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. METHOD: The case notes for 11 patients with adult Hirschsprung's disease, treated operatively between January 1986 and December 2007, were reviewed and data on preoperative diagnosis, operative procedures and postoperative complications were retrieved. RESULTS: Diagnosis was by open rectal biopsy in nine patients and by rectal suction biopsy in two patients. The time taken for preoperative bowel preparation ranged from 10 to 35 days, and colostomy was required in three patients to facilitate bowel cleansing. The Soave and Swenson procedures were used in six and five patients, respectively, leading to anastomotic stricture in one patient treated using the Soave procedure and to leakage in two patients treated using the Swenson procedure. All procedures took longer than 300 min, and 300-800 ml of blood or plasma was transfused. CONCLUSION: Therapeutic procedures for adult Hirschsprung's disease may result in life-threatening anastamotic complications. A protective colostomy is recommended when Swenson's procedure is used.

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.

Pre-adolescent gunshot injuries: anatomy of a Jamaican problem

This retrospective analysis explores the apparent increase in gunshot injuries among pre-adolescent Jamaican children. During the five-year study period (2001-2005), 74 children less than 12 years old were treated for gunshot injuries at the Bustamante Hospital for Children. In the last four years of the study, the hospital incidence of such child shootings rose by 155%. Children between six and eleven years of age were seen to be at particular risk. Shootings were likely to occur between 4:00 pm and 10:00 pm in the evening, at or near home, in inner city communities. Affected children were unlikely to have been under direct adult supervision at the time of injury and were reported to be intended targets of the shooting in 49% of cases. Injuries to the limbs occurred most frequently, resulting chiefly in soft tissue injuries and open fractures. Half required operative intervention, most avoiding blood transfusion. Hospital stay was usually less than a week. Though clearly needed, social support services were underutilized A mortality rate of 4% was seen but long-term morbidity was uncommon. Routine social and psychiatric evaluation of victims, organized after school-care, establishment of paediatric paramedical services, establishment of a dedicated paediatric interhospital transfer team and more widespread training in paediatric trauma management are recommended to improve the quality of care given to paediatric victims of firearm injuries.

Este análisis retrospectivo explora el aumento evidente de las heridas de bala entre los niños jamaicanos pre-adolescentes. Durante un período de cinco años de estudio (2001–2005), 74 niños menores de 12 años fueron atendidos debido a heridas de bala en el Hospital Pediátrico Bustamante. En los últimos cuatro años de este estudio, la incidencia en el hospital de heridas producidas con armas de fuego a niños aumentó en un 155%. Niños entre seis y once años de edad se considerabanparticularmente en riesgo. Los tiroteos ocurrían probablemente entre 4 pm de la tarde y 10 pm de la noche, en la casa o en el vecindario, en las comunidades de los suburbios citadinos internos (conocidos como inner cities). Es poco probable que los niños afectados hayan estado bajo supervisión directa dealgún adulto en el momento de recibir la herida, y se reportó que fueron objetivo expreso de los disparos en el 49% de los casos. Las heridas en las extremidades ocurrieron con mayor frecuencia, trayendo como consecuencia principalmente heridas en tejidos blandos y fracturas abiertas. La mitadde ellos requirió intervención quirúrgica, evitándose la transfusión sanguínea en la mayoría de los casos. La estadía en el hospital por lo general duró menos de una semana. Aunque evidentemente senecesitaban servicios de apoyo social, hubo una marcada subutilización de los mismos. Se observó una tasa de mortalidad del 4% pero la morbilidad a largo plazo resultó poco común. La evaluación psiquiátrica y social de rutina de las víctimas, organizada después de la atención en la escuela, el establecimiento de servicios paramédicos pediátricos, el establecimiento de un equipo dedicado de transferencia interhospitalaria pediátrica, y un entrenamiento más amplio en el tratamiento de traumaspediátricos, se recomiendan a fin de mejorar la calidad de la atención brindada a las víctimas pediátricas de heridas por arma de fuego.

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.

Pre-adolescent gunshot injuries: anatomy of a Jamaican problem.

This retrospective analysis explores the apparent increase in gunshot injuries among pre-adolescent Jamaican children. During the five-year study period (2001-2005), 74 children less than 12 years old were treated for gunshot injuries at the Bustamante Hospital for Children. In the last four years of the study, the hospital incidence of such child shootings rose by 155%. Children between six and eleven years of age were seen to be at particular risk. Shootings were likely to occur between 4:00 pm and 10:00 pm in the evening, at or near home, in inner city communities. Affected children were unlikely to have been under direct adult supervision at the time of injury and were reported to be intended targets of the shooting in 49% of cases. Injuries to the limbs occurred most frequently, resulting chiefly in soft tissue injuries and open fractures. Half required operative intervention, most avoiding blood transfusion. Hospital stay was usually less than a week. Though clearly needed, social support services were underutilized A mortality rate of 4% was seen but long-term morbidity was uncommon. Routine social and psychiatric evaluation of victims, organized after school-care, establishment of paediatric paramedical services, establishment of a dedicated paediatric interhospital transfer team and more widespread training in paediatric trauma management are recommended to improve the quality of care given to paediatric victims of firearm injuries.

Hermaphroditism: cytogenetics, gonadal pathology and gender assignment: a case report / Hermafroditismo: citogenética, patología gonadal y asignación de género

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.

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.

Open splenectomy in Jamaican children with sickle cell disease / Esplenectomía abierta en niños jamaicanos con la enfermedad de células falciformes

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.

El presente trabajo revisa un total de 110 pacientes con la enfermedad de células falciformes, que fueran sometidos a una esplenectomía abierta en el Hospital Universitario de West Indies, a lo largo de un período de 10 años. Los pacientes con enfermedad de células falciformes homocigóticas fueron 94, con talasemias S b0 y S b+ fueron 11 y 4 respectivamente, y un paciente presentaba la enfermedad por hemoglobina SC. El síndrome torácico agudo postoperatorio resultó ser la complicación más común (9 de 110). No hubo emergencias con riesgo de vida ni mortalidades. Once pacientes recibieron transfusión de sangre en el postoperatorio y los tiempos de operación fueron cortos, con un promedio de 60 minutos entre los 110 pacientes. La esplenectomía abierta sigue siendo la norma de oro para los pacientes con la enfermedad de células falciforme que requieren esplenectomía.

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.

"Minimal intervention management" for gastroschisis: a preliminary report.

The replacement of eviscerated bowel, without anaesthesia, has been performed safely in stable neonates with gastroschisis. This technique, termed "minimal intervention management", was used in three infants treated at the Newborn Special Care Nursery of the University Hospital of the West Indies. Two infants had excellent results but one had bowel perforation during the procedure, necessitating conversion to formal laparotomy under general anaesthesia. In selected patients, advantages of this technique include the ability to be guided by patient response during the procedure in order to avoid excessive intra-abdominal tension, the avoidance of anaesthesia and minimal cost. This technique is proposed for wider use in developing countries.

"Minimal intervention management" for gastroschisis: a preliminary report / "Tratamiento de Intervención Mínima" para la Gastroquisis Un Informe Preliminar

The replacement of eviscerated bowel, without anaesthesia, has been performed safely in stable neonates with gastroschisis. This technique, termed [quot ]minimal intervention management[quot ], was used in three infants treated at the Newborn Special Care Nursery of the University Hospital of the West Indies. Two infants had excellent results but one had bowel perforation during the procedure, necessitating conversion to formal laparotomy under general anaesthesia. In selected patients, advantages of this technique include the ability to be guided by patient response during the procedure in order to avoid excessive intra-abdominal tension, the avoidance of anaesthesia and minimal cost. This technique is proposed for wider use in developing countries

El reemplazo del intestino eviscerado, sin anestesia, se ha realizado de manera segura en neonatos estables con gastroquisis. Esta técnica, denominada "tratamiento de intervención mínima", se usó en tres infantes tratados en la Guardería de Cuidados Especiales del Recién Nacido en el Hospital Universitario de West Indies. Los resultados fueron excelentes en dos de los infantes, pero el tercero tuvo una perforación intestinal durante el procedimiento, por lo cual se hizo necesaria la conversión a la laparotomía formal bajo anestesia general. En pacientes seleccionados, esta técnica incluye entre sus ventajas la posibilidad de ser guiada por la respuesta del paciente durante el procedimiento, para prevenir así una tensión intra-abdominal excesiva, evitar la anestesia, y asegurar un costo mínimo. Se propone que el uso de esta técnica se haga extensivo en los países en vías de desarrollo.

Newborn circumcision using the Plastibell device: an audit of practice.

Circumcision of newborn male infants is widely practiced but controversial. Our experience gained circumcising 205 Jamaican neonates, using the Plastibell device is presented. Circumcisions, were requested by parents usually for hygienic reasons, and were brief outpatient procedures. Subcutaneous penile ring block with 1% lidocaine provided the most effective form of procedural analgesia. Bell separation usually occurred within 10 days of the procedure. Cosmetic results met with unanimous parental acceptance. Minor complications occurred in 2.4% of circumcisions. Physicians circumcising newborns must be readily accessible post procedure to address parental concerns and allay anxiety.

Newborn circumcision using the Plastibell device: an audit of practice

Circumcision of newborn male infants is widely practiced but controversial. Our experience gained circumcising 205 Jamaican neonates, using the Plastibell device is presented. Circumcisions, were requested by parents usually for hygienic reasons, and were brief outpatient procedures. Subcutaneous penile ring block with 1 lidocaine provided the most effective form of procedural analgesia. Bell separation usually occurred within 10 days of the procedure. Cosmetic results met with unanimous parental acceptance. Minor complications occurred in 2.4 of circumcisions. Physicians circumcising newborns must be readily accessible post procedure to address parental concerns and allay anxiety

Antenatal diagnosis of a choledochal cyst. Case management and literature review.

Antenatal abdomino-pelvic ultrasound done on a 36-year-old woman at 19 weeks' gestation revealed a subhepatic cyst in the foetus. Postnatally, a type I choledochal cyst was confirmed in the newborn female infant. Biliary obstruction necessitated cyst excision and hepaticojejunostomy at age 16 days. We recommend early surgical intervention for neonates with choledochal cysts manifesting objective evidence of biliary obstruction. Non-obstructed patients are best explored later (> three months of age) when the risk of iatrogenic injury to the biliary tree and hepatic vessels is reduced.

Antenatal diagnosis of a choledochal cyst. Case management and literature review

Antenatal abdomino-pelvic ultrasound done on a 36-year-old woman at 19 weeks' gestation revealed a subhepatic cyst in the foetus. Postnatally, a type I choledochal cyst was confirmed in the newborn female infant. Biliary obstruction necessitated cyst excision and hepaticojejunostomy at age 16 days. We recommend early surgical intervention for neonates with choledochal cysts manifesting objective evidence of biliary obstruction. Non-obstructed patients are best explored later (> three months of age) when the risk of iatrogenic injury to the biliary tree and hepatic vessels is reduced.