How to suspect the presence of high-risk esophageal varices and when to start endoscopic surveillance in children with biliary atresia?

BACKGROUND AND AIM: Portal hypertension determines the outcome of children with biliary atresia (BA) and is common even after a successful Kasai portoenterostomy (KPE). However, there are no clear-cut guidelines on the age of starting surveillance and the modality (endoscopy vs non-invasive tests [NITs]). In this cohort study, we analyzed our database to find out the utility of NITs in detecting high-risk esophageal varices in BA. METHODS: From June 2010 to May 2022, consecutive children of BA who underwent upper gastrointestinal (UGI) endoscopy were included. Esophageal varices were classified as high-risk (grade II with red-color signs or grade III or IV irrespective of red-color signs. NITs such as splenomegaly (clinical and USG), platelet count, aspartate transaminase to platelet ratio index (APRI), and platelet-to-spleen diameter ratio were compared between cases with high-risk and low-risk varices. RESULTS: A total of 110 children, 75 boys (66 successful KPE and 44 failed/KPE not performed) were enrolled. The median age at KPE was 85 days (IQR 63-98). Thirteen (11.8%) children presented with UGI bleeding. The first endoscopy revealed gastroesophageal varices in 75.4% of cases, and 32% of them had high-risk varices. Multivariate analysis revealed failed KPE, history of UGI bleeding, bigger spleen size (> 3.5 cm), lower platelet count (< 150 000), and higher APRI (> 2) are independent predictors of the presence of high-risk esophageal varices. CONCLUSION: Endoscopy is the best in predicting the presence of high-risk varices that might bleed; hence, early surveillance endoscopy should be started in children with splenomegaly, thrombocytopenia, and high APRI score to prevent variceal bleeding.

Assessment of Gastric Residual Volume with Ultrasound in Children at Fasting and after Oral Intake of Carbohydrate-Rich Fluid in the Preoperative Period.

Purpose: Despite standard preoperative fasting guidelines, children are subjected to prolonged fasting due to various reasons. This does not reduce gastric residual volume (GRV) further, instead causes hypoglycemia, hypovolemia, and unnecessary discomfort. We calculated the cross-sectional area (CSA) of antrum and GRV in children in fasting state and 2 h after intake of oral carbohydrate-rich fluid, using gastric ultrasound. Methods: Anteroposterior and craniocaudal gastric antral diameters were measured by ultrasonography in the right lateral decubitus position, at fasting and at 2 h after 8 ml/kg of pulp-free fruit juice ingestion. CSA of antrum and GRV was calculated using validated mathematical models. Results: Data of 149 children of age >1-12 years were analyzed. Greater than ninety-nine percent of children emptied ≥95% of the ingested pulp-free fruit juice volume within 2 h. One hundred and seven (71.8%) children had reduced CSA and GRV at 2 h after fruit juice ingestion (2.01 ± 1.00 cm2 and 7.77 ± 6.81 ml) as compared to fasting state (3.18 ± 1.40 cm2 and 11.89 ± 7.80 ml). Fourty-nine (28.2%) children had slightly increased CSA and GRV at 2 h after fruit juice (2.46 ± 1.14 cm2 and 10.61 ± 7.26 ml) than at fasting (1.89 ± 0.92 cm2 and 8.61 ± 6.75 ml), but this increased GRV was grossly lower than limit of risk stomach (26.54 ± 8.95 ml). Conclusion: Carbohydrate-rich drink in the form of pulp-free fruit juice may be safely permitted up to 2 h before anesthetic induction, as it promoted gastric emptying in ≈ 72% of children and 28% of children, although GRV was slightly higher at 2 h after fruit juice ingestion than fasting but remained considerably lower than limit of risk stomach.

Gastrointestinal Polyps and Polyposis in Children: Experience of Endoscopic and Surgical Outcomes.

INTRODUCTION: There are gaps in the literature regarding outcome of multiple polyps and dilemmas in the management issues in polyposis syndromes in children. OBJECTIVE: We aimed to study the clinical behaviour of gastrointestinal (GI) polyps with emphasis on therapeutic outcomes of polyposis syndrome. METHODS: Proven cases of GI polyp(s) on endoscopy were classified as single polyp, multiple polyps, and polyposis syndrome. Complex presentation was defined as 1 or more of the following: severe anaemia, anasarca, intussusception, rectal mucosal prolapse, and diarrhoea. A clinico-endoscopic criterion was applied in polyposis syndrome patients for the decision of surgery versus endoscopic therapy with surveillance. RESULTS: Of total 240 patients, there were no significant differences between single (52.5%, n = 126) versus multiple polyps (27.5%, n = 66) with respect to age, symptoms, histology, and recurrence. Polyposis syndrome (20%, n = 48) presented with complex symptoms (50%), higher family history, significantly lower haemoglobin, total protein, and albumin as compared to single and multiple polyps (p < 0.01). Nineteen polyposis patients with favourable clinico-endoscopic criteria were endoscopically eradicated for polyps in 3 (1-4) sessions with sustenance of laboratory parameters at 1 year and 30% symptomatic recurrence at follow-up of 23.5 (7-40) months. There were no major endoscopic complications. Nineteen patients required proctocolectomy with improvement in laboratory parameters 6 months post-surgery. CONCLUSIONS: Multiple polyps behave similar to single polyps in children. A clinico-endoscopic criterion may guide for optimal management of polyposis syndrome. Colectomy may be effectively deferred in a large proportion of polyposis syndrome patients if maintained on an endoscopic protocol.

Non-variceal gastrointestinal bleed in children: surgical experience with emphasis on management challenges.

PURPOSE: This exclusively surgical series on pediatric non-variceal gastrointestinal bleed (NVGIB) defines three levels of bleed site and describes etiology, bleed severity, diagnostic algorithm, and surgical management for each bleed site. Management challenges are detailed. METHODS: Patients aged ≤ 18 years treated surgically for NVGIB were analysed. RESULTS: Bleed site (n = 87) was classified as: upper gastrointestinal bleed (UGIB; n = 11); small bowel bleed (SBB: n = 52); and lower GIB (n = 24). Four etiology-based groups were identified: lesions with ectopic gastric mucosa (EGM; n = 33), tumours (n = 23), ulcers (n = 21), and vascular pathology (n = 8). Bleed severity spectrum was: acute severe bleed (n = 12); subacute overt bleed (n = 59); and occult GIB (n = 16). Preoperative diagnosis was obtained in all UGIB and LGIB lesions. Eighty-two percent of surgical SB lesions were diagnosed preoperatively on Tc99m pertechnetate scan, computed tomography enterography-angiography, and capsule endoscopy; remaining 18% were diagnosed at laparotomy with intra-operative enteroscopy (IOE). Surgical management was tailored to bleed site, severity, and etiology. Indications of IOE and approach to management challenges are detailed. CONCLUSIONS: The commonest site-specific bleed etiologies were duodenal ulcers for UGIB, EGM lesions for SBB, and tumours for LGIB. SBB presented diagnostic challenge. Diagnostic algorithm was tailored to bleed site, age-specific etiology, bleed severity, and associated abdominal/systemic symptoms. Management challenges were acute severe bleed, occult GIB, SBB, obscure GIB, and rare etiologies. IOE has a useful role in SBB management.

Yolk Sac Tumor of Stomach: Case Report and Review of Literature.

Gastric yolk sac tumors (YSTs) are very rare. Only seven cases of pure YST of the stomach, with a median age of 65 years, and five cases of YST combined with adenocarcinoma have been reported. We report a 3-year-old boy who presented to us with recurrent episodes of melena and anemia of 2 months' duration. Upper gastrointestinal endoscopy revealed a deeply excavated ulcer on the lesser curvature, 4 cm from the gastroesophageal junction. Computed tomography (CT) abdomen revealed large heterogeneously enhancing soft-tissue mass lesion in the lesser sac with indistinct fat planes with the lesser curvature of the stomach with multiple peripherally enhancing lesions in both lobes of liver and omentum, suggestive of metastasis. Serum alpha-fetoprotein (AFP) was 21,000 ng/ml. Tru-cut biopsy from the mass was suggestive of YST. Following three courses of chemotherapy based on Cisplatin, Etoposide and Bleomycin (PEB), sleeve gastrectomy with excision of the mass with omentectomy with biopsy of the liver nodules with a sampling of perigastric lymph nodes was done. Histopathology revealed only necrotic tissue with no evidence of residual malignancy. Three-month follow-up CT revealed no residual mass with AFP of 3 ng/ml.

Congenital Benign Intrahepatic Cyst with Cystobiliary Communication: An Intraoperative Diagnostic Enigma.

This manuscript reports a 15-month-old male presenting with a congenital solitary benign intrahepatic cyst, wherein the presence of a cyst with frank bile and a large cystobiliary communication came as an intraoperative enigma. The stepwise approach to diagnosis, management, and review of relevant literature carry a useful message.

Blood Glucose Variability and Outcomes in Critically Ill Children.

OBJECTIVES: To find the incidence of hyperglycemia (blood glucose [BG] ≥150 mg/dl), hypoglycemia (BG ≤60 mg/dl), and variability (presence of hypoglycemia and hyperglycemia) in critically ill children in the 1st week of Intensive Care Unit (ICU) stay and their association with mortality, length of ICU stay, and organ dysfunction. MATERIALS AND METHODS: The design was a retrospective observational cohort study. Consecutive children ≤18 years of age admitted from March 2003 to April 2012 in a combined adult and pediatric closed ICU. Relevant data were collected from chart review and hospital database. RESULTS: Out of 258 patients included, isolated hyperglycemia was seen in 139 (53.9%) and was unrelated to mortality and morbidity. Isolated variability in BG was noted in 76 (29.5%) patients and hypoglycemia was seen in 9 (3.5%) patients. BG variability was independently associated with multiorgan dysfunction syndrome on multivariate analysis (adjusted odds ratio [OR]: 7.1; 95% confidence interval [CI]: 1.6-31.1). Those with BG variability had longer ICU stay (11 days vs. 4 days, on log-rank test, P = 0.001). Insulin use was associated with the occurrence of variability (adjusted OR: 3.6; 95% CI: 1.8-7.0). CONCLUSION: Glucose disorders were frequently observed in critically ill children. BG variability was associated with multiorgan dysfunction and increased ICU stay.

Esophageal exclusion and bypass for corrosive injury: The lessons learnt.

While it is always preferable to excise and replace the diseased esophagus in corrosive injuries, the surgeon may be compelled to exclude and bypass it by a substernal conduit in select situations wherein excision is technically hazardous. This case illustrates the lessons learnt from a potentially life-threatening complication of bipolar esophageal exclusion.

Discrimination of pediatric cryptogenic multifocal ulcerous stenosing enteritis from small bowel Crohn's disease and gastrointestinal tuberculosis: A retrospective study (with videos).

INTRODUCTION: Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare entity that mimics various inflammatory strictures of the small intestine. Pediatric literature is scarce. We analyzed the clinical, radiological, endoscopic and histopathological features of children with CMUSE that differentiate it from small bowel Crohn's disease (SBCD) and gastrointestinal tuberculosis (GITB). METHODS: CMUSE was diagnosed by the following criteria: (1) unexplained small bowel strictures with superficial ulcers, (2) chronic/relapsing ulcers of small bowel after resection, (3) no signs of systemic inflammation, (4) absence of other known etiologies of small bowel ulcers. SBCD and GITB were diagnosed based on standard criteria. The clinical features, laboratory parameters, radioimaging, endoscopy (including video capsule endoscopy [VCE], intra-operative endoscopy), histopathological features and treatment outcome were noted. RESULTS: Out of 48, CMUSE was diagnosed in 13 (27%) isolated small bowel and ileocecal strictures, while GITB and SBCD accounted for 41% and 21% cases, respectively. Common presentations were sub-acute obstruction (46%), obscure gastrointestinal bleeding (38%) and protein-losing enteropathy (38%). CMUSE patients had significantly longer disease duration compared to SBCD and GITB (p < 0.001). SBCD (90.0%) and GITB (85%) cases had elevated C-reactive protein (CRP), none with CMUSE had elevated CRP (p < 0.001). The disease was localized in jejunum (100%) and proximal ileum (56%) in CMUSE, ileocecal region (85%) in GITB, but evenly distributed in small intestine in SBCD. Endoscopy showed evenly placed, superficial, circumferential ulcers with strictures in CMUSE, deep linear ulcers in SBCD and circumferential ulcers in GITB. Upfront immunosuppression was given in four; three (75%) of them relapsed. Only surgery was done in three with one (25%) having relapse. Upfront surgery followed by immunosuppression was used in six, but all relapsed and two required repeat surgery. CONCLUSION: CMUSE is important but underdiagnosed in children. Lack of constitutional symptoms, normal inflammatory parameters and characteristic ulcers with strictures helped in differentiating CMUSE from GITB and SBCD.

Variations in biliary ductal and hepatic vascular anatomy and their relevance to the surgical management of choledochal cysts.

PURPOSE: An aberrant biliary ductal and vascular anatomy presents a technical challenge for choledochal cyst (CDC) surgery. Mismanagement may have unfavourable implications. This study highlights the spectrum, approach to their identification and management. METHODS: Forty of 117 (34 %) cases were identified to have an aberrant biliary ductal (n = 17) or arterial (n = 26) anatomy; 3 had both. The pancreaticobiliary anatomy was defined by an intraoperative cholangiogram (IOC) before January 2005 and a preoperative magnetic resonance cholangiopancreatogram (MRCP) subsequently. RESULTS: IOC missed 3 of 4 aberrant biliary ducts, while an MRCP accurately delineated 10 of 13 aberrant bile ducts. The significant biliary anomalies were: an aberrant right sectoral/segmental duct joining the common hepatic duct (CHD) or the cyst itself (n = 14), cystic duct (n = 1) and cystic duct-CHD junction (n = 1). The aberrant duct was incorporated into the biliary-enteric anastomosis (B-EA) by: (i) double ostia B-EA (n = 1), (ii) ductoplasty with single ostium B-EA for aberrant duct and CHD (n = 2), and (iii) transection of the CHD/cyst distal to the aberrant duct orifice with a single ostium B-EA (n = 13). The arterial anomalies were (i) replaced or accessory right hepatic artery (RHA) (n = 11) and (ii) RHA crossing anterior to the cyst (n = 15), which was repositioned posterior to the B-EA. CONCLUSION: It is important to consciously look for, appropriately identify and manage aberrant biliovascular anatomy. MRCP facilitates accurate preoperative delineation of aberrant duct anatomy. All major aberrant ducts need to be incorporated into the B-EA and aberrant arteries should not be ligated.

Achalasia cardia sub-types in children: Does it affect the response to therapy?

BACKGROUND: Achalasia sub-types affect treatment response in adults, but there is no similar data in children. We studied the differences in clinico-laboratory features and response to therapy between achalasia sub-types in children. METHODS: Forty-eight children (boys:girls-25:23, 14 [0.9-18] years) with achalasia (clinical, barium, high-resolution manometry [HRM], gastroscopy) were evaluated. The sub-type was determined by Chicago classification at HRM. Pneumatic dilatation (PD) or surgery was the primary therapy. Success was defined as Eckhardt score of ≤ 3. RESULTS: Dysphagia (95.8%) and regurgitation (93.8%) were the most common symptoms. Forty of 48 cases had an adequate HRM study: Type I (n-19), II (n-19) and III (n-2). Types I and II had similar clinical profile. Type II had higher basal lower esophageal sphincter (LES) pressure (30.5 [16.5-46] vs. 22.5 [13-43] mmHg; p = 0.007) and less dilated esophagus on timed barium esophagogram (TBE, 25 [13-57] vs. 34.5 [20-81] mm; p = 0.006) than type I. Both types had similar success (86.6% [13/15] vs. 92.8% [13/14]; p = 1) after first PD and need of post-PD myotomy (5/17 vs. 1/16; p = 0.1) in follow-up. Twenty-three cases had TBE before and after PD; 15 (65.2%) had good clearance. These subjects required myotomy (1/15 vs. 4/8; p = 0.03) and repeat PD (5/15 vs. 4/8; p = 0.08) less often than those with poor clearance on TBE. CONCLUSION: Types I and II achalasia have similar frequency and clinical profile. Type II has higher LES pressure and less dilated esophagus than Type I. Both respond equally well to initial PD. Type I required post-PD myotomy more often, though not significantly. TBE is useful for assessing therapeutic response.

Portosystemic Shunt Surgery for Extrahepatic Portal Venous Obstruction Beyond Endoscopic Variceal Eradication: Two Decades of Pediatric Surgical Experience.

Background: This exclusively surgical series on pediatric extrahepatic portal venous obstruction (EHPVO) defines surgical indications beyond endoscopic eradication of esophageal varices (EEEV), the selection of an appropriate surgical procedure, and the long-term post-surgical outcome. Methods: EHPVO management protocol at the reporting institute has been endotherapy until esophageal variceal eradication and surgery for select adverse sequelae manifesting after EEEV. Results: One hundred and thirty-nine EHPVO cases underwent surgery for the following indications in combination: i) massive splenomegaly with severe hypersplenism (n = 132, 95%); ii) growth retardation (GR, n = 95, 68%); iii) isolated gastric (IGV) and ectopic varices (n = 49, 35%); iv) Portal cavernoma cholangiopathy (PCC) (n = 07, 5%). A portosystemic shunt (PSS) was performed in 119 (86%) cases. Types of PSS performed were as follows: central end-to-side splenorenal shunt with splenectomy (n = 104); side-to-side splenorenal shunt (n = 4); mesocaval shunt (n = 1); inferior mesenteric vein (IMV) to left renal vein shunt (n = 2); IMV to inferior vena cava shunt (n = 3); H-graft interposition splenorenal shunt (n = 1); spleno-adrenal shunt (n = 3); makeshift shunt (n = 1). Esophagogastric devascularization (n = 20, 14%) was opted for only for non-shuntable anatomy. At a median follow-up (FU) of 41 (range: 6-228) months, PSS block was detected in 13 (11%) cases, with recurrent variceal bleeding in 4 cases. PCC-related cholestasis regressed in 5 of 7 cases. Issues of splenomegaly were resolved, and growth z-scores improved significantly. Conclusions: Endotherapy for secondary prophylaxis until EEEV has resulted in a shift in surgical indications for EHPVO. Beyond EEEV, surgery was indicated predominantly for non-variceal sequelae, namely massive splenomegaly with severe hypersplenism, GR, and PCC. Varices warranted surgery infrequently but more often from sites less amenable to endotherapy, i.e., IGV and ectopic varices. The selection of PSS was tailored to anatomy and surgical indications. On long-term FU post surgery, PSS block was detected in 13% of patients. PCC-related cholestasis regressed in 71%, and issues of splenomegaly resolved with significantly improved growth Z scores.

Shear Wave Elastography: A Reliable Secondary Parameter for Diagnosing Biliary Atresia in Infants With Neonatal Cholestasis.

Objective In this study, we aimed to optimize various grayscale, Doppler, and elastography parameters and evaluate their diagnostic performance in the preoperative diagnosis of biliary atresia (BA). Materials and methods A total of 158 infants aged <6 months with neonatal cholestasis (NC) were enrolled in the study and sonography was performed after four hours of fasting. For comparison of elastography, 31 exclusively age-matched controls, not suffering from liver disease, were included separately. Triangular cord and gallbladder (GB) parameters were considered as primary parameters, while right hepatic artery (RHA) caliber, RHA-to-right portal vein (RPV) ratio, hepatic subcapsular flow (HSF), and shear wave elastography (SWE) were considered as secondary parameters. Diagnosis of infants with BA was confirmed on histopathology. Data were presented as mean ±standard deviation (SD) and frequency. Differences between groups were compared using the Chi-square test and the unpaired student t-test. Receiver operating characteristic (ROC) curve analysis was done for individual ultrasound/Doppler/SWE parameters to calculate the optimal cutoff value. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy were calculated for each parameter and their combinations. Results Of the primary parameters, GB contractility index (CI) and length showed the highest sensitivity and specificity respectively. A cutoff of 14 kPA was derived for SWE for the diagnosis of BA. Among secondary parameters, SWE had the best diagnostic performance, better than even the individual primary parameters. A combination of primary parameters with SWE in series showed the highest accuracy. Conclusion Among secondary parameters, elastography can prove to be highly useful. The highest accuracy in diagnosing BA can be obtained by combining primary parameters with SWE.

Identification of high-risk group and therapeutic options in children with liver abscess.

The outcome of children with liver abscess (LA) depends upon prompt diagnosis and intervention. We evaluated the etiology, clinical profile, various interventional modalities of management and outcome of children with LA. A total of 39 hospitalized children (mean age 7.2 ± 3.9 years) with radiologically proven LA were analyzed. Parenteral antibiotics, percutaneous drainage (PD) or open surgical drainage (OSD) was done as required. Cases with ruptured or impending rupture of LA, upper gastrointestinal bleed, jaundice, pleural effusion or consolidation were labeled as "high risk" cases. Triad of fever, pain and hepatomegaly was the most common presentation. Single abscess was present in 66.7% and right lobe was involved in 69.2% of cases. Majority of LA were pyogenic (PLA, 25/39). Amebic liver abscess (ALA) and PLA had similar clinical and laboratory profile except that multiloculated abscess on ultrasonography was a feature of PLA (12/25 vs. 0/11; p = 0.006). Cases with ALA settled significantly more often with antibiotics alone (5/11 vs. 3/25; p = 0.04) than PLA and none required surgery (0/11 vs. 7/25; p = 0.03). Subjects with "high-risk" LA (n - 26) had significantly larger abscesses, more polymorphonuclear leucocytosis (74 ± 15% vs. 61 ± 13%; p = 0.01) in peripheral blood and need of drainage (24/26 vs. 7/13; p = 0.03) than patients with average-risk LA. Based on the results, 38/39 children recovered, with complete abscess resolution in 28, over 48 ± 63.8 days. In conclusion, ALA, although similar in presentation, are uniloculated, and patients with ALA recover more often without drainage than patients with PLA. Patients with "high risk" LA are more common and have a good outcome with drainage. PD, being safe, efficacious and less invasive than OSD, should be the preferred drainage procedure.

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.

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.

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, H 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 H-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.

Outcome of portosystemic shunt surgery on pre-existing cholangiopathy in children with extrahepatic portal vein obstruction.

BACKGROUND: This study was performed to assess the effect of portosystemic shunt surgery (PSS) on portal cavernoma cholangiopathy (PCC) in children with extrahepatic portal vein obstruction (EHPVO). METHODS: Children with EHPVO and PCC (unfit for Meso-Rex shunt) underwent magnetic resonance cholangiogram (MRC) and magnetic resonance portovenogram (MRPV) before non-selective PSS. PCC was graded by modified Llop classification. Those with patent shunt were re-evaluated at least 6 months after surgery with MRC, MRPV and compared with pre-shunt images. RESULTS: Twenty-five children underwent PSS (central end to side splenorenal shunt with splenectomy [n = 24], mesocaval shunt [n = 1]). Pre-surgery MRC showed PCC grade I in 11, grade II in 1 and grade III in 13. MRPV showed superior mesenteric vein (SMV) block in 20. Re-assessment for PCC 18 (6 to 54) months after surgery showed grade I in 6 and grade III in 19. Thus, PCC was progressive in 6 and static in 19. Density of peribiliary collaterals decreased in 5 (SMV patent, static PCC), increased in 3 and remained unchanged in 17. Splenomegaly-related problems, gastroesophageal varices and other intra-abdominal (esophageal, perisplenic and perigastric) collaterals ameliorated in all. CONCLUSION: Non-selective PSS decompresses esophago-gastro-splenic venous circuit effectively but fails to ameliorate cholangiopathy and peribiliary collaterals. Persistence of cholangiopathy is attributable to SMV block.