REVIEW: Solving difficult hepatobiliary problems in children

Most difficult hepatobiliary (HPB) problems in infancy and childhood result from pathological anatomical/mechanical derangements; therefore, surgery on the liver and bile ducts depends on a detailed understanding of liver structure, function and repair response to injury or disease. The surgeon must be aware of the very diverse range of anatomical variations. Perhaps key toimproving the outcome of paediatric HPB surgery is centralised management and associating this with a paediatric liver transplant programme, which adds expertise and, frequently, the added benefit of adult HPB surgical input to paediatric surgical care. In the United Kingdom, this has resulted in excellent measurable benefit, particularly in the management of biliary atresia, but also of choledochal cysts, portal hypertension and liver tumours. Theseconditions are briefly discussed here, with focus on the technical aspects of operative management

Can fireworks-related injuries to children during festivities be prevented?

Objective. To determine the epidemiological features and outcome of fireworks-related injuries among children 0 - 13 years old.Design. A retrospective study from the trauma registry of a children’s hospital from 2001 - 2009.Results. Fifty-five children were treated for injuries from fireworks. The mean age was 8.8 years, 78% were boys, and the largest age group was 5 - 9 years old. Firecrackers accounted for 95% of the injuries; the most commonly injured body sites were hands (44%), eyes (42%) and face (31%); 47% of the patients had more than one injury. The most common injury type was burns (67%); 25 children were admitted, mostly to the burns and ophthalmology units. The mean length of hospital stay was 3.5 days. Surgical intervention was required in 38% of the patients. Most of the fireworks accidents occurred in or around the patients’ homes. There were more fireworks-related injuries around Guy Fawkes Day (85%) than New Year’s Eve (9%).Conclusion. Consumer fireworks cause serious but preventable injuries to children, either as users or bystanders. Children and their families should be encouraged to enjoy pyrotechnical displays conducted by professionals at designated areas. All fireworks for individual private use should either be supervised by an adult or banned. Current legislation should be more strictly enforced, especially the sale to under-age children

Liver transplantation at Red Cross War Memorial Children's Hospital

The liver transplant programme for infants and children at Red Cross War Memorial Children's Hospital is the only established paediatric service in sub-Saharan Africa. Referrals for liver transplant assessment come from  most provinces within Smith Africa as well as neighbouring countries. Patients and methods. Since 1987, 81 children (range 6 months - 14 years) have had 84liver transplants with biliary atresia being the most frequent diagnosis. The indications for transplantation include biliary atresia (48), metabolic (7), fulminant hepatic failure (10), redo transplants (3) and other (16). Four combined liver/kidney transplants have been performed. Fifty-three were reduced-size transplants with donor/recipient weight ratios  ranging from 2:1 to 11:1 and .32 children weighed less than 10 kg.Results. Sixty patients (74%) survived 3 months -14 years post transplant. Overall cumulative 1- and 5-year patient survival figures are 79% and 70% respectively. However, with the introduction of prophylactic intravenous ganciclovir and the exclusion of hepatitis B virus (HBV) IgG core Ab-positive donors, the 1-year patient survival is 90% and the projected 5-year  paediatric survival is> 80%. Early(< 1 month) postliver- transplantmortality was low. Causes include primary malfunction (1), inferior vena cava   thrombosis (1), bleeding oesophageal ulcer (1), sepsis (1) and cerebral oedema (1). Late morbidity and mortality was mainly due to infections: de novo hepatitis B (5 patients, 2 deaths), Epstein--Barr virus (EBV)related post-transplantation lymphoproliferative disease (12 patients, 7 deaths) and cytomegalovirus (CMV) disease (10 patients, 5 deaths). Tuberculosis (TB) treatment in 3 patients was complicated by chronic rejection (1) and TB-drug-induced subfulminant liver failure (1).Conclusion. Despite limited resources, a successful paediatric programme has been established with good patient and graft survival figures and excellent quality of life. Shortage of donors because of infection with HBV and human immunodeficiency virus (HIV) leads to significant waiting-list mortality and infrequent transplantation

Liver transplantation at Red Cross War Memorial Children's Hospital

No Abstract. South African Medical Journal Vol. 96(9) (Part 2) 2006: 960-96

Four decades of conjoined twins at Red Cross Children's Hospital - lessons learned

No Abstract. South African Medical Journal Vol. 96(9) (Part 2) 2006: 931-94

Overview of a paediatric renal transplant programme

No Abstract. South African Medical Journal Vol. 96(9) (Part 2) 2006: 955-95

Congenital cardiovascular defects in children with intestinal malrotation

Intestinal malrotation (IM) and cardiovascular defects (CCVD) are both common congenital defects. We investigated the prevalence and types of CCVD in a 25-year IM population, and its association with post-IM-operative morbidity and mortality. Data on the type of CCVD, other congenital defects, syndromes, associations, post-IM-operative morbidity and mortality were retrospectively reviewed from the records of IM patients born between 1980 and 2005. Data were analyzed on (significant) differences between CCVD subgroups, and risk factors for both morbidity and mortality were calculated. Seventy-seven of 284 IM patients (27.1%) were diagnosed with a major or minor CCVD (37 and 40 patients, respectively). Syndromes and associations were more frequently diagnosed in patients with major than with a minor CCVD (67.6 vs. 40%, respectively). Post-IM-operative complications, although frequently observed (61%), did not differ between patients with major and minor CCVD. Physical CCVD signs before IM surgery increased post-IM-operative morbidity significantly (OR 4.0, 95% CI 1.4–11.0). Fifteen patients died (19.5%), seven due to cardiovascular cause. Mortality risk was increased by intestinal ischemia and post-IM-operative complications and by major CCVD after correction for age at weight at the time of IM operation. Congenital cardiovascular defects in children with intestinal malrotation are common, with high morbidity and mortality rates after IM operation. Elective IM surgery in young patients with CCVD should be performed in a centre with adequate paediatric cardiac care. Benefits of laparoscopic intervention need further study

Nutritional interventions and outcomes of children with short bowel syndrome in a tertiary hospital setting in South Africa

Objectives: To describe the impact of nutritional interventions on the outcomes of children with short bowel syndrome (SBS). Design: This was a retrospective descriptive observational review where data were obtained from the patient’s medical records. Subjects and setting: Children with SBS between the ages of 0 and 24 months who obtained this diagnosis between January 2005 and December 2015 at a tertiary paediatric hospital in Cape Town were investigated. Results: There were 46 patients (62% male, 38% female) included in the study. The median duration of parenteral nutrition (PN) support was one month (0.6, 2.2 months), after which 83% of patients were weaned from PN. Enteral nutrition was commenced in 96% of patients, with the majority (n = 36; 82%) starting on day six (±6; range 1–29 days) postoperatively and 80% of patients attaining full feeds at median 1.2 months (0.2, 36 months). Patients displayed a mean weight and length gain of 15 g/day (±4; range 19–92 g) and 2 cm/month (±1.4; range 0.25–4 cm) respectively. The main complications were PN-associated cholestasis (n = 17), fat malabsorption (n = 13) and vitamin D deficiency/insufficiency (n = 5). Conclusion: This study showed that early initiation of PN support was attained, and that most patients were able to achieve enteral autonomy

Burn wound infection

Infection is one of the commonest causes of death in burn patients, particularly those with extensive damage. Continuing Medical Education Vol. 26 (9) 2008: pp. 440-44