Situs inversus abdominalis and duodenal atresia

Fewer than 20 patients born with situs inversus and duodenal atresia have been reported in the literature. We present a patient with this condition. A newborn baby presented shortly after birth with persistent bilious vomiting. An abdominal radiograph showed a right-sided stomach bubble and a second bubble on the left - typical of duodenal atresia but with mirror image configuration. Laparotomy confirmed the diagnosis of situs inversus abdominalis, which was also demonstrated by contrast studies and ultrasound. Duodenoduodenostomy was performed and the patient discharged on day 8 postoperatively. Situs inversus is associated with other congenital malformations including splenic malformations, left-sided liver and cardiac abnormalities; it is rarely associated with duodenal atresia. Duodenal obstruction in the presence of situs inversus has been described, including obstruction due to a web, stenosis, pre-duodenal portal vein and complete atresia. The patient presented in this paper had a duodenal web in the second part of the duodenum. Before undertaking surgery it is important to establish the presence of associated gastrointestinal and cardiac abnormalities

The Management of Pediatric Polytrauma: Review

Polytrauma is a major cause of mortality and morbidity in both developed and developing countries. The primary goal of this review is to provide a comprehensive overview on current knowledge in the management of pediatric polytrauma patients (PPPs). A database review was con- ducted based on a search in the Embase, Medline OVID-SP, Web of Science, Cochrane central, and Pubmed databases. Only studies with “paediatric population” and “polytrauma” as criteria were included. A total of 3310 citations were retrieved. Of these, 3271 were excluded after screening, based on title and abstract. The full texts of 39 articles were assessed; further selection left 25 articles to be included in this review. The most crucial point in the management of PPPs is preparedness of the staff and an emergency room furnished with age-appropriate drugs and equipment combined with a systemic approach

Disseminated mucormycosis and necrotizing fasciitis in immune-compromised patients: Two case reports

We present two case reports of disseminated mucormycosis and necrotizing fasciitis in an immunecompromised patient. First, a 3-month-old with untreated HIV infection presented in septic shock with abdominal wall-necrotizing fasciitis. Laparotomy  revealed extensive abdominal wall necrosis, bowel, liver, kidney and subsequent retroperitoneal, posterior diaphragm and inferior vena cava involvement. Second, a  3-year-old on chemotherapy for Burkitt’s lymphoma presented with pancytopenia, sepsis, abdominal wall-necrotizing fasciitis and left lower limb ischaemia. At surgery, there was necrosis of the abdominal wall, the large bowel and the ureter and thrombosis of the iliac vessels. Histology in both cases showed necrosis with fungal invasion consistent with mucormycosis. Both patients suffered mortality. We discuss  mucormycosis and review the literature regarding mucormycosis in immune-compromised paediatric patients.Keywords: child, immune-compromised, mucormycosis, necrotizing fasciitis, paediatri

The evolving management of Burkitt's lymphoma at Red Cross Children's Hospital

No Abstract. South African Medical Journal Vol. 96(9) (Part 2) 2006: 950-95

The evolving management of Burkitt's lymphoma at Red CrossChildren's Hospital

Background. Treatment for Burkitt’s lymphoma at Red Cross Children’s Hospital has evolved from the use of aggressive surgery and less intensive chemotherapy to a conservative surgical approach with more intensive chemotherapy. Methods. The study was a retrospective folder review of patients diagnosed with Burkitt’s lymphoma at RCCH between 1984 and 2004. Results. Ninety-two children were treated for Burkitt’s lymphoma at RCCH between 1984 and 2004. There were 10 patients with group A or fully resected disease, 52 with group B or extensive localised disease, and 30 with dissemination to the bone marrow and/or central nervous system or group C disease. Protocol 1 (less intensive chemotherapy based on the COMP regimen) was used from 1984, with protocol 2 (more intensive chemotherapy based on the LMB regimen) introduced in 1988 for group C disease, 1991 for group B disease and 1996 for group A disease. Overall 5-year survival increased from 20% with protocol 1 to 66% with protocol 2 for group C disease, and from 76.5% with protocol 1 to 88.2% with protocol 2 for group B disease. There were more admissions for neutropenic fever in patients on protocol 2 and more episodes of mucositis, and these patients required more red cell and platelet transfusions. With a more conservative surgical approach, biopsy largely replaced attempts to partially resect the tumour at primary surgery, and there was a consequent decline in surgical complications. Conclusions. Intensive chemotherapy with protocol 2 has resulted in improved survival for group C and group B patients, but with more morbidity. Protocol 1, which is less intensive with less morbidity, remains a viable strategy for group A and group B disease in resource-poor settings

Comparison in the incidence of anorectal malformations between a first- and third-world referral center

PURPOSE : Aim of study was to evaluate the differences in incidence and presentation of anorectal malformations (ARMs) between selected Pediatric Surgery Divisions in the Republic of South Africa (ZAR) and Italy. METHODS : A retrospective cohort study involved analysis of clinical records of patients with ARM born between 2005 and 2012. Type of ARM, maternal age, birth weight, gestational age, presence of associated anomalies and delayed diagnosis were analyzed. RESULTS : 335 patients were included in this study. Of note, statistically significant differences between the African and European patient groups were observed in a male predominance in the ZAR patient population. In addition, female recto-perineal fistulas were diagnosed in significantly more Italian patients than in ZAR. Furthermore, a more advanced maternal age and a lower gestational age was noted in the European cohort with a minimal delay in initial diagnosis as opposed to the African counterpart. Both centers reported recto-perineal fistula as the most common malformation in male patients. CONCLUSION : With the exception of perineal fistulas in females, the incidence of specific subtypes of ARMs was similar in the two groups. This may be of importance when extrapolating European study conclusion to the South African setting.http://link.springer.com/journal/3832016-08-31hb201

Salvageability of renal function following renal revascularisation in children with Takayasu’s arteritis-induced renal artery stenosis

Background. Renal artery revascularisation procedures are usually carried out on children with renal artery stenosis from varied causes, including Takayasu’s arteritis. Reports on the outcome of such procedures in children usually refer to the improvement in blood pressure, with only minimal mention of effects on renal function.Objective. Salvageability of renal function in children who underwent renal revascularisation for Takayasu’s arteritis-induced renal artery stenosis (TARAS) was the focus of this study.Methods. We undertook a retrospective analysis of children aged ≤16 years with angiographically confirmed TARAS who underwent renal artery revascularisation procedures between 1990 and 2010. Outcomes of renal function were studied over a period of 2 years and were defined as: (i) improvement: >20% increase in estimated glomerular filtration rate (e-GFR) from presurgery value; (ii) stabilisation: e-GFR within 20% of presurgery value; and (iii) failure: >20% deterioration in e-GFR from presurgery value. The GFR was estimated using the Schwartz formula.Results. Twenty children (9 males and 11 females, age range 2 - 14 years) had 27 renal artery revascularisation procedures. Thirteen of the patients (65.0%) had bilateral renal artery stenosis. The baseline mean e-GFR was 88.6 (standard deviation (SD) 25.4) mL/min/1.73 m2 and the mean duration of follow-up was 28.80 (SD 25.62) months. All the patients had stable or improved renal function until the 2-year follow-up, when the proportion decreased to 92.3% (12/13), as failure was recorded in one child. Bilateral revascularisation was found to be significantly associated with improvement in renal function in the early postoperative period (p=0.04).Conclusion. Renal artery revascularisation procedures are successful in salvaging renal function in children with TARAS

A review of blood transfusions in a trauma unit for young children

Background. Trauma is the leading cause of mortality and morbidity worldwide. Blood transfusions play an incremental role in the acute phase, yet practice varies owing to variations in transfusion thresholds and concerns about potential complications, especially in children. Objectives. To evaluate protocol adherence to blood transfusion thresholds in paediatric trauma patients and determine the degree of blood product wastage, as defined by discarded units. Methods. A retrospective, descriptive study of trauma patients (age 0 - 13 years) who received a blood transfusion in the trauma unit at Red Cross War Memorial Children’s Hospital, Cape Town, South Africa, over a 5.5-year period (1 January 2009 - 1 July 2014). Haemoglobin (Hb) transfusion thresholds were defined as 10 g/dL for neurotrauma patients and patients requiring skin grafting or a musculocutaneous flap (group 1). All other trauma patients had an Hb transfusion threshold of 7 g/dL (group 2). Results. A total of 144 patients were included (mean age 5.2 years (standard deviation (SD) 3.3), 68.1% male). The mean Hb increase after transfusion was 3.5 g/dL (SD 1.7). Adherence to the transfusion Hb threshold protocol was 96.7% for group 1 v. 34.0% for group 2. No complications were reported. Average blood wastage was 3.5 units per year during the study period. Conclusions. Adherence to paediatric blood transfusion protocol was low in the Hb threshold group <7 g/dL. However, transfusion-related complications and wastage were minimal. Further prospective research is required to determine optimal blood transfusion guidelines for paediatric trauma patients