Prednisolone administration monitored by postoperative stool color achieves high jaundice clearance after laparoscopic portoenterostomy for biliary atresia.

PURPOSE: Stool color (SC) for monitoring prednisolone use in biliary atresia (BA) patients after laparoscopic portoenterostomy (LPE) was reviewed. METHODS: Subjects were 47 post-LPE BA patients given a reducing dose course of intravenous prednisolone. The course started at 4 mg/kg/day and gradually reduced, ultimately reaching a final total dose (TD) of 31.5 mg/kg. Normal SC indicated a course could progress until finished and was repeated until jaundice clearance (JC) was achieved. Abnormal SC persisting for two consecutive courses was the absolute indication for redo or liver transplantation (LTx). RESULTS: JC was achieved in 38/47 (80.9%) LPE cases and 4/6 redos to give an overall JC rate (JCR) of 42/47 (89.4%). Outcomes after one course (n = 5; JCR: 80.0%; median TD: 30.0 mg/kg, interquartile range [IQR: 26.0-31.5]), two courses (n = 10; JCR: 90.0%; median TD: 62.5 mg/kg [IQR: 60.8-66.0]), three courses (n = 13; JCR: 92.3%; median TD: 90.0 mg/kg [IQR: 86.0-90.0]), four courses (n = 10; JCR: 80.0%; median TD: 120.0 mg/kg [IQR: 116.7-123.3]), five courses (n = 7; JCR: 100%; median TD: 156.0 mg/kg [IQR: 154.3-157.5]), six courses (n = 1; JCR: 100%; TD: 189.0 mg/kg), ten courses (n = 1; JCR: 100%; TD: 308 mg/kg). CONCLUSION: Indications for repeat prednisolone and timing of redo/LTx based on SC monitoring appeared effective based on high JCR and successful redo/LTx. LEVELS OF EVIDENCE: III.

Choledochal cyst.

Choledochal cyst (CC) or congenital biliary dilatation, has a skewed distribution with hereditary features that is far more common in East Asian females. CC is usually associated with pancreaticobiliary malunion (PBMU) forming a common channel. CC requires early definitive diagnosis, since there is a risk for malignancy occurring in the CC and/or intrahepatic bile ducts (IHBD). Complete CC excision and Roux-en-Y hepaticoenterostomy is required and can be performed by open or minimally invasive surgery with hepatojejunostomy the recommended procedure of choice. Principles of open surgical intervention form the basis of minimally invasive management with laparoscopy and robotic assistance. Current surgical management is associated with fewer early and late complications, such as hepaticoenterostomy anastomotic leakage, cholangitis, anastomosis stricture, and cholangiocarcinoma. Specific features of CC management at Juntendo include: intraoperative endoscopy of the common channel and IHBD for inspecting and clearing debris to significantly reduce post-operative pancreatitis or stone formation; near infra-red fluorescence with indocyanine green for visualizing tissue planes especially during minimally invasive surgery for CC; and a classification system for CC based on PBMU that overcomes inconsistencies between existing classification systems and clinical presentation.

Biochemical Evaluation of Laparoscopic Portoenterostomy for Treating Biliary Atresia and Redo for Failed Portoenterostomy.

Background: Postoperative outcomes of portoenterostomy (PE) and redo-PE were evaluated using selected biochemical markers (SBM) and biochemical status categories (BSC). Methods: Subjects were 70 consecutive PE performed for biliary atresia. SBM were aspartate aminotransferase (AST)/alanine aminotransferase (ALT), cholinesterase (ChE), and platelet count (PLT) assessed at 1, 2, 3, 6, and 12 months, and thence, annually for a maximum of 10 years. BSC were as follows: all SBM normal (N-SBM), normal AST/ALT (N-SLT), normal ChE (N-ChE), normal PC (N-PLT), all abnormal (A-SBM), abnormal AST/ALT (A-SLT), abnormal ChE (A-ChE), and abnormal PC (A-PLT). Subjects achieving jaundice clearance (JC) and surviving with native livers (SNL) also had gamma glutamyl transpeptidase assessed. Redo-PE indicated for failed PE was assessed postoperatively using the same SBM/BSC protocol. Results: PE were laparoscopic (LPE; n = 40) or open (OPE; n = 30). Mean age/weight at PE and duration of follow-up were similar. For JC, LPE = 34/40 (85.0%) and OPE = 22/30 (73.3%); P = .23. For SNL, LPE = 29/40 (72.5%) and OPE = 16/30 (53.3%); P = .10. LPE and OPE were similar for SBM/BSC, except for a single significant increase in ALT in OPE at 6 months. Redo-PE was performed 17-180 days (mean 67.1 days) after primary PE. AST was significantly increased at the last preredo assessment 3 months after primary PE; P < .05. After redo, AST decreased and SBM/BSC results were equivalent to nonredo subjects. Conclusion: Postoperative biochemical data for all PE cases were comparable; redo-PE would appear to be viable for restoring SBM, and AST could be valuable as a single marker of deterioration in redo cases.

Laparoscopic portoenterostomy for biliary atresia: single-center experience and review of literatures.

AIM: The aim of this report was to present the laparoscopic portoenterostomy (LapPE) procedure developed by the Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine (JLapPE). We also attempted to obtain an understanding of the current status of laparoscopic portoenterostomy in the world as reported in the English literature to compare with our experience. METHODS: There were 22 BA patients who had JLapPE between 2009 and 2016. BA classification was type III (n = 19) and type II (n = 3). There was 1 case of syndromic BA and 1 case was positive for cytomegalovirus. A systematic search in PubMed of all BA patients treated by LapPE in the English literature was conducted. Jaundice clearance (JC) and survival with the native liver (SNL) were compared. RESULTS: Mean age at JLapPE was 67.1 days (range 29-119). Mean postoperative follow-up was 4.6 years (1.3-8.3). Mean operative time was 514 min (240-662) and mean blood loss was 13.4 g (3-21). Postoperative JC (Total bilirubin ≤ 1.5 mg/dL) was 77.3% (17/22) at 3 months and 90.9% (20/22) at 6 months. SNL at 6 months of age was 90.9% (20/22); at 1 year of age was 77.3% (17/22), at 2 years of age was 73.7% (14/19); and at 3 years of age was 81.3% (13/16). CONCLUSIONS: Despite recent reports that outcome of LapPE for BA may be unfavorable compared with the conventional open portoenterostomy, our results would suggest that JLapPE can be performed successfully, because it is performed exactingly according to a standard protocol. JLapPE will continue to be our procedure of choice for treating BA.

Classification of biliary atresia in the laparoscopic era.

PURPOSE: According to Kasai's classification of biliary atresia (BA), type III is diagnosed when micro bile ducts (MBD) cannot be identified macroscopically on the surface of the biliary remnant transected at the porta hepatis. However, during laparoscopic Kasai (lapKasai), magnification produced by a 30° 10 mm scope at a focal length of 5 cm is ×38 and ×100 when zooming, enabling more MBD to be identified than with the naked eye rendering Kasai's original classification questionable in the laparoscopic era. METHODS: Intraoperative video recordings of 36 consecutive lapKasai cases (2009-2015) were reviewed to confirm MBD visibility. 85 consecutive open Kasai cases examined macroscopically served as controls. RESULTS: MBD were not visible under regular laparoscopic magnification during lapKasai in 6/36 (lapMBD-; 16.7 %) cases and visible in 30/36 (83.3 %). However, in open cases, MBD could not be identified macroscopically in 77/85 (macroMBD-; 90.6 %), a typical result reported internationally. For our lapKasai cases, jaundice clearance was lower in lapMBD-cases (4/6 = 66.7 % versus 26/30 = 86.7 %), which was not statistically significant (p = 0.26).  Conversely, survival with the native liver was significantly lower in lapMBD-cases (4/6 = 66.7 % versus 23/30 = 76.7 %) (p < 0.05). CONCLUSIONS: BA classification may benefit from revision to include laparoscopic findings to categorize BA more comprehensively.

Comprehensive assessment of prognosis after laparoscopic portoenterostomy for biliary atresia.

PURPOSE: Total bilirubin (T-bil) is used universally for monitoring post-portoenterostomy (PE) biliary atresia (BA) patients although other biochemical markers [BM; AST/ALT and platelet count (PC)] are also prognostic. We compared open PE (OPE) with laparoscopic PE (LPE) using T-bil, AST/ALT, and PC (3BM) as more comprehensive indicators of postoperative clinical status. METHODS: Subjects were 31 PE cases (LPE: n = 17; OPE: n = 14). BA classification was type III (n = 16), type II (n = 1) in LPE and type III (n = 12), type I (n = 1), type II (n = 1) in OPE. RESULTS: Mean ages and weights at PE were similar: 65.5 days, 4.4 kg (LPE) versus 69.3 days, 4.1 kg (OPE); and mean follow-up was 2.5 years for both LPE and OPE. Jaundice clearance (T-bil ≤1.2 mg/dL) was achieved in 16/17 (94.1 %) after LPE versus 10/14 (71.4 %) after OPE (p = NS), but 3BM were closer to normal after OPE. At the time of review, 13/17 LPE cases (76.5 %) were alive with native livers and 4/17 had received LTx (23.5 %) and 10/14 OPE cases (71.4 %) were alive with native livers and 4/14 had received LTx (28.6 %). CONCLUSIONS: Although JC was better after LPE, 3BM were better after OPE. Further follow-up will prove the comprehensive prognostic value of 3BM.

Modified Snodgrass tubularized incised plate urethroplasty prevents fistula formation in hypospadias.

Urethrocutaneous fistula is a common complication after redo urethroplasty for hypospadias, or urethroplasty for patients with thin urethral plate. We modified Snodgrass' tubularized incised plate urethroplasty (STIPU) by stripping the most superficial skin from both sides of the U-shaped incision to expose more subcutaneous tissue along the suture line. Our modified STIPU would appear to be effective in preventing postoperative urethrocutaneous fistula formation in redo urethroplasty for hypospadias, and urethroplasty for patients with thin urethral plate.

Laparoscopic surgery for biliary atresia and choledochal cyst.

Minimally invasive surgery in children has evolved to the extent that complex procedures can be performed with safety and outcome comparable with open surgery, with the advantage of minimal scarring. Here we describe the latest laparoscopic techniques used by us at the Juntendo University Hospital, Japan, for treating biliary atresia and choledochal malformation, with presentation of our postoperative management and discussion of preliminary outcomes.