Childhood portal hypertension in Jamaica

Portal hypertension and bleeding from oesophageal varices in children remain a difficult medical problem. The clinical course and management of children with portal hypertension seen over a 14-year period was reviewed. There were 5 females and 2 males with a mean age of 3.6 years at presentation. Five patients presented with severe upper gastrointestinal bleeding and two with severe hypersplenism. All patients had extra-hepatic portal hypertension. Five patients were treated with endoscopic sclerotherapy, including one who had bleeding five years post-splenectomy. A mean of 9 sclerotherapy sessions was performed in each patient. Complete obliteration of varices was not achived in any patient and a single rebleeding episode occurred in four. Three children underwent operative management consisting of splenectomy in two and splenectomy and central spleno-renal shunt in one. There was no mortality in either group after a mean follow-up of 4.3 years. Sclerotherapy may not be totally successful in long-term management of childhood portal hypertension. Surgical therapy or a combination of sclerotherapy and surgery may be the best approach

Outcome of solid renal tumours in children at the University Hospital of the West Indies

Of the twenty-nine children with solid tumours treated at the University Hospital of the West Indies (UHWI) between January, 1972 and December, 1991, there were twenty-eight cases of nephroblastoma and one of mesoblastic nephroma. Peak incidence was between the ages of two and four years. Twenty-five children had radical nephrectomy while one had bilateral partial nephrectomy. In thirteen cases, pre-operative chemotherapy and radiotherapy was used. Post-operative chemotherapy and radiotherapy were used in 24 and 13 cases, respectively. Stage of the tumour was the most decisive factor influencing the outcome. Whereas there was 100 per cent cure rate in Stages I and II, Stage III had only a 55.5 per cent survival rate and non of the Stage IV survived. Bilateral (Stage V) tumours are curable if individual tumours are localised, as in one of the two cases. The benign mesoblastic nephroma, in a one-month-old infant, was cured by nephrectomy alone. While the present therapy of radical nephrectomy along with combination chemotherapy is satisfactory for early stages, more aggressive adjuvant therapy is needed for improving the results in Stages III and IV

Prognosis of neuroblastic tumours in children

A retrospective analysis of 26 children with neuroblastic tumours treated at the University Hospital of the West Indies (UHWI) between 1970 and 1991 was undertaken to evaluate factors affecting prognosis. The peak incidence was between 48 months and 60 months of age, and 75//of the deaths occurred in children older than 3 years. The abdomen was the most common site of the tumours 79//of which were from the adrenal gland and carried a dismal outcome. Other sites were thoracic, pelvic and cervical. Extra-adrenal tumours have a good outcome even when the histology is unfavourable; 92//of the tumours were in Stages III or IV at the time of initial presentation. Stage IV disease accounted for all but one of the deaths. Of the 8 children with favourable histology (ganglioneuroma or well-differentiated ganglioneuroblastoma), only one (12.5//) died, whereas of the remaining 18 cases with unfavourable histology (neuroblastoma and undifferentiated ganglioneuroblastoma), eleven (61//) died. When managed by surgery alone or with adjuvant chemo- and/or radio-therapy, only 36.4//succumbed while all 4 children with chemotherapy only died. Ten children are alive without disease for more than three years post-therapy. A scoring system was designed which takes into account the factors influencing the outcome in neuroblastic tumours, namely, age, location, stage and histological types of the tumours and therapy. All the children with a score of 21 or less survived, whereas all those with scores of 22 or above succumbed. This underlines the multifactorial influences on the final outcome of neuroblastic tumours

The rapunzel syndrome: report of a case and review of the literature

The Rapunzel Syndrome, a rare manifestation of trichobezoar, occurs when bolus gastrointestinal obstruction is produced by an unusual trichobezoar with a long tail that extends to or beyond the ileocaecal valve. A five-year-old Jamaican girl presented with this abnormality and was found at laparotomy also to have an ileo-ileal intussuception. For the Rapunzel Syndrome, we recommended bezoar extraction at laparotomy via multiple enterotomies. In addition, psychiatric evaluation and therapy is essential due to the commonly associated finding of underlying emotional stress.

Inguinal hernia in children

Two hundred and seventy one cases of inguinal hernias and hydrocoels in children treated at the University Hospital of the West Indies over a five-year period have been reviewed. The dominance of the boys and the right side is borne out. The younger infant has the highest risk of incarceration which is more common in boys and in right-sided hernias. Females with inguinal hernias were screened for testiculaar feminization and none was found. There were three girls with hydeocoels of the Canal of Nuck. The incidence of metachronous presentations of contralateral hernia occurred only in 6.6 por ciento of cases. When routine explorartion of the contralateral side was undertaken in 5.2 por ciento of cases, a hernia sac was found only in less than half of them. These findings support the present policy of not routinely exploring the opposite side in unilateral inguinal hernias. Herniotomy was adequate for 92.6 por ciento of cases. Herniorrhaphy was seen in only one case. One testicular atrophy occurred following incarceration. Eighty-six per cent of these children were managed as day cases. Inpatient management was required when the hernia was complicated or when there were associated other medical conditions.