Newton D Duncan

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.

CT appearances of the Rapunzel syndrome: an unusual form of bezoar and gastrointestinal obstruction

Abstract The CT appearances of an unusual type of trichobezoar which causes the Rapunzel syndrome are described. This syndrome is characterized by intestinal obstruction secondary to a bezoar possessing a tail which extends to or beyond the ileo-cecal valve. Most cases of bezoar are diagnosed with plain films or barium meal, but CT may be requested in patients who present with abdominal masses. The Rapunzel syndrome may be diagnosed with a combination of the CT findings and the history.

Laparoscopic cholecystectomy for chronic cholecystitis in Jamaican patients with sickle cell disease: preliminary experience

Laparoscopic cholecystectomy, with its advantages of reduced postoperative pain and shorter hospitalization is the accepted standard of care for patients with symptomatic cholelithiasis. A retrospective study was done to assess the outcome of laparoscopic cholecystectomy in patients with sickle cell disease, a group known for its high postoperative morbidity. The study sample comprised of patients seen at the University Hospital of the West Indies during the period 1999 to 2004. Twelve patients were females and four were males. Their mean age was 28.5 years (range 13-43 years). Fifteen underwent elective cholecystectomy for recurrent episodes of cholecystitis while one patient required an emergency procedure. All patients underwent endoscopic retrograde cholangiopancreatography, which successfully removed common bile duct stones which were present in 25% of the cases. There were four conversions to open cholecystectomy as a result of obscure anatomy due to scarring and adhesions. The duration of surgery ranged from 70-150 minutes. Six patients developed postoperative complications, four of whom had acute chest syndrome. This resulted in death in one patient. The mean postoperative hospitalization period was 5.5 days. This report indicates that patients with sickle cell disease remain a high risk group with the potential for significant morbidity even when subjected to minimal access surgery.

Urethral prolapse in girls

Urethral prolapse is a benign lesion of the terminal urethra, usually seen in young Negroid girls. Twenty-seven children with urethral prolapse treated at the University Hospital of the West Indies between January 1982 and December 1991 were prospectively studied; 74% were between the ages of 3 and 7 years. Bloody discharge was the most common symptom. Vulval bleeding together with a bruised appearance of the urethra resulted in erroneous initial suspicion of sexual abuse in 3 children. Prolapse was graded from I to IV depending on the extent of prolapse and degree of inflammation. Initial therapy in the 25 prepubertal girls consisted of sitz baths and topical application of either an antimicrobial ointment (15 girls) or 0.5% oestrogen cream (10), chosen randomly. Complete resolution of the prolapse occurred in only 4 children, 3 of them from the oestrogen group. Although the oestrogen led to a better response, it also caused theliarchy in 2 girls. Three children who had only transient improvement and 2 post-pubertal patients had surgical excision of the prolapse. Post-excision there were no complications or recurrence of prolapse. Three girls who had improvement with non-operative therapy continued to have grade II prolapse even after puberty. Initial therapy of urethral prolapse in prepubertal girls can be non-surgical, however, when this fails to achieve resolution surgical excision is simple, safe, and curative.

Cholecystectomy in Jamaican children with homozygous sickle-cell disease.

Twenty-seven children aged 18 years and under with homozygous sickle-cell disease had open cholecystectomy for symptomatic gallstones over the 12-year period 1985–1997. Emergency procedures (done during period of acute exacerbation of symptoms) were performed on 16 patients. Four with haemoglobin levels greater than 1 g/dl below their steady state received a simple blood transfusion preoperatively designed to raise haemoglobin levels to 10 g/dl. All had acute or acute on chronic cholecystitis based on histological examination of gallbladder specimens. Twelve had common bile duct stones. In two patients calculi were missed intraoperatively but these subsequently passed into the duodenum after a period saline irrigation via an in-situ t-tube. Six developed the acute chest syndrome (aetiology not determined) and this progressed to multi system failure and death in one. This high level of postoperative mobility and mortality may in part be due to the high proportion of emergency procedures.

Adult Hirschsprung's disease in Jamaica: operative treatment and outcome

Aim  This study reviewed the operative management and outcome of patients with adult Hirschsprung’s disease treated at the University Hospital of the West Indies, Mona, Jamaica.

Open splenectomy in Jamaican children with sickle cell disease

A total of 110 patients with sickle cell disease who had open splenectomy at the University Hospital of the West Indies over a 10-year period are reviewed Patients with homozygous sickle cell disease numbered 94, S beta0 and S beta+ thalassaemias (11 and 4 respectively) and one patient with SC disease. Postoperative acute chest syndrome was the most common complication (9 of 110). There were no life threatening emergencies and no mortalities. Eleven patients received preoperative blood transfusion and operative times were short averaging 60 minutes among the 110 patients. Open splenectomy remains the gold standard for patients with sickle cell disease requiring splenectomy.

Obstruction of the duodenum by a preduodenal portal vein in situs inversus

Mirror image transposition of abdominal and thoracic viscera is termed situs inversus. Duodenal obstruction in situs inversus is rare. A preduodenal portal vein, though not uncommon in situs inversus, rarely causes duodenal obstruction. Where obstruction by a preduodenal portal vein is diagnosed, a duodeno-duodenostomy is the recommended treatment. A duodenal diaphragm and other more common causes of duodenal obstruction should also be excluded in these patients.

Hermaphroditism: cytogenetics, gonadal pathology and gender assignment a case report

True hermaphroditism is a rare intersex disorder in which individuals possess both testicular and ovarian gonadal tissue. A case of true unilateral hermaphroditism presenting with ambiguous external genitalia, right scrotal testis and left pelvic ovotestis is herein outlined Phallic, gonadal and genetic factors were considered before male gender was assigned. Gender assignment procedures have been questioned by intersex activists opposed to early genital surgery. Western societies have a binary perspective on gender and this leads to a stigma being placed on intersex cases. A multidisciplinary approach to this problem involving paediatric specialists in the field, of endocrinology, surgery and psychiatry is necessary, along with educational programmes that promote tolerance in society to variations in gender.

Penoplasty--Not Circumcision--for Buried Penis.

Buried or concealed penis is an uncommon condition in which the penile shaft is partially or completely obscured by preputial skin. In the majority of cases, it is congenital. Many patients are ill-advisedly referred for circumcision, a procedure which can compromise future repair and cosmetic outcome. We report on the management and outcome of two boys with congenital buried penis, one of whom was initially referred to the authors for circumcision.