Postsplenectomy course in homozygous sickle cell disease

OBJECTIVE: To determine whether children with homozygous sickle cell (SS) disease and splenectomy are at greater risk of death, overwhelming septicemia, or other complications. METHODS: A total of 130 patients with SS treated by splenectomy (46 recurrent acute splenic sequestration, 84 chronic hypersplenism) over a 22.5-year period at the Sickle Cell Clinic of the University Hospital of the West Indies, Kingston, Jamaica, were compared with a control group matched for sex, age, and duration of follow-up in a retrospective review. Deaths and bacteremias were examined over the whole study period. Painful crises, acute chest syndromes, and febrile episodes were compared in the 90 patients completing 5 years of postsplenectomy follow-up. FINDINGS: Mortality and bacteremic episodes did not differ between the splenectomy and control groups. Painful crises were more common in the splenectomy group than in the control group (P = .01) but did not differ between splenectomy indications. Acute chest syndrome was more common in the splenectomy group than in the control group (P < .01) and was more common in the acute splenic sequestration group than in the hypersplenism group (P = .01). Febrile events did not differ between the groups or between the indications for splenectomy. CONCLUSION: Splenectomy does not increase the risk of death or bacteremic illness in patients with SS disease and, if otherwise indicated, should not be deferred for these reasons (Au)

Dumping syndrome in a young Jamaican child

The dumping syndrome in childhood is an uncommon complication of gastro-oesophageal surgery, principally Nissen fundoplication. A Jamaican child developed the syndrome after fundoplication and pyloroplasty to relieve gastro-oesophageal reflux complicating the repair of a congenital tracheo-oesophageal fistula. He developed marasmus and failed to gain weight on the standard remedial milk-based high energy diet. An oral glucose tolerance test confirmed the diagnosis of dumping syndrome. A low sugar low milk diet based on adult type meals with continous nibbling of fried dumplings relieved his diarrhoea and hypoglycaemia and he gained weight. This is a cheaper and more practical dietary therapy than the regimens described previously(AU)

Hydrostatic reduction of acute intussusception: a prospective study

Data were collected prospectively on 57 Jamaican children presenting with 62 episodes of acute intussusception over a two year period, for whom operative and hydrostatic methods of reduction were employed. 31 (54 percent) of 57 episodes were reduced successfully using barium (42), saline (11) and air (4) hydrostatically. Among the 31 other episodes, 15 had ileo-colic intussusception, seven caeco-colic, six ileo-ileo-colic and one ileo-ileal. Two patients had spontaneous reduction discovered at surgery. There were two episodes of barium hydrostatic perforation of the colon leading to death in one patient. Hydrostatic reduction is recommended as the first therapeutic option for acute intussusception because it spares the patient a major operative procedure when successful(AU)

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 (AU)

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

Trichuriasis: spontaneous and stimulated Th1/Th2 cytokine production by isolated T-cells - abstract

Although trichuriasis is the most prevalent known infection in the CARICOM countries, only a small proportion of infected children acquire intense infection, i.e., Trichuris Dysentry Syndrome (TDS). Hypotheses to be investigated are that there is a specific T-Cell anergy to the parasite in such hosts or that they have general predisposition to the "TH2" T-helper-cell response, which would be in keeping with the TDS local anaphylactic response we have shown by various techniques in our other reports. We investigated 3 groups of children: controls from a surgical private practice unlikely to have ever been significantly infected TDS cases. We used the ELISPOT technique with primary and secondary monoclonal antibodies to show cytokine production by cells separated from peripheral blood or colonic mucosal biopsies, expressing the final result in numbers of positive cells per 100,000 CD3+ cells (T-cells). Interferon-gamma detection was taken as indicative of the TH1 phenotype and Interleukin 4 (IL4) as indicative of TH2. Spontaneous production in short-term culture was compared with that after stimulation ionophore, staphylococcal enterotoxin B (superantigen) or T. trichiura excretory-secretory (ES) antigen. There was great variability in proportions of cytokine positive cells after culture, both spontaneously and after stimulation. There was also variability in the rank of potency of a given stimulant to "the T-cells of different children. ES antigen provoked the greatest IL4 response for one active TDS case, refuting the anergy hypothesis. The active cases had two highest productions (spontaneous) of interferon-gamma and (stimulated) of IL4. No significant difference in Th1/Th2 phenotypic profile emerged between the control and ex-TDS groups. We conclude that it is not innate character in T-helper-cell response to mitogen or antigen that accounts for predisposition to intense trichuriasis (AU)

Sixty-two episodes of acute intussusception: a Jamaican prospective study - abstract

Data were collected prospectively on 57 patients presenting with 62 episodes of acute intussusception between September, 1992 and August, 1994. Both operative and hydrostatic methods (barium, air, saline) of reduction were used. Fifty-seven episodes were subject to hydrostatic reduction, 31 of them being reduced successfully, a success rate of 54 percent. Barium, saline and air were employed in 42, 11 and 4 cases, respectively. There were 2 cases of barium colonic perforation leading to mortality in 1. Among 31 cases not having successful hydrostatic reduction, 15 had ileocolic intussusception, 7 had caecocolic, 6 had ileo-ileo-colic, 1 had ileoileal and 2 were found spontaneously reduced at surgery. We believe that hydrostatic methods should be a first treatment option for acute intussusception in third world institutions. Our study shows that satisfactory rates of reduction can be achieved outside of the first world with the main advantages relating to cost and patient morbidity (AU)

Acceleration in linear growth after splenectomy for hypersplenism in homozygous sickle cell disease

Chronic hypersplenism in homozygous sickle cell (SS) disease markedly increases haemolysis and the resulting erythropoietic expansion is likely to have a high metabolic cost. Splenectomy for hypersplenism in SS disease is followed by highly significant changes in haematological indices and an increase in height, but not weight, velocity. This pattern is similar to that observed in the trichuris dysentery syndrome after treatment, and differs from the increase in both height and weight velocity that follow nutritional supplementation of severely malnourished children. It is postulated that accelerated linear growth after the reduction in erythropoietic stress may implicate a specific nutrient deficiency in hypersplenic children with SS (AU)

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 percent of the deaths occurred in children older than 3 years. The abdomen was the most common site of the tumours 79 percent 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 percent 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 percent) died, whereas of the remaining 18 cases with unfavourable histology (neuroblastoma and undifferentiated ganglioneuroblastoma), eleven (61 percent) died. When managed by surgery alone or with adjuvant chemo- and/or radio-therapy, only 36.4 percent 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 (AU)

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. (AU)

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.

Gallstone-related morbidity in Jamaican children with homozygous sickle-cell disease: a six-year review

The clinical details of 17 patients aged 18 years and under with homozygous sickle-cell disease (SS) who presented with gallstone-related symptoms to the University Hospital of the West Indies, and the Bustamante Hospital for Children are analysed. All patients had right upper quadrant signs pre-operatively and all were confirmed to have acute or chronic cholecystitis based on gallbladder (GB) histology. Other gallstone-related complications were obstructive jaundice in 2, mucocoele of the GB in 1 and GB empyema in 1. While 16 of 17 had gallstones detected preoperatively ultrasound, only 2 of 6 patients later confirmed to have common bile duct (CBD) stones, were also detected. One patient had retained CBD stones post-operatively which were successfully dislodged by saline irrigation via t-tube. There was no mortality but 3 patients developed the acute chest syndrome, 4, unexplained pyrexia and 1, wound abscess. We believe that gallstone-related morbidity in children with SS disease may so far be underestimated. Even the youngest develop life-threatening complications. The risk of associated choledocholithiasis and obstructive complications may be just as high in paediatric SS disease patients with gallstones as in adults similarly affected (AU)

Acute intussusception at the University Hospital of the West Indies: a six year review

Acute intussusception is the leading cause of intestinal obstruction in Jamaican infants. All children with this diagnosis seen at the University Hospital of the West Indies over a six year period 1986 - 1991 were reviewed. The objective being to determine the success of present management practice which employs barium enema hydrostatic reduction in clinically stable patients without peritonitis. Of the thirty-eight patients reviewed there was a male preponderance of 4.5:1. Ages ranged between four months and seven years with 71 percent less than one year old. 65 percent of patients presented with a history of less than 24 hours duration. The single fatality presented 30 hours after onset of symptoms. Common symptoms and signs were vomiting 89 percent, rectal bleeding 75 percent, abdominal mass 58 percent and abdominal pain 52 percent of cases. Barium enema was successful in 91 percent of cases attempted. The study vindicates a management approach which relies on clinical assessment as the only factor determining choice of treatment and suggests that barium enema reduction employed on this basis is a safe option. (AU)

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 percent cure rate in Stages I and II, Stage III had only a 55.5 percent 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 (AU)

Common bile duct stones in sickle-cell disease - abstract

Common bile duct (CBD) stones were found intra-operatively in 12 patients (11 SS, 1 SC) among 36 patients with homozygous sickle-cell (ss) disease and 2 patients with sickle-cell haemoglobin C (SC) disease subjected to cholecystectomy between January, 1985 and December, 1992. Pre-operatively, ultrasonography had identified common bile duct stones in only 5 of these 12 patients. Operative cholangiography was diagnostic in 11 and equivocal in one. Following exploration of the common bile duct, immediate closure was performed in one, transduodenal sphincteroplasty in one, insertion of a T-tube in 4, and choledochoduodenostomy (CDD) in 6. Retained CBD stones were detected in 2 patients on routine T-tube cholangiography performed on the 10th postoperative day, and in both were successfully dislodged by saline irrigation. The high frequency of CBD stones in this symptomatic group of patients receiving cholecystectomy justifies routine diagnostic operative cholangiography. Saline irrigation intra-operatively may successfully remove stones, reducing the risk of iatrogenic damage by ductal instrumentation. Marked ductal dilatation with calculi can be treated successfully by CDD (AU)

The management of perforated necrotizing enterocolitis - abstract

Fourteen patients with perforated, necrotizing enterocolitis (NEC) managed at the University Hospital of the West Indies are reviewed. Seven patients had peritoneal drains sited under local anaesthesia as a resuscitative measure in preparation for laparotomy. Six demised, five within 24 hours of siting drains and one soon after laparotomy. The single survivor after peritoneal drainage responded well enough not to require laparotomy. Four patients had immediate exploration, leading to death in one. Two patients had localized perforation, resulting in abscess formation. Both were drained but were complicated by intestinal stricture. Of these two patients, one survived. Perforated NEC was diagnosed at post-mortem in one patient. We conclude that a high index of suspicion is required for the early detection of NEC perforation. Peritoneal drains have limited application in this condition: only in low birth weight and in unstable, perforated neonates under 1,500 gm. Intestinal resection and stoma formation is the recommended operative approach but primary anastomosis is justified for fit, well-resuscitated neonates (AU)

Solid renal tumours in children at the University Hospital of the West Indies - abstract

Between January 1972 and December 1991, twenty-nine children with solid renal tumours were treated at the UHWI. Twenty-eight of these were nephroblastomas and one was a mesoblastic nephroma. Peak incidence was between the ages of two and four years. Abdominal distension, mass and pain were the common presenting features. A palpable mass was present in all, which could be confirmed by intravenous urogram and/or ultrasound to be of renal origin. Chest X-ray revealed metastic lesions in six cases. Twenty-five children had radical nephrectomy while one had bilateral partial nephrectomy. In thirteen cases, pre-operative chemotherapy ñ radiotherapy was used. Post-operative chemotherapy and radiotherapy were used in twenty-four and thirteen cases, respectively. Stage of the tumour was the most decisive factor influencing the outcome. Whereas there was a 100 per cent cure rate in Stages I and II, Stage III had only 55.5 per cent survival rate and none of the Stage IV survived. The histology or size of the tumour were not critical factors. While the present therapy of radical nephrectomy along with the combination chemotherapy is unsatisfactory for early stages, more aggressive chemotherapy and improved radiotherapy are needed for improving the results in Stages III and IV. Bilateral (Stage V) tumours are curable if individual tumours are localised, as in one of two cases. The benign mesoblastic nephroma, in a one-month old infant, was cured by nephrectomy alone (AU)

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 percent of cases. When routine explorartion of the contralateral side was undertaken in 5.2 percent 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 percent 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. (AU)