Richa Lal

Histopathological features and accuracy for diagnosing biliary atresia by prelaparotomy liver biopsy in developing countries

Background and Aim:  A major challenge in neonatal cholestasis (NC) is to differentiate biliary atresia (BA) from other non‐atretic causes. In developing countries there are considerable problems of late referral of NC cases and performing surgery without prelaparotomy liver biopsy that contributes to a high proportion of negative laparotomy and increased morbidity. We evaluated the hepatic histopathology for presence of features that correlate best with the diagnosis of BA and assessed the accuracy of percutaneous liver biopsy.

Encephalopathy assessment in children with extra-hepatic portal vein obstruction with MR, psychometry and critical flicker frequency

BACKGROUND & AIMS Mild cognitive and psychomotor deficit has been reported in patients with extra-hepatic portal vein obstruction. This prospective study was done to ascertain the presence of minimal hepatic encephalopathy by neuropsychological testing and its correlation with diffusion tensor imaging derived metrics, T1 signal intensity, brain metabolites in (1)H magnetic resonance spectroscopy, blood ammonia and critical flicker frequency in patients with extra-hepatic portal vein obstruction. METHODS Neuropsychological tests, critical flicker frequency, blood ammonia, diffusion tensor imaging, T1 signal intensity and (1)H magnetic resonance spectroscopy were determined in 22 extra-hepatic portal vein obstruction and 17 healthy children. Bonferroni multiple comparison post hoc analysis was done to compare controls with patient groups. RESULTS Based on neuropsychological tests, 7/22 patients had minimal hepatic encephalopathy, and significantly increased Glx/Cr ratio, blood ammonia, mean diffusivity and globus pallidus T1 signal intensity with decreased critical flicker frequency in comparison to controls and in those without minimal hepatic encephalopathy. Cho/Cr, mI/Cr ratio and fractional anisotropy were unchanged in patient groups compared to controls. A significant inverse correlation of neuropsychological test with mean diffusivity, Glx/Cr ratio and blood ammonia and a positive correlation among mean diffusivity, blood ammonia and Glx/Cr ratio was seen. CONCLUSIONS Extra-hepatic portal vein obstruction is a true hyperammonia model with porto-systemic shunting and normal liver functions that results in minimal hepatic encephalopathy in one-third of these children. Hyperammonia results in generalized low grade cerebral edema and cognitive decline as evidenced by increased Glx/Cr ratio, mean diffusivity values and abnormal neuropsychological tests.

Endoscopic treatment of tracheoesophageal fistula using electrocautery and the Nd:YAG laser

BACKGROUND Endoscopy is a well-established means of diagnosis in recurrent and congenital H-type tracheoesophageal fistulas. There is only limited knowledge of its role in the treatment using either electrocautery or laser. Laser application has not yet been reported in the treatment of recurrent tracheoesophageal fistula. METHODS From 1993 to 1997 five patients with tracheoesophageal fistula (two congenital H-type and three recurrent) were treated endoscopically using a rigid ventilating bronchoscope. The final diagnosis was made on bronchoscopic examination. In two of the patients with recurrent fistulas and one with congenital fistula, the mucosal lining was fulgurated with electrocautery via an insulated wire. In one patient each with recurrent and congenital fistula, the mucosal lining was vaporized with the Nd:YAG laser via a 600 microm bare quartz fiber. RESULTS Obliteration of the fistula was achieved in both the patients in whom laser was used, but it was unsuccessful in two of the three children in whom electrocautery was used. The obliquity of the congenital H-type fistula renders it more amenable to obliteration compared with the short and direct recurrent fistula. There was significant respiratory distress in the postoperative period after use of electrocautery. However, it was uneventful with the use of laser. CONCLUSIONS Endoscopic treatment of recurrent and congenital H-type tracheoesophageal fistula is technically easier than open surgical procedures and helps avoid the risks associated with the latter. The Nd:YAG laser is qualitatively better than electrocautery for the obliteration of the fistula.

Management of childhood pancreatic disorders: a multidisciplinary approach.

Introduction Data on therapeutic endoscopy and radiologic interventions for the management of childhood pancreatic disorders are relatively limited. This study focuses on the multidisciplinary approach to the management of pancreatitis in children. Patients and Methods Children with pancreatic disorders were studied from January 1992 to May 2001. Acute pancreatitis (AP) was diagnosed by clinical evaluation, serum amylase more than three times normal, and morphologic abnormalities of the pancreas on imaging. Children with recurrent abdominal pain, pancreatic calcification or ductal stones on imaging, and pancreatic ductal changes on endoscopic retrograde cholangiopancreatography (ERCP) were diagnosed with chronic pancreatitis (CP). Patients were treated by gastroenterologists, surgeons, and interventional radiologists. Pancreatic exocrine insufficiency was diagnosed in appropriate settings. Results Fifteen children—6 with AP (posttrauma, 3; gallstone disease, 1; and viral, 1), 7 with CP, and 2 with pancreatic exocrine insufficiency—were diagnosed. Local complications observed in children with AP included pseudocyst in three, and infected acute fluid collection, right-sided pleural effusion, and ascites in one patient each. Complications of AP were managed with percutaneous catheter drainage (n = 3; pseudocyst, 2; infected fluid collection, 1), additional pancreatic duct stenting (n = 2), surgical drainage (n = 1), and octreotide for pleural effusion (n = 1). Signs of CP included abdominal pain (n = 7), obstructive jaundice resulting from lower common bile duct stricture (n = 2), and bleeding from gastroduodenal artery pseudoaneurysm (n = 1). Pancreatic duct stenting relieved pain in one patient, and steel coil embolization arrested bleeding from the pseudoaneurysm. Common bile duct strictures were managed by surgical bypass (n = 2), one of which required preoperative endoscopic bile duct stenting for management of cholangitis. Two other patients with CP required no intervention. Conclusion A multidisciplinary approach of radiologic and endoscopic interventions and surgery are complimentary to each other in achieving successful outcomes of complicated childhood pancreatitis.

Quality of life in children managed for extrahepatic portal venous obstruction.

Objectives: There are no studies on health-related quality of life (HRQOL) in children with extrahepatic portal venous obstruction (EHPVO). The present study evaluated the QOL in children with EHPVO, prevariceal and postvariceal esophageal variceal eradication, and postsurgery in comparison with healthy controls. Methods: Children with EHPVO and variceal bleeding were divided into 3 groups: group A, before variceal eradication (n = 50); group B, after variceal eradication (n = 50); and group C, after surgery (n = 12). Group D comprised healthy children (n = 50). Clinical details and investigations were recorded. The Pediatric Quality of Life Inventory parent-proxy HRQOL questionnaire was used for assessment of QOL. Results: Compared with controls, patients with EHPVO in groups A, B, and C had lower median QOL scores in physical, emotional, social, and school functioning health domains. Esophageal variceal eradication had no significant effect on QOL (median total QOL score pre- and postvariceal eradication of 87.5 vs 86.3). Increasing size of spleen (mild 92.5, moderate 88.2, and severe 76.2; P < 0.001), presence of hypersplenism (90 vs 73.7, P = 0.001), and growth retardation (90 vs 82.5, P = 0.04) caused significant reduction of the total QOL score. On multivariate regression analysis, splenic size and growth retardation were found to be independent predictors that affect the QOL. After surgery, a trend toward improvement in physical, psychosocial, and total QOL scores was present, but it was not significant. Conclusions: Children with EHPVO have a poor QOL that is not affected by variceal eradication. Splenomegaly and growth retardation significantly affect the HRQOL. A trend toward improvement of QOL scores is observed in the postsurgery group.

Fulguration of posterior urethral valves using the Nd:YAG laser

Abstract Endoscopic management is the accepted form of treatment for posterior urethral valves (PUV) . The Nd:YAG laser has been in clinical use for many decades, but has been used only sporadically for ablating PUV. In this study, PUV were diagnosed by micturating cystourethrogram (MCU). In the 9-month period beginning March 1997, 23 boys 3 months to 9 years of age underwent endoscopic Nd:YAG laser surgery for PUV using a 600 quartz bare fibre through the catheter channel of an 8.5 F cystoscope. The laser power and total energy delivered ranged from 20 to 50 W and 50–1085 J, respectively. Type I PUV were present in 20 boys; 1 had type III, and in 1 boy each there were residual valves and a urethral stricture from a previous electrocautery ablation. All operations were done with the patient under general anaesthesia supplemented by caudal morphine. Routine postoperative cystoscopy was done in the first 5 cases only. All patients had a good stream of urine without bleeding or any other complication after valve ablation. Mucosal tags seen on follow-up cystoscopy in 5 patients were fulgurated by laser. In 4 other patients recystoscopy was required for deterioration of the urinary stream and persistent posterior urethral dilatation on MCU; residual valves were refulgurated by laser. An indwelling catheter was left in only 4 patients, 2 of whom were primarily operated and 2 following refulguration. All except the first 2 were treated as out-patients. Application of the Nd:YAG laser has added a new dimension for treating PUV. The initial experience has been very encouraging: the destruction of valves is precise, there are no complications, and it can be practised on a day-care basis without indwelling catheterisation. The bare fibre can be used repeatedly, thus reducing the cost of treatment.

Effect of surgical portosystemic shunt on prevalence of minimal hepatic encephalopathy in children with extrahepatic portal venous obstruction: assessment by magnetic resonance imaging and psychometry.

Objective: The aim of this study was to evaluate the effect of surgical portosystemic shunt (PSS) on the prevalence of minimal hepatic encephalopathy (MHE) in patients with extrahepatic portal venous obstruction. Patients and Methods: Forty-two children with extrahepatic portal venous obstruction (17 with surgical PSS, 25 without surgical shunt) and 20 healthy children were evaluated with blood ammonia (BA), psychometry, 1H magnetic resonance spectroscopy, critical flicker frequency (CFF), and diffusion tensor imaging. Serum tumor necrosis factor-α and interleukin-6 were measured in 10 patients and 8 controls. Results: Patients with surgical PSS had significantly higher BA and glutamine/creatine on 1H-MR spectroscopy than those without surgical shunt. Both groups of patients had significantly higher BA and glutamate/creatine than controls. Myoinositol was reduced in patients with surgical PSS. MHE was present in 41% cases with and 32% cases without surgical PSS (p-ns). Raised mean diffusivity on diffusion tensor imaging signifying low-grade cerebral edema was seen only in MHE cases. Patients had significantly higher serum tumor necrosis factor-α and interleukin-6 levels than controls. CFF was abnormal in 5 of 15 patients with MHE. Conclusions: Patients with surgical PSS have significantly higher BA and Glx/creatine than those without surgical PSS. MHE prevalence was higher in surgically shunted than in the nonshunted patients, but the difference was not significant. Cerebral edema is present in patients with MHE. CFF has limited diagnostic utility for MHE in children.

Management of Complicated Choledochal Cysts

Objectives: The presentation of choledochal cysts (CDCs) is altered by complications such as acute severe cholangitis, hepatolithiasis, spontaneous perforation, portal hypertension, pancreatitis, malignancy of the biliary tract and previous surgery in the form of internal drainage of the cyst. The management and the outcome of such complicated cysts differ from that of an uncomplicated CDC. This presentation is an analysis of our experience with complicated CDCs. Patients and Methods: One hundred and forty-four patients with CDCs were managed at a tertiary level referral hospital in North India, between January 1989 and June 2004. Thirty-three (23%) CDCs were associated with complicating factors: severe cholangitis requiring a biliary drainage procedure prior to definitive operative procedure (n = 11), spontaneous perforation (n = 3), hepatolithiasis (n = 6), acute cholecystitis (n = 1), recurrent acute pancreatitis (n = 1), chronic pancreatitis (n = 1), gastric outlet obstruction (n = 1), portal hypertension (n = 6), biliary tract malignancy (n = 4) and previous internal drainage of the cyst (n = 5). Five patients had more than one complication. The management outcome of complicated CDCs was compared with that of uncomplicated CDCs. Results: Complicated CDCs were significantly more common with type IV-A anatomy. Endoscopic, percutaneous or external surgical biliary drainage procedure was performed in 14 complicated CDCs prior to cyst excision. Desired definitive surgical procedure could be performed in 26/33 (79%) patients with complicated CDCs as compared to 107/111 (96%) patients with uncomplicated CDCs. Four (12%) patients with complicated CDCs had early postoperative complications as compared to 9/111 (8%) patients with uncomplicated CDCs. During a median follow-up of 13 months (2 months to 8 years), 2 patients with complicated CDCs died due to advanced secondary biliary cirrhosis and advanced gall bladder cancer, respectively. Six patients reported complications. Of the 6 patients, 3 required reoperation for a strictured hepaticojejunostomy (n = 2) and hepatolithiasis (n = 1). In contrast, there was no disease-related mortality and only 1 out of 111 patients with uncomplicated cysts had a complication during follow-up. Conclusion: Complicated CDCs merit a carefully planned management strategy including percutaneous, endoscopic and surgical procedures. The management may have to be staged. The outcome depends on the nature of complication and the management strategy adopted.

Urinary continence following posterior urethral valves treatment

This retrospective study discusses the incidence of symptomatic voiding dysfunction, the etiological factors and the changes with age and with increasing duration of follow up in 67 boys treated for posterior urethral valves and followed up for a period ranging from 1–21 years. Twenty three of the 67 patients (34.3%) had symptomatic voiding dysfunction; the commonest symptom being nocturnal enuresis with diurnal urgency and frequency. Data in this study suggests a definite decrease in the incidence of urinary incontinence as the patient grows older or as the duration of follow up after valve fulguration increases. A Tc-99m DRCG based uroflowmetry was available in 31 of the 67 patients at the time of this review and evidence of detrusor dysfunction was identified in 9 of the 31 patients on the basis of uroflowmetry. Of significance was the detection of detrusor dysfunction on uroflowmetry in 3 patients with a history of normal urinary control and urinary stream with persistent post treatment hydroureteronephrosis eventually culminating into renal failure.Imipramine therapy was given to 10 of the 17 patients with symptoms of urinary frequency, urgency and nocturnal enuresis with a significant symptomatic improvement in 9 of these patients.Urinary incontinence, detrusor dysfunction and its effect on the upper tracts and renal function is a significant determinant of the long term outcome of the boys treated for posterior urethral valves and this paper emphasises on the need to carefully evaluate and treat this aspect of all valve patients.

Bladder-neck repair in urinary bladder exstrophy.

Abstract A simple modification of an existing technique for bladder-neck reconstruction in exstrophy of the urinary bladder is reported. The technique involves tubularization of the posterior urethra up to just below the ureteric orifices. It differs from other techniques in that no part of the bladder tissue is used for buttressing the repair, but all is utilized for enhancing the bladder volume. Only 2 of 20 patients remained incontinent after bladder-neck reconstruction; the remaining 18 have achieved socially acceptable continence.