Childhood pancreatic trauma: Clinical presentation, natural history and outcome.

OBJECTIVES: To study the presentation, management strategies and long-term natural history of children with pancreatic trauma. METHODS: Children admitted with pancreatic trauma were analyzed for their presentation, management and outcome. Management included nasojejunal feeds, total parenteral nutrition (TPN), octreotide, drainage (radiological and endoscopic), endoscopic retrograde cholangiopancreatography (ERCP) and surgery. Patients were assessed in follow-up for development of chronic pancreatitis (CP). RESULTS: 36 children [29 boys, age 144 (13-194) months] presented at 30 (3-210) days after trauma. Most common cause of trauma was bicycle handle bar injury [n = 18,50%]. Presenting features were abdominal pain [n = 26,72%], lump [n = 16, 44.4%], ascites [n = 13,36%], pleural effusion [n = 9,25%] and anasarca [n = 3,8.3%]. All presented with sequelae of ductal disruption with pseudocyst, ascites or pleural effusion. Fifteen (41.6%) patients each had Grade III and IV injury, 4 (11%) had grade V, and grading was unavailable in 2. Other organs were injured in 4 (11%) cases. Management consisted of various combinations of nasojejunal feeds [n = 17,47.2%], TPN [n = 5,13.8%], octreotide [n = 13,36%], pseudocyst drainage [radiological (n = 18,50%), endoscopic (n = 3,8.3%)] and ERCP [n = 12,33.3%]. Surgical intervention was done in 2 (5.5%) cases [cystojejunostomy and peritoneal lavage in 1 each]. Two (5.5%) patients died due to sepsis. Of the 32 cases in follow-up, 19 (59.3%) recovered and 13 (40.6%) developed CP, with half (6/13) of them being symptomatic with recurrent pain. CONCLUSION: Multi-disciplinary non-operative management is effective for managing pancreatic trauma in 94.4% of children, with 75% requiring radiological or endoscopic intervention. 40% developed structural changes later but only half were symptomatic.

Rapid Eye Movement (REM) Sleep Behavior Disorder and REM Sleep with Atonia in the Young.

BACKGROUND: Rapid eye movement (REM) sleep behavior disorder (RBD) and REM sleep without atonia (RWA) have assumed much clinical importance with long-term data showing progression into neurodegenerative conditions among older adults. However, much less is known about RBD and RWA in younger populations. This study aims at comparing clinical and polysomnographic (PSG) characteristics of young patients presenting with RBD, young patients with other neurological conditions, and normal age-matched subjects. METHODS: A retrospective chart review was carried out for consecutive young patients (<25 years) presenting with clinical features of RBD; and data were compared to data from patients with epilepsy, attention deficit hyperactivity disorder (ADHD), and autism, as well as normal subjects who underwent PSG during a 2-year-period. RESULTS: Twelve patients fulfilling RBD diagnostic criteria, 22 autism patients, 10 with ADHD, 30 with epilepsy, and 14 normal subjects were included. Eight patients with autism (30%), three with ADHD (30%), one with epilepsy (3.3%), and six patients who had presented with RBD like symptoms (50%) had abnormal movements and behaviors during REM sleep. Excessive transient muscle activity and/or sustained muscle activity during REM epochs was found in all patients who had presented with RBD, in 16/22 (72%) autistic patients, 6/10 (60%) ADHD patients compared to only 6/30 (20%) patients with epilepsy and in none of the normal subjects. CONCLUSION: We observed that a large percentage of young patients with autism and ADHD and some with epilepsy demonstrate loss of REM-associated atonia and some RBD-like behaviors on polysomnography similar to young patients presenting with RBD.

Troubles du comportement en sommeil paradoxal et sommeil paradoxal sans atonie musculaire chez les jeunes. Contexte: Les troubles du comportement en sommeil paradoxal (TCSP) et le sommeil paradoxal sans atonie musculaire ont acquis une grande importance clinique. En effet, des données à long terme ont montré de quelle façon ils pouvaient progresser chez des adultes âgés atteints de maladies neurodégénératives. Toutefois, on en sait beaucoup moins au sujet des TCSP et du sommeil paradoxal sans atonie musculaire au sein des groupes d'âges plus jeunes. Cette étude entend donc comparer les caractéristiques cliniques et polysomnographiques (PSG) de jeunes patients donnant à voir des signes de TCSP à celles d'autres jeunes patients atteints d'autres troubles neurologiques et de sujets en bonne santé appariés en fonction de l'âge. Méthodes: Nous avons passé en revue de façon rétrospective les dossiers de jeunes patients (< 25 ans) donnant à voir des signes cliniques de TCSP et ayant été vus consécutivement. Les données recueillies ont été comparées aux données de patients atteints d'épilepsie, de troubles de l'attention avec hyperactivité et d'autisme ainsi qu'à celles de sujets en bonne santé soumis à des examens de PSG pendant une période de deux ans. Résultats: Au total, on a diagnostiqué chez 12 patients des TCSP. Ajoutons que 22 d'entre eux étaient atteints d'autisme alors que 10 étaient atteints de troubles de l'attention avec hyperactivité et 30 d'épilepsie. Mentionnons par ailleurs que 14 sujets en bonne santé ont été inclus dans cette étude. Après analyse, il s'est avéré que 8 patients atteints d'autisme (30 %), 3 de troubles de l'attention avec hyperactivité (30 %), 1 d'épilepsie (3,3 %) et 6 ayant donné à voir des symptômes ressemblant à ceux des TCSP (50 %) montraient des mouvements et des comportement anormaux en sommeil paradoxal. Des signes d'activité musculaire transitoire excessive et/ou d'activité musculaire durable lors d'épisodes de sommeil paradoxal ont été détectés chez tous les patients satisfaisant aux critères des TCSP, chez 16 patients autistes sur 22 (72 %), chez 6 patients atteint de troubles de l'attention avec hyperactivité sur 10 (60 %) en comparaison avec seulement 6 patients épileptiques sur 30 (20 %) et aucun parmi les sujets en bonne santé. Conclusion: Lors d'examens polysomnographiques, nous avons en définitive observé qu'une forte proportion de jeunes patients atteints d'autisme et de troubles de l'attention avec hyperactivité, ainsi que quelques-uns atteints d'épilepsie, donnaient à voir des signes de perte de sommeil paradoxal associés à l'atonie musculaire ainsi que des comportements ressemblant à ceux de jeunes patients atteints de TCSP.

An Approach to Investigations of Chronic Liver Disease.

Chronic liver disease (CLD) in children is more diverse compared to adults with respect to the etiology, progression and response to therapy. After history and clinical examination, the first step is to confirm the presence of CLD with basic blood investigations and ultrasonography. Markers of portal hypertension are splenomegaly, increased portal vein diameter, thrombocytopenia and presence of varices on endoscopy. The next step is to evaluate the etiology of CLD which will depend on the age of the child and needs targeted investigations as metabolic and inherited causes predominate in early childhood. CLD progression ought to be monitored regularly and several non-invasive markers are available but they have to be evaluated further in children. Since CLD progresses, complications have to be detected early not only to initiate appropriate treatment but also to prognosticate.

Prevalence and Precipitants of Hepatic Encephalopathy in Hospitalized Children With Chronic Liver Disease.

Objective: Hepatic encephalopathy (HE) is a marker of poor prognosis in adults with chronic liver disease (CLD). We prospectively studied the prevalence and precipitants of HE in children with CLD as there is a paucity of literature on the same. Methods: Children (1-18 years) admitted with CLD were examined daily for the presence and grading of HE (West Haven/Whittington grading). Precipitants were classified as infection, dyselectrolytemia, gastrointestinal bleeding, constipation and dehydration. Changes in grades of HE and outcome were noted. Results: One hundred and sixty children (age 120 [84-168] months) were enrolled. HE was present in 50 (31.2%) patients with a total of 61 episodes. Maximum grade of HE was grade I (n = 16), II (n = 23), III (n = 11) and IV (n = 11). Forty-two cases had single and 8 had recurrent (2-5) episodes. Median duration of HE episodes was 96 (72-192) hours. Precipitants were identified in 55/61 (90.2%) episodes with infection (45/61, 73.7%) and dyselectrolytemia (33/61, 54%) being the most common. Lower albumin and sodium, higher INR and presence of infection were significantly associated with presence of HE. Overall, HE resolved in 33 (54%) episodes, while it progressed and persisted in 28 (45.9%) episodes. Patients with HE had a poorer outcome (25/50 vs 13/110; P < 0.01) with both higher in-hospital (11/50 vs 9/110; P = 0.02) and 1-month post discharge (14/39 vs 4/101; P < 0.01) mortality than those without HE. Conclusion: One-third of admitted CLD children have HE, with identifiable precipitants in 90% of cases. Children with HE have poorer liver functions, higher rate of infections and worse outcome than those without HE.

Diagnosis and management of pediatric acute liver failure: consensus recommendations of the Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition (ISPGHAN).

Timely diagnosis and management of pediatric acute liver failure (PALF) is of paramount importance to improve survival. The Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition invited national and international experts to identify and review important management and research questions. These covered the definition, age appropriate stepwise workup for the etiology, non-invasive diagnosis and management of cerebral edema, prognostic scores, criteria for listing for liver transplantation (LT) and bridging therapies in PALF. Statements and recommendations based on evidences assessed using the modified Grading of Recommendations Assessment, Development and Evaluation (GRADE) system were developed, deliberated and critically reappraised by circulation. The final consensus recommendations along with relevant published background information are presented here. We expect that these recommendations would be followed by the pediatric and adult medical fraternity to improve the outcomes of PALF patients.

Pediatric acute viral hepatitis with atypical variants: Clinical dilemmas and natural history.

Classical acute viral hepatitis (AVH) has an uncomplicated outcome. Acute liver failure has a grave prognosis. Atypical manifestations of AVH are a group of disorders that causes significant morbidity and dilemmas in children. These include prolonged cholestasis, relapsing hepatitis, ascitic form of AVH, late-onset hepatic failure (LOHF), intravascular hemolysis, and provoking an autoimmune trigger leading to autoimmune hepatitis. These entities cause significant liver dysfunction or worsening and are often difficult to differentiate from chronic liver disease (CLD). Ascitic form of AVH, LOHF, decompensated CLD and acute-on-chronic liver failure have significant overlapping features that need to be carefully dissected out. In many cases, only on long-term follow-up, these clinical entities can be separately identified. Intravascular hemolysis is usually caused by associated glucose-6-phosphate dehydrogenase deficiency. Rarely CLD such as Wilson disease and autoimmune hepatitis can also present with hemolysis in the initial presentation, which can mimic AVH with hemolysis. Identifying deviations from typical manifestations aid in avoiding unnecessary investigations, allowing focused therapy and alleviating anxiety.

Mitochondrial hepatopathy: Anticipated difficulties in management of fatty acid oxidation defects and urea cycle defects.

Fatty acid oxidation defects (FAOD) and urea cycle defects (UCD) are among the most common metabolic liver diseases. Management of these disorders is dotted with challenges as the strategies differ based on the type and severity of the defect. In those with FAOD the cornerstone of management is avoiding hypoglycemia which in turn prevents the triggering of fatty acid oxidation. In this review, we discuss the role of carnitine supplementation, dietary interventions, newer therapies like triheptanoin, long-term treatment and approach to positive newborn screening. In UCD the general goal is to avoid excessive protein intake and indigenous protein breakdown. However, one size does not fit all and striking the right balance between avoiding hyperammonemia and preventing deficiencies of essential nutrients is a formidable task. Practical issues during the acute presentation including differential diagnosis of hyperammonemia, dietary dilemmas, the role of liver transplantation, management of the asymptomatic individual and monitoring are described in detail. A multi-disciplinary team consisting of hepatologists, metabolic specialists and dieticians is required for optimum management and improvement in quality of life for these patients.

Portal Cavernoma Cholangiopathy in Children and the Management Dilemmas.

Portal cavernoma cholangiopathy (PCC) is one of the most harrowing complications of extrahepatic portal venous obstruction, as it determines the long-term hepatobiliary outcome. Although symptomatic PCC is rare in children, asymptomatic PCC is as common as that in adults. However, there are major gaps in the literature with regard to the best imaging strategy and management modality in children. Moreover, natural history of PCC and effect of portosystemic shunt surgeries in children are unclear. Neglected PCC would lead to difficult or recalcitrant biliary strictures that will require endoscopic therapy or bilioenteric anastomosis, both of which are challenging in the presence of extensive collaterals. There are limited studies on the effect of portosystemic shunt surgeries on the outcome of PCC in children compared to adults. In this review, we aimed to collate all existing literature on PCC in childhood and also compare with adult studies. We highlight the difficulties of this disease to provide a comprehensive platform to foster further research on PCC exclusively 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.

Peritoneal Lymphomatosis Masquerading as Pyoperitoneum in a Teenage Boy.

A 16-year-old boy presented with 1 month of fever, abdominal pain, and distension. The ascitic tap drained pus-like fluid, and ultrasonography showed diffuse thickening of the omentum and mesentery with echogenic ascites. A diagnosis of pyoperitoneum due to peritoneal tuberculosis with secondary infection was suspected, and antitubercular therapy was started elsewhere, but there was no improvement. Computed tomography of the abdomen revealed enhancing soft-tissue thickening in the retroperitoneum, extending into the mesentery and encasing the superior and inferior mesenteric vessels. The ascitic fluid appearance deceptively resembled pus, but further analysis revealed atypical lymphocytes. Omental and bone marrow biopsies confirmed Burkitt lymphoma. Awareness of this rare presentation is imperative for making a correct diagnosis.