Hypertrophic pyloric stenosis in a newborn with propionic acidaemia

Hypertrophic pyloric stenosis is exceedingly rare in the first week of life. This report describes a rare case of congenital pyloric stenosis in a newborn with propionic acidaemia. Although newborns with propionic acidaemia may present with feeding problems in the form of vomiting, associated congenital pyloric stenosis should be considered in the differential diagnosis. These patients should be investigated with abdominal ultrasound and or barium meal and once diagnosed, they should undergo pyloromyotomy

Hypertrophic pyloric stenosis in a newborn with propionic acidaemia

Hypertrophic pyloric stenosis is exceedingly rare in the first week of life. This report describes a rare case of congenital pyloric stenosis in a newborn with propionic acidaemia. Although newborns with propionic acidaemia may present with feeding problems in the form of vomiting, associated congenital pyloric stenosis should be considered in the differential diagnosis. These patients should be investigated with abdominal ultrasound and or barium meal and once diagnosed they should undergo pyloromyotomy

Unusual Mesoblastic nephroma: a report of two cases

Congenital mesoblastic nephroma [CMN] is a very rare tumour of early infancy with 80% of the cases being diagnosed within the first month of life. Complete surgical excision is almost always curative. We report two unusual large renal tumours in a 22-month-old boy and a 4-year-old girl who were diagnosed preoperatively as rhabdoid tumour and Wilms' tumour respectively. One of them was subjected unnecessarily to preoperative chemotherapy. Histological evaluation after complete radical excision demonstrated CMN

Spontaneous central venous catheter fracture and the intracardiac migration in an infant: Successful percutaneous retrieval

The use of central venous lines including totally implantable venous devices has revolutionized the care and management of infants and children in general and oncology patients in particular and, although considered to be generally safe, they are however associated with significant morbidity. One of the rare but potentially life-threatening complications is catheter fracture and migration, which is commonly seen in adults but rarely in children. We report here the case of an infant with catheter fracture and migration to the right ventricle, which was successfully retrieved by a percutaneous transfemoral endovascular technique

Congenital duodenal diaphragm in eight children

Congenital duodenal obstruction [CDO] is a common and usually easy to diagnose cause of intestinal obstruction in the newborn, except when the cause of the obstruction is a duodenal diaphragm. We describe our experience with eight children who had intrinsic duodenal obstruction secondary to a duodenal diaphragm. The medical records of 22 children with the diagnosis of congenital intrinsic duodenal obstruction were reviewed for age at diagnosis, sex, gestation, birth weight, clinical features, associated anomalies, method of diagnosis, treatment and outcome. Operative findings and procedures were obtained from the operative notes. Eight of the 22 children [36.4%] had congenital duodenal diaphragm [CDD]. In all children, the diagnosis was made from plain abdominal X-ray, which showed the classic double-bubble appearance, and barium meal, which showed duodenal obstruction. Four patients had associated anomalies, including two with Down's syndrome. Intraoperatively, five patients were found to have duodenal diaphragm with a central hole, while the other three had complete duodenal diaphragms. Postoperatively, all patients did well. Six required total parenteral nutrition. Conclusions: The 100% survival rate among these children is comparable to that in Western countries, and can be attributed to the lack of major associated abnormalities, good perioperative management, and the availability of total parenteral nutrition

Neuroblastoma - the Tawam Hospital experience

Over the past two decades, there have been significant improvements in the survival of children with different types of cancer. Neuroblastoma is the exception, as in spite of many different kinds of chemotherapeutic regimens, the prognosis of advanced neuroblastoma remains unsatisfactory. This study is an analysis of our experience in the management of 53 children [25 males and 28 females] with neuroblastoma in this part of the world. Their ages at presentation ranged from birth to 10 years [mean 2.9 years]. The majority of our patients [83%] had intra-abdominal neuroblastoma. Five had intrathoracic neuroblastoma, 2 had intracranial neuroblastoma, 1 had oropharyngeal neuroblastoma, and one had metastatic neuroblastoma without a known primary. The distribution of our patients according to stage was as follows: 3 stage I, 5 stage II, 9 stage III, 33 stage IV and 3 stage IV-S. All our patients with stage I, II, III, and IV-S survived. A large number of our patients [62.3%] presented with advanced stage IV neuroblastoma, and this contributed to the high mortality rate [34%] in our series. The exact incidence of neuroblastoma in the United Arab Emirates [UAE] is not known, but it accounted for 7.5% of the total number of malignancies in the paediatric age group. The recently established cancer registry in the UAE should prove useful regarding the incidence and prevalence of cancer in the future