were collated prospectively. RESULTS: Differences in duration of follow-up (LPE: 38.8 months; OPE: 38.1 months), jaundice clearance (LPE: 12/13 = 92.3 %; OPE: 9/13 = 69.2 %), survival with the native liver (LPE: 10/13 = 76.9 %; OPE: 9/13 = 69.2 %), incidence of cholangitis, hypersplenism, and incidence of esophageal varices were not significant. Mean intraoperative PaCO2 was significantly higher in LPE (LPE: 50.1 mmHg; OPE: 40.7 mmHg, p < 0.05). Liver function impairment was not statistically different, although LPE results were slightly worse. There was no overall delay in growth observed, although height/weight gain was more consistent in LPE. The pattern of developmental delay observed was similar for LPE and OPE suggesting that developmental delay is not PE-related; in other words, PP is not implicated in developmental delay. CONCLUSIONS: PP during LPE would appear to have no adverse effects on overall growth/development and liver function in BA patients.

Long-term survival with cloacal dysgenesis sequence.

Cloacal dysgenesis sequence (CDS) is a rare and lethal malformation. We report such a case of long-term survival, currently to 12 years of age. In the fetal period, she received a timely placement of vesico-amniotic shunt for a megabladder due to a severe urethral obstruction. Postnatally, cystostomy and colostomy were created because of no perineal opening of urethra, vagina, and anus. Anorectoplasty, construction of efferent conduit, and colostomy closure were performed at 4 years of age. Ileovaginoplasty and ileovesicostomy which was a Mitrofanoff-type of conduit, and labioplasty were performed at the age of 11 years. To the best of our knowledge, only five survivors with CDS over 1 year of age have been reported.

Minimally invasive repair of hypospadiac urethral duplication.

Urethral duplication is a rare congenital anomaly with various clinical presentations, and multiple techniques have been described for its repair. We report a 1-year-old boy with hypospadiac urethral duplication who presented with double urinary stream. Voiding cystourethrography, retrograde urethrography, and cystourethroscopy showed the normal-caliber ventral urethra was dominant and the distal dorsal (non-dominant) urethra had a good caliber. Urethral reconstruction was performed with an incision of the adjoining walls of the both urethra in a side-to-side urethrourethrostomy fashion.

Radiographic signs predictive of success of hydrostatic reduction of intussusception.

AIM: Outcome of hydrostatic reduction of intussusception (HRI) was analyzed according to specific radiographic signs to improve success. METHODS: At our institution, a pediatric surgical team performs HRI using a standardized protocol. We reviewed 266 consecutive HRI performed from 1998 to 2008 according to patient demographics, symptomatology, parameters of inflammation (peak WBC, peak CRP), position of the tip of the intussuscepted bowel and an intussusception bowel ratio (IBR). RESULTS: Of the 266 cases, 250 (94%) were successful (group A) and 16 (6%) failed (group B). Average age was significantly higher in group A than in group B (14.9 +/- 12.4 vs. 8.33 +/- 3.93 months) (P < 0.01). Duration of symptoms was significantly shorter in group A than in group B (15.0 +/- 12.5 vs. 25.0 +/- 9.7 h) (P < 0.05). The position of the tip was ascending colon (Ac): A = 34 (14%), B = 1 (6%); right transverse colon (RTc): A = 112 (45%), B = 1 (6%); left transverse colon (LTc): A = 84 (34%), B = 12 (75%); descending colon (Dc): A = 15 (6%), B = 0 (%); and sigmoid colon (Sc): A = 5 (2%), B = 2 (13%). The tip was located in LTc, Dc and Sc significantly more often in group B (14/16, 88%) than group A (104/250, 42%) (P < 0.01). IBR for group B (1.68 +/- 0.47) was significantly larger than group A (1.13 +/- 0.28) (P < 0.01). Differences in parameters of inflammation were not significant. CONCLUSIONS: We found that the position of the tip and IBR are predictive of success of HRI. Having a dedicated team perform HRI using a standardized protocol with consideration of IBR and the position of the tip eliminates bias, fosters reliability and ensures reproducibility, while at the same time it allows patients with inappropriate data to be spared potentially dangerous attempted HRI.

Laparoscopy-assisted percutaneous endoscopic gastrostomy.

OBJECTIVE: Percutaneous endoscopic gastrostomy (PEG) placement is associated with considerable complications. We added laparoscopic monitoring to improve outcome. METHODS: Thirty-four patients who had laparoscopy-assisted PEG (LAP-PEG) were reviewed. A 5 mm supraumbilical trocar and two 5 mm working ports were required for LAP-PEG. A needle was placed percutaneously into the stomach under laparoscopic and gastroscopic control. A wire was placed through the needle, encircled with a snare, and the PEG completed. The anterior wall of the stomach was then anchored to the abdominal wall. RESULTS: Thirty-one subjects had cerebral palsy. Age at LAP-PEG ranged from 5 months to 25 years (mean, 8.1 years). Weight ranged from 4.7 kg to 25.9 kg (mean, 12.2 kg). In 23 patients, LAP-PEG was performed with laparoscopic Nissen fundoplication. In 11 patients, it was performed for reasons such as gastric volvulus and nutritional supplementation. Mean operating time was 67 minutes, and all procedures were performed safely without intra- or postoperative complications. CONCLUSION: LAP-PEG is our method of choice for gastrostomy because it allows the first and last parts of a conventional PEG procedure to be well controlled and safe instead of being blind.

Is intraoperative anal endosonography necessary during laparoscopy-assisted anorectoplasty for high/intermediate type imperforate anus?

PURPOSE: The aim of this study was to evaluate the relevance of anal endosonography (AES) during Georgeson laparoscopy-assisted anorectoplasty (GLA) for high/intermediate type imperforate anus (HIIA) using a continence evaluation questionnaire (CEQ) and postoperative AES (post-AES). METHODS: Fifteen HIIA patients who underwent GLA were enrolled in this prospective study. Six had intraoperative AES (op-AES) to confirm the accuracy of the positioning of the pull-through (PT) canal and 9 did not (no-AES). All subjects were reviewed prospectively by one surgeon and all had post-AES to measure the external sphincter (ES) and puborectalis (PR) at 3 and 9 o'clock to confirm that the PT colon was central. A CEQ (5 parameters: frequency of motions, severity of staining, severity of perianal erosions, anal shape, and requirement for medications; maximum score: 10) was administered to 9 subjects (4 from op-AES and 5 from no-AES) followed-up for over 3 years to assess continence. RESULTS: Mean age at GLA for both groups was not statistically different. There were no statistical differences between the two groups for thickness of the ES and PR on post-AES. Mean annual CEQ scores for the op-AES group 1, 2, 3, 4, and 5 years after GLA were 5.3, 6.3, 7.4, 8.4, and 8.5, respectively, while for the no-AES group were 5.9, 7.1, 8.1, 8.2, and 8.2, respectively; all differences were not statistically significant. CONCLUSIONS: Our results suggest that op-AES is not necessary during GLA because there is no difference in clinical and functional status that can be attributed to op-AES.