Amir Azmy

Kasabach-Merritt syndrome

Eight infants with hemangiomata associated with severe consumption coagulopathy are reviewed; three died. The presentations and method of management are discussed. The varied response to different modalities of therapy initiated this report and led to a search of the world literature in an attempt to identify the pattern of response, if any, to particular therapy. From our own experience with two cases and from the review of the literature, it appears that radiotherapy alone or in combination with steroids gives a superior overall treatment in terms of reducing the death rate.

Complete duplication of the hindgut and lower urinary tract with diphallus

An infant is reported with complete duplication of the colon, rectum, anus, terminal ileum up to the point of Meckels diverticulum, doubling of the genitalia with completely formed penes, double bladder and urethra, multiple spinal anomalies, omphalocele, and large lower abdominal wall hernia with wide separation of the symphysis pubis.

The long-term outcome of the endoscopic subureteric implantation of polydimethylsiloxane for treating vesico-ureteric reflux in children: a retrospective analysis of the first 195 consecutive patients in two European centres.

To determine the long‐term efficacy of endoscopically implanting polydimethylsiloxane (PDS, Macroplastique®, Uroplasty BV, Geleen, the Netherlands) for treating vesico‐ureteric reflux (VUR) in children.

Chromosomal translocation in mesenchymal hamartoma of liver: what is its significance?

Mesenchymal hamartoma of the liver is generally considered to be benign in nature, and surgical excision has been the treatment of choice. There have been 3 reports on the association of cytogenetic abnormalities with this tumor, and the possibility of malignant potential has been raised. The authors report a fourth case with a previously unreported complex chromosomal translocation between chromosomes 11, 17, and 19 at bands q12, p11, and q13.3, respectively in all cells examined and discuss the significance of this finding.

A review of thermal injuries in young children

A group of 472 children under 3 yr of age were treated for thermal injuries in a 5-yr period; 65% were boys. Injuries were due mainly to scalding (82%) and involved predominantly the upper part of the body. Full thickness skin loss resulted in 30% of cases, and skin grafting was needed in 29%. Forty-two episodes of infection arose, and 50 patients (11%) developed other complications--hyperpyrexia, respiratory problems, fluid imbalance and convulsions were the commonest. The mortality rate was 1% (4 cases) and the mean duration of stay in hospital was 17 days. The plan of management is outlined with emphasis on the essential differences in treatment of young children, and the results are reported.

Neonatal tumours: a single-centre experience

Abstract.Solid tumours are uncommon in the neonatal period. We present our experience of managing neonatal tumours in a tertiary reference centre to study the incidence, pathology and types, efficacy of treatment, and impact of antenatal diagnosis on the management in our practice in a retrospective study of case-notes and pathology reports. Eighty-three neonates with solid tumours were seen over a 45-year period (1955–1999); 62 (74%) presented at birth. Only 11 were diagnosed antenatally. Teratomas were the commonest type (n = 33, 40%) followed by neuroblastomas (NB) (14), renal (13), soft-tissue (10), hepatic (4), and miscellaneous tumours (2). Twenty-three (28%) were malignant, 50% of these being NBs. Surgery remains the mainstay of treatment. Chemotherapy has also become safer. Therapeutic complications were responsible for 50% of deaths before 1986; from 1986 onwards, there has been no therapy-related mortality. Only one-third of the recent cases were diagnosed antenatally. Counseling the family and in-utero transfer is the best option. In our limited series, there was no significant difference in management and outcome in the antenatally-diagnosed cases. The small numbers of neonatal tumours seen by individual centres underline the need for an international effort to optimise therapy and improve understanding of these tumours.

Collagen-coated Vicryl mesh: A new bioprosthesis in pediatric surgical practice

Collagen-coated Vicryl mesh (C.C.V.M.) was used in 28 children who needed repair of thoracic and abdominal wall defects. Herein the authors report the clinical experience, surgical technique, and outcome in these cases.

Thoracic cage deformity: A late complication following repair of an agenesis of diaphragm

Abstract A thoracic cage deformity is reported as a long-term complication following survival of a child with a unilateral agenesis of diaphragm and repair of the defect with a patch of lyophilized dura.

Oxidized cellulose haemostat mimicking a possible recurrence of neuroblastoma.

A 2-year-old boy presented with a large right upper quadrant mass; initial investigations (ultrasonography and CT) showed a heterogeneous mass in the right adrenal gland, which was con®rmed to be a neuroblastoma on needle biopsy and by positive tumour markers. After chemotherapy, the tumour shrank and was predominantly of low attenuation on CT (Fig. 1). At surgery the tumour was completely resected and the kidney preserved. Oxidized regenerated cellulose (Surgicel@, Ethicon, Somerville, NJ, USA) was used to achieve haemostasis. Routine CT 4 months later showed a 2-cm low-attenuation mass at the site of the resected tumour (Fig. 2). The appearance was suspicious of tumour recurrence and at laparotomy the mass was removed. Histology con®rmed that the mass was retained Surgicel and there was no evidence of tumour recurrence.

Angiomyolipoma causing life-threatening hematuria in a child with tuberous sclerosis.

A case of a 12-year-old boy with tuberous sclerosis who developed life-threatening hematuria is presented. An angiomyolipoma of the lower two thirds of the left kidney resulted in a tumor hemorrhage that was successfully treated by partial nephrectomy.