Alastair J. W. Millar

Angiographic features of 26 children with Takayasu's arteritis

Abstract Background. Takayasus arteritis (TA) is a chronic idiopathic inflammatory disease affecting primarily the aorta, its proximal branches and the pulmonary arteries. Objectives. To retrospectively review the angiograms of children with TA so as to describe the patterns of vascular involvement. Patients and methods. Twenty-six children with TA who differed from most other studies in that almost all of them presented with hypertension, reflecting the incidence of abdominal aortic and renal artery involvement. Results. The most consistent finding was stenosis of the aorta. Marginal irregularity/undulation of the aorta was also a useful angiographic diagnostic feature in subtle disease. The incidence of aneurysms was high compared to other studies and both fusiform and saccular aneurysms were encountered. Percutaneous transluminal angioplasty (PTA) was successful in all eight patients in whom it was performed. MRI, CT angiography and US are discussed as less invasive imaging alternatives. TA is a significant cause of renovascular hypertension in children in South Africa where there is a high incidence of tuberculous infection. Knowledge of the angiographic features and pattern of aortic involvement is essential for diagnosis and initiation of early and appropriate treatment, including PTA.

Efficacy of mupirocin in methicillin-resistant Staphylococcus aureus burn wound infection.

Methicillin-resistant Staphylococcus aureus strains (MRSA) have become increasingly prevalent as nosocomial pathogens, especially in burn wounds. MRSA constituted 38% of all S. aureus isolates in our 25-bed burns unit despite the utilization of a combination of 1% silver sulfadiazine and 0.2% chlorhexidine as topical therapy. Mupirocin, a new antibiotic, has proved in vitro and in vivo to be highly effective in the treatment of MRSA infections. A prospective clinical trial with mupirocin ointment in MRSA burn wound infection was untertaken. Forty-five children with 59 discrete burn wounds and from whom MRSA were isolated were treated with 2% mupirocin ointment under occlusive dressings, applied twice daily for 5 days. The average burned area treated was 8% (range, 2 to 20%) of the total body surface area. The burn wounds were assessed clinically and bacteriologically daily. Mupirocin eliminated MRSA in all 59 wounds treated, with the maximum therapeutic response seen within 4 days. In three wounds, gram-negative organisms persisted after 5 days of topical therapy. Treatment was well tolerated by all children. We recommend that mupirocin in its present polyethylene glycol base should be used only on a selective basis, when current prophylactic topical therapy has failed to control MRSA infection in burns of less than 20% of the total body surface area, and that it should be applied only for a limited period of 5 days. The safety and the efficacy of mupirocin in burns exceeding 20% of the total body surface area need to be established.

Conjoined twins--the Cape Town experience.

This paper records our experience in the management of 25 sets of conjoined twins seen over a 32 year period (1964–1996). The twins were classified into 14 complete and symmetrical sets and 11 incomplete or heteropagus. The 14 symmetrical sets included 9 thoracopagus, 2 ischiopagus, I craniopagus and 1 omphalopagus twins. In the incomplete heteropagus group there was I ischiopagus, one twin being anencepahlic, 2 dipygus, 5 parasitic, 2 fetus-in-fetu and I cranial and caudal. The management is detailed case by case. Overall 10 of 14 symmetrical sets underwent attempts at separation with 16 surviving the procedure, but there were 3 late deaths. In the incomplete group 10 of 11 were operated on with 9 survivors. The importance of a multi-disciplinary approach, the extensive investigations required pre-operatively to define areas of organ and bony conjuction, congenital anomalies of each twin and surgical teamwork is emphasized. Specific problems encountered were indentified. In thoracopagus twins the hearts were of paramount importance as conjuction was usually fatal, being associated with major congenital defects. The greater the extent of thoracic cage fusion the greater the chance of associated severe anomaly. Skin expansion to assist coverage of the defects after separation was of great assistance, as was the use of collagen coated vicryl. Evaluation of the liver and pancreatico-biliary systems with isotope excretion scanning was crucial to pre-operative planning. Where there was fusion of the duodenum a single pancreatico-biliary system could be expected and prior strategies for separation and Rouxen-Y enteric drainage of both pancreatic and biliary secretion should be planned. Gastro-oesophageal reflux led to considerable morbidity in both twins of a thoraco-omphalopagus set. In ischiopagus and dipygus conjoined twins bilateral posterior iliac osteotomies were an essential component to anatomic reconstruction of the pelvic ring and wound closure. Also in this group, due to the frequency and extent of shared genital, urinary and ano-rectal structures, long-term morbidity was expected and a component of this might be due to spinal cord tethering, or as in one of our cases, a progressive hydrosyringomyelia. Timing of separation was ideally set at between 5 and 9 months with 6 to 8 weeks of prior tissue expansion but earlier operation was frequently required because of cardio-respiratory problems or organ failure in one twin. In most cases the goal of obtaining separate, independant and intact individuals was achieved.

Abdominal aortic aneurysms in children

Abdominal aortic aneurysms are rare in children. Causes include mycotic aneurysms, vasculitides (eg, Takayasus arteritis), connective tissue diseases (eg, Marfans syndrome, Ehlers-Danlos syndrome, and tuberous sclerosis) and traumatic false aneurysms. Four cases are described. Case 1 was a 12-year-old boy who presented with an acute unheralded rupture of the subdiaphragmatic aorta accompanied by lower limb paralysis and ischemia. Attempted repair failed because of extensive friability of the large arteries. Histological evaluation confirmed cystic medial necrosis despite Marfanoid phenotype. Cases 2 and 3 were boys aged 12 and 11 with Takayasus arteritis who presented with hypertensive encephalopathy and heart failure. Although both had involvement of the origins of the renal arteries, one aneurysm was predominantly suprarenal and the other infrarenal. Currently both children are being managed successfully with antihypertensive therapy. Case 4 was a 5-year-old girl who presented with hypertension and a pulsatile abdominal mass after treatment of infective endocarditis 18 months previously. Arteriography and three-dimensional computed tomography confirmed an aneurysm (6 x 5 x 4 cm) arising from the aorta and involving the right renal artery. Aneurysmectomy, removal of a small ischemic right kidney, and Gore-Tex grafting resulted in cure of the hypertension and uneventful recovery. The present series confirms that rupture is a fatal complication, renovascular complications are common, and medical control of hypertension is an essential part of management. Management strategies need to be highly individualized, and may be successful without surgical intervention. Close clinical and ultrasound follow-up of those managed nonoperatively is essential.

Is a gastric drainage procedure necessary at the time of antireflux surgery

BACKGROUND Gastroesophageal reflux is part of a generalized foregut motility disorder, which may also include delayed gastric emptying. With persistence of gastroesophageal reflux, or the presence of complications, including recurrent aspiration syndrome and esophageal stricture formation, surgical correction may be indicated. It is uncertain whether a procedure to resolve delayed gastric emptying is indicated at this time as well. METHODS Sixty-seven children with proven gastroesophageal reflux had preoperative gastric emptying assessed using 99Technetium-Sn-colloid labelled milk. Delayed gastric emptying was defined as a gastric residual activity of more than 40% at 2 hours after feeding. The antireflux operation was a partial anterior fundoplication. Postoperative milk scans assessed the effect of surgery on gastric emptying. RESULTS Gastric emptying at 2 hours improved overall from a median of 22% before surgery to 17% after surgery. In 17 patients delayed gastric emptying was identified before surgery; in 15 of those it returned to within normal limits after surgery. In 50 children with normal gastric emptying before surgery (gastric residual activity at 2 hours 16%), 14 (28%) showed delayed gastric emptying in the postoperative scan. CONCLUSIONS Delayed gastric emptying is common in children who undergo surgery for gastroesophageal reflux disease. A partial anterior fundoplication antireflux operation improves gastric emptying to within normal limits in the majority (88%) in this group, rendering a synchronous gastric drainage procedure unnecessary.

The surgical management of cricopharyngeal achalasia in children

OBJECTIVE The objective of this study was to evaluate the clinical presentation of children with cricopharyngeal achalasia and to document the diagnostic process followed and evaluate the immediate and long-term results of those children treated with a cricopharyngeal myotomy. METHODS Five children who underwent cricopharyngeal myotomies since 1976 were identified and the hospital records were reviewed in detail. Four patients were followed up to establish the long-term results of surgery. RESULTS The age of initial presentation ranged from birth to 6 months with a universal delay in establishing the diagnosis ranging from 11 to 138 months. Two children had pre- and post-operative manometry of the upper esophageal sphincter. Post-operative upper esophageal sphincter pressures were reduced to 29 and 47% in relation to pre-operative values. Nissen fundoplications were performed in two patients to control documented gastro esophageal reflux. No post-operative complications were noted and complete symptomatic relief was obtained in all children. The long-term follow up was 2, 10, 12 and 14 years with all children remaining free of symptoms. CONCLUSION Cricopharyngeal achalasia is an important but relatively seldom diagnosed cause of dysphagia in children. The diagnosis is almost always delayed because the condition is not widely recognised amongst physicians. If the diagnosis and effective treatment is delayed significant morbidity or even mortality, mainly due to pulmonary aspiration, may result. Cricopharyngeal myotomy is a safe and effective operation with excellent results. Symptomatic relief is immediate and complete with no long-term recurrence documented in this series.

Intrahepatic fetus-in-fetu: A case report and review of the literature

The authors report the third case since 1964 of fetus-in-fetu presenting at Red Cross Childrens Hospital. A fetal liver abnormality was detected during routine antenatal ultrasound scan, and the postnatal diagnosis of an intrahepatic, multiple fetus-in-fetu was made with abdominal ultrasound scan, plain radiograph of the abdomen and computed tomography. The mass was removed surgically by hemihepatectomy when the patient was 4 months of age. Imaging and the macroscopic and microscopic appearances of the abnormality confirmed the diagnosis and amplified it to include an adjacent teratoma. Multiple small subcapsular and perimass vascular hamartomas were coincidentally discovered in the liver. Two of the 3 nodules within the amniotic-lined cavity of the intrahepatic mass individually comply with the accepted criteria for fetus-in-fetu, and the third nodule has the accepted features of a benign teratoma. The existence of multiple fetus-in-fetu and fetus-in-fetu combined with a teratoma, is recorded in the medical literature. The imaging as well as the macroscopic and microscopic features are illustrated.

Patterns of pediatric injury in South Africa: an analysis of hospital data between 1997 and 2006.

BACKGROUND Pediatric injuries are associated with significant morbidity and mortality, especially in low- and middle-income countries. Despite the magnitude of this burden, there is lack of data to characterize the etiology and risk factors associated with childhood injuries, especially in low- and middle-income countries. The aim of this article is to describe the demographics, mechanisms, and severity of injuries during a 10-year time period using hospital-based data in Cape Town, South Africa. METHODS Data from Childsafe South Africa’s registry were used to study injured children younger than 13 years who presented with either intentional or unintentional injuries to the Trauma Unit of the Red Cross War Memorial Children’s Hospital’s (RCH) Causality Department between 1996 and 2007. Univariate and bivariate analyses were performed for demographic characteristics and injury mechanisms. Poisson regression analysis was used to analyze the age-adjusted annual incidence of injury presenting to RCH. RESULTS Between 1997 and 2006, 62,782 children with a total of 68,883 injuries presented to RCH. The mean age was 5.4 years (standard deviation ± 3.5 years) and 61.7% were male. Mechanism of injury included falls (39.8%), road traffic injuries (15.7%), burns (8.8%), and assault (7.4%). The majority of injuries occurred in and around the home. Abbreviated injury severity scoring showed 60.2% of injuries were minor, 36.6% were moderate, and 3.2% were severe. Sixty-six deaths occurred in the trauma casualty department. Thirty-one percent of patients were admitted to the hospital; children who suffered burn and head injuries were more likely to require admission. CONCLUSION Age, gender, mechanism, and severity of injury in pediatric populations have not been described elsewhere in South African national or sub-Saharan regional data. This retrospective, observational study uses Level II evidence to suggest the need for targeted interventions to address risk factors for pediatric injuries, emphasizing the importance of pediatric surveillance systems as a tool to study injuries in developing countries. LEVEL OF EVIDENCE Prognostic study, level II.

Training and practice of pediatric surgery in Africa: past, present, and future.

The evolution and recognition of pediatric surgery as a specialty in Africa can be divided into 4 distinct phases, starting from early 1920s till the present. The pace of development has been quite variable in different parts of Africa. Despite all recent developments, the practice of pediatric surgery in Africa continues to face multiple challenges, including limited facilities, manpower shortages, the large number of sick children, disease patterns specific to the region, late presentation and advanced pathology, lack of pediatric surgeons outside the tertiary hospitals, and inadequate governmental support. Standardization of pediatric surgery training across the continent is advocated. Collaboration with well-established pediatric surgical training centers in Africa and other developed countries is necessary. The problems of delivery of pediatric surgical services need to be addressed urgently, if the African child is to have access to essential pediatric surgical services like his or her counterpart in the high-income parts of the world.

Kaposi sarcoma in children with HIV: a clinical series from Red Cross Children's Hospital

AIM The study aimed to assess the clinical presentation, diagnosis, and treatment of Kaposi sarcoma in our HIV-positive paediatric population; analyse the increase in Kaposi sarcoma with the HIV epidemic; and look at unique surgical presentations and their implications for gastrointestinal surveillance. The incidence of Kaposi sarcoma, a herpesvirus 8-associated neoplasm of endothelial cells, has increased with the onset of the HIV epidemic. Surgical interest was prompted by a case of Kaposi sarcoma presenting with intussusception. METHODS All HIV-positive children with Kaposi sarcoma from 2003 to 2007 were included in the study. A retrospective analysis of clinical data was performed. MAIN RESULTS There were 9 children who ranged in age from 1 to 10 years (median, 7.1 years). Clinical presentations included oropalatal and laryngeal disease (5), pleural effusion (2), inguinoscrotal and skin disease (4), lymph node involvement (3), and gastrointestinal disease with haemorrhage (2), including one as a result of intussusception. Most patients presented with more than one clinical area involved. CD4 counts ranged from 14 to 2105. Two patients developed Kaposi sarcoma on antiretroviral treatment (ART); the remaining patients were started on antiretroviral treatment at presentation. Two patients died from overwhelming disseminated disease and one patient was lost to follow-up. Chemotherapy was required to achieve remission in the remaining 6. Before the HIV epidemic, this hospital treated one patient with Kaposi sarcoma every 4 years; the incidence has now increased to 2 patients per year. CONCLUSION Kaposi sarcoma is an increasingly important paediatric cancer in the era of the HIV epidemic. The clinical presentation in children is commonly oropalatal and inguinoscrotal disease. Gastrointestinal involvement was highlighted by a presentation with intussusception. The surgeons role in diagnosis and management includes clinical recognition, biopsy, and definitive treatment.