Magnetic resonance imaging of the Brain in children with non neurological Wilson's disease

Wilson's disease [WD], an autosomal recessive disorder of copper metabolism characterized by diminished biliary excretion of copper and its tissue accumulation, is a multisystem disease dominated by hepatic and/ or neurological symptoms. The choice of first line drug therapy for WD depends on the extent and site of affection [whether hepatic or neurologic]. MR imaging is a useful modality for assessing cerebral involvement in Wilson's disease. The purpose of this study is to find out if there are detectable brain MRI lesions in patients with purely hepatic or asymptomatic forms of WD. Magnetic resonance [MR] examination of 24 patients with WD was done. Eleven patients had isolated hepatic involvement, six were asymptomatic [relatives of patients] and seven patients had neurological symptoms [neurological form of the disease]. Abnormal MR findings of the brain were found in 70.5% [12/17] of neurologically free patients, in the form of T2 hyperintensities in globus pallidus, midbrain, putamen or pons in 64.7%, 52.9%, 35.2% and 0.058% Respectively. Cortical loss [of mild degree] was revealed in 58.8% and atrophy of cerebellar hemispheres was evident in 11.7%. None of the hepatic or presymptomatic cases showed caudate affection [a finding present in 42.8% of neurological patients]. Brain changes are present in non neurological Wilson's patients even in presymptomatic ones, MR follow up images of these patients are recommended to see if these changes are reversible in the course of chronic chelation therapy

Laparoscopic management of chronic intermittent gastric volvulus in children

The aim of the study was to review the infants who presented with chronic intermittent gastric volvulus in the past 5 years, analyze pitfalls in the diagnosis and define the role of laparoscopy in the evaluation and treatment of this condition. Gastric volvulus [GV] is an abnormal rotation of the stomach around its axis.[1] It has traditionally been considered a rare entity in children, and standard texts on paediatrics typically make scant reference to it.[2] In our experience, however, a detailed medical history and a careful radiographic study of children with digestive symptoms reveals gastric volvulus to be more frequent than is commonly thought. We performed this study on all children treated for chronic intermittent gastric volvulus who presented to the Department of Pediatric Surgery, Ain Shams University School of Medicine since 2001. All the patients [10 boys, 5 girls] were term infants, mean age 11 months at diagnosis. The principal symptoms were upper abdominal pain, colic, distension and non-bilious vomiting. The mean age at onset of symptoms was 2 months. Diagnosis was in all cases on the basis of upper intestinal transit studies. All patients underwent conservative treatment; 7 of these patients showed no significant improvement and thus underwent surgery. We performed simple laparoscopic anterior gastropexy in 3 patients and laparoscopic gastrostomy in four. All patients showed good recovery after surgery. Careful examination of patients with gastrointestinal problems, and even repeated respiratory infections may reveal chronic gastric volvulus with greater frequency than has traditionally been thought. We believe that this entity is often undetected, and is often inappropriately treated. We also showed that laparoscopy is feasible, safe and efficient in the management of these cases

Clinical implications of abdominal ultrasound in early onset neonatal sepsis

Abdominal sonography may be of great value in the evaluation and early detection of sequel of neonatal sepsis. A significant number of neonates develop clinical signs compatible with necrotizing enterocolitis, cholecystitis but with inconclusive plain radiographs, which may result in delayed, inappropriate or unnecessary therapy. Therefore, this prospective study is an attempt to assess the abdominal ultrasonographic abnormalities in neonatal sepsis, correlate it with the clinical findings and evaluate its impact on the early diagnosis of sequel of neonatal sepsis. This study was conducted on 56 consecutive neonates [36 males and 20 females] with sepsis admitted to the NICU of the Obstetrics and Gynecology Department, Ain Shams University Hospitals. Their mean gestational age was 34.0 +/- 4.5 weeks; their mean birth weight was 2.2 +/- 0.530kg. All patients were subjected to history taking, clinical evaluation, laboratory investigations and radiological evaluation, including plain chest and abdominal x-rays as well as abdominal ultrasonographic examination. The study showed that 59% of patients had hepatomegaly, 25% had splenomegaly, and 7% had ascites. Specific findings of necrotizing enterocolitis [NEC] were detected in 14 patients [25%], while gall bladder abnormalities per se were detected in 57% of septic patients. Mixed abdominal ultrasonographic findings were recorded in 20 cases [35.71%]. Specific findings of NEC included pneumatosis intestinalis [17.9%], thickened intestinal wall [25%] and air in portal vein [7.14%]. As regards gall bladder affection the following sonographic abnormalities were detected: thickened gall bladder wall [28.5%], biliary sludge / mud [17.9%], pneumobilia [7.14%] and hydrops [3.57%]. Risk factors for developing NEC included sepsis, prematurity, total parentral nutrition [TPN], umbilical vein catheterization and birth asphyxia. The most frequent risk factors for gall bladder affection were sepsis, nothing per [NPO], TPN, blood or blood products transfusion as well as mechanical ventilation. In conclusion, NEC, gall bladder affection and hepatosplenomegaly are complications of neonatal sepsis in NICU and could be detected early by ultrasound examination prior to plain radiography. Abdominal ultrasound is a simple, quick, easy and cost effective method, therefore it is recommended to be used for the evaluation and follow up of septic infants in NICU