Medicine and the Law.

Congenital high airway obstruction syndrome (CHAOS) is a rare condition that can be diagnosed antenatally by ultrasound. It is usually lethal without immediate intervention at delivery. A 24-year-old woman was diagnosed with fetal CHAOS at 27 weeks' gestation. The couple declined termination of pregnancy. A multidisciplinary team including obstetricians, geneticists, paediatric surgeons, neonatologists and anaesthetists was constituted to plan an ex utero intrapartum treatment (EXIT) procedure. After several simulations, a caesarean section was performed at 38 weeks' gestation under deep inhalational anaesthesia. The fetus was fully delivered with placenta remaining in utero to maintain perfusion. A surgical airway was established via tracheostomy in approximately 5 minutes. The operation was then completed with no maternal complications. The child remains well at 3 years of age. To our knowledge, this is the first EXIT procedure performed for CHAOS in the public sector. This procedure can be lifesaving and is possible with proper planning.

COVID-19: Experience of a tertiary children's hospital in Western Cape Province, South Africa.

The COVID-19 pandemic necessitated rapid changes in healthcare systems and at Red Cross War Memorial Children's Hospital (RCWMCH), Cape Town, South Africa. Paediatric services in particular required adjustment, not only for the paediatric patients but also for their carers and the staff looking after them. Strategies were divided into streams, including the impact of COVID-19 on the hospital and the role of RCWMCH in Western Cape Province, communication strategies, adaptation of clinical services at the hospital, specifically with a paediatric-friendly approach, and staff engagement. Interventions utilised: (i) Specific COVID-19 planning was required at a children's hospital, and lessons were learnt from other international children's hospitals. A similar number of patients and staff were infected by the virus (244 patients and 212 staff members by 21 December 2020). (ii) Measures were put in place to assist creation of capacity at metro hospitals' adult services by accepting children with emergency issues directly to RCWMCH, as well as accepting adolescents up to age 18 years. (iii) The communication strategy was improved to include daily engagement with heads of departments/supervisors by earlymorning structured information meetings. There were also changes in the methods of communication with staff using media such as Zoom, MS Teams and WhatsApp. Hospital-wide information and discussion sessions were held both on social platforms and in the form of smallgroup physical meetings with senior hospital administrators (with appropriate distancing). Labour union representatives were purposefully directly engaged to assess concerns. (iv) Clinical services at the hospital were adapted. These included paediatric-friendly services and physical changes to the hospital environment. (v) Staff engagement was particularly important to assist in allaying staff anxiety, developing a staff screening programme, and provision and training in use of personal protective equipment, as well as focusing on staff wellness. In conclusion, visible management and leadership has allowed for flexibility and adaptability to manage clinical services in various contexts. It is important to utilise staff in different roles during a crisis and to consider the different perspectives of people involved in the services. The key to success, that included very early adoption of the above measures, has been hospital staff taking initiative, searching for answers and identifying and implementing solutions, effective communication, and leadership support. These lessons are useful in dealing with second and further waves of the COVID-19 pandemic.

What is the availability of services for paediatric ENT surgery and paediatric surgery in Africa?

BACKGROUND: Children constitute 50% of Africa's population. Sub-Saharan Africa has the highest under-five mortality rates in the world. This study is the first to document the availability of paediatric ENT and paediatric surgery services in Africa. OBJECTIVE: To determine the availability of paediatric ENT services in Africa, as well as that of paediatric surgery that would complement paediatric ENT. METHOD: A descriptive observational study in the form of an online questionnaire was distributed by email to known ENT and paediatric surgeons based in Africa. RESULTS: Surgeons from twelve of 23 African countries responded to the survey. Seven countries had both ENT and paediatric surgery responses. In 8 of the 11 countries, the number of ENT surgeons per country was<6% of that of the UK, with 1 ENT surgeon per 414,000 people and 1 paediatric surgeon per 1,181,151 people. Ten of 11 countries reported hearing assessments in schools were poor/unavailable. Seventy-three percent responded positively for access to rigid laryngoscopes, bronchoscopes, cameras and fibre optic cables, tracheostomy, anaesthesia and nurse practitioners. Access was reported as poor/unavailable for balloon dilators 73% (8/11 countries); CPAP machines 73% (8/11) and sleep studies 82% (9/11 countries). Flexible endoscopes were available in 50% (4/8 countries), 75% (6/8 countries) had access to a camera, monitor and stack. Thirty-eight percent (3/9 countries) reported no ENT specialists with paediatric training. CONCLUSIONS: There is a great shortage of paediatric ENT and paediatric surgery services in Africa. More regional training opportunities and health infrastructure for these surgical specialties are needed. Collaborative development of paediatric ENT, surgery and anaesthesia should be considered to improve ENT-related child health in Africa.

The value of WhatsApp communication in paediatric burn care.

BACKGROUND: Telemedicine is increasingly applied in developed settings to facilitate transfer of information to and from burn surgeons across vast geographic areas. WhatsApp is a widely available and extremely user-friendly encrypted smartphone application that does not require the expensive physical and personnel infrastructure that characterizes many of these telemedicine systems. The aim of this study was to review the use of WhatsApp to facilitate paediatric burn injury consultations to a regional burn centre in a developing country, where burn care continues to be thwarted by administrative apathy, poor resource allocation and lack of attention to medical and nursing education at all levels. METHODS: A retrospective review was undertaken of all consultations using WhatsApp over an 18-month period, received by the burn centre's two senior medical practitioners. The specific origin and nature of the telemedicine requests for advice, transfer or follow-up were collected, as were data relating to the demographics of the patients, the aetiology, mechanism and extent of the burn injury. The impact of the system of communication in terms of reductions in admissions and clinic visits was assessed, and a cost analysis was undertaken. Feedback was also obtained from those health practitioners regularly using the service. RESULTS: 838 communications occurred during the study period, which included 1562 distinct clinical queries. 486 interactions (58%) originated from within the hospital, the majority of which were initiated by surgeons in training or burn nurse practitioners. 352 (42%) consultations were from outside the hospital. Queries related to the full spectrum of burn care, including emergency management and stabilization, triage and transfer, the need for escharotomy, fluid resuscitation, wound care, the timing and nature of surgical intervention, as well as follow-up and rehabilitation. While no significant changes in the number of surgical interventions or admissions were observed when compared to the five years prior to the intervention, outpatient visits reduced significantly during the study period. It was estimated that over 150 unnecessary admissions were also avoided as a result of the triage made possible by WhatsApp, which translated into considerable cost saving for the institution. DISCUSSION: Incorporating WhatsApp technology into the daily processes of burn care has significantly improved the quality of paediatric burn care referrals to specialist burn services. Specifically, WhatsApp has contributed to reductions in unnecessary referrals and outpatient visits, facilitated opportunities for continuing medical education, improved the care of major burn injuries through more effective prehospital communication, and enabled greater allocation of scarce specialist resources at the burn centre. This study motivates for the wider application of WhatsApp for burn care referrals, especially in developing countries.

Surgical management and outcomes of 12 cases of Wilms tumour with intracardiac extension from a single centre.

PURPOSE: To review demographics, effect of preoperative chemotherapy on tumour thrombus, imaging, operative strategy, and outcomes of 12 patients presenting with intracardiac extension of Wilms tumour thrombus. METHODS: A retrospective audit was undertaken on patients with intracardiac extension of Wilms tumour. Patients were identified from the oncology database and information obtained on clinical presentation, stage, preoperative treatment, surgical procedures and complications, histology, and survival status. Ethics approval was obtained from the University of Cape Town Human Research Ethics Committee. RESULTS: From 1984 to 2016, 337 children with Wilms tumour were treated. Twelve (3.6%) had intracardiac extension of tumour thrombus, nine into the right atrium, and three into the right ventricle. Ultrasound, computerized tomography, magnetic resonance imaging, and echocardiograms were used to assess thrombus level. Patients were staged as stage III(8) and IV(4). All patients received preoperative chemotherapy. Thrombus retracted from the heart in two cases. One patient died preoperatively. Eleven underwent laparotomy, median sternotomy, and cardiopulmonary bypass (CPB). Four underwent cavectomy. Five required cavoatrial patches. Thrombus extending into the hepatic veins was extracted in five patients. There was one intraoperative complication and one perioperative death. Thrombus histology showed viable tumour in 9 of 11 patients. Three patients died of progressive disease. Seven patients are currently disease free. CONCLUSION: A combination of imaging is required to determine thrombus extent, and this facilitates surgical planning. Preoperative chemotherapy may cause thrombus regression, thus avoiding CPB. CPB offers appropriate conditions for safe tumour thrombus excision. Full management in centres with appropriately experienced staff and facilities for CPB is recommended.

Anorectal malformations, associated congenital anomalies and their investigation in a South African setting.

PURPOSE: This study was undertaken to investigate the types of anorectal malformations (ARM), incidence of associated abnormalities and investigative methods used in patients treated at Red Cross War Memorial Children's Hospital and to determine whether these are in keeping with recent literature. Mortality rates were also reviewed. METHODS: A retrospective review of patients with ARM between 1993 and 2016 was undertaken. Clinical notes were reviewed and correlated with radiology and cardiac databases. Abnormalities were grouped according to genitourinary, musculoskeletal, gastrointestinal and cardiovascular systems. The data were separated into three periods to ascertain whether the workup strategy had changed over the years. RESULTS: A total of 282 patients were included. There were 134 (47.5%) high and 116 (41.1%) low lesions and unspecified in 32 (11.3%) patients. There were 59 (20.9%) vestibular fistulae, 46 (16.3%) combined rectourethral fistulae (rectoprostatic, rectobulbar and unspecified rectourethral) and 42 (14.9%) perineal fistulae. Associated abnormalities were detected in 152/221 (69%). Abnormalities were: Genitourinary 88/204 (43.1%), musculoskeletal 80/188 (42.5%), cardiac 44/218 (20.1%) and gastrointestinal 12/216 (5.6%). Twenty patients demised. CONCLUSION: Vestibular fistulae were most common followed by rectourethral and perineal fistulae. Musculoskeletal and genitourinary abnormalities were the most common associated findings. The mortality rate was 7% and cardiac lesions contributed to mortality. As knowledge of ARM improved, so has awareness of associated malformations. This has led to improved, more active workup, in keeping with the latest literature.

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.

Button batteries in the oesophagus: A surgical emergency.

We describe two cases of missed battery ingestion that led to extensive morbidity, requiring surgical management that would not have been necessary had the batteries been removed timeously.

Fatal non-occlusive mesenteric ischemia and the use of propranolol in paediatric burns.

Abdominal complications without abdominal injury are infrequently seen in children with major burns. They are divided into those that occur early during the emergency phase of treatment and those that occur late in the course of treatment. One of the most serious late onset complications is non-occlusive mesenteric ischaemia associated with the use of vasoactive drugs. We report on 2 children who late in the course of their burn injury developed ischaemic necrosis of their entire intestine. Both were on propranolol, the administration of which was continued with even during the periods of septic shock which preceded their demise. We are of the opinion that endogenous catecholamine release during hypotensive and septic episodes in conjunction with ß-adrenergic blockage from propranolol could lead to severe splanchnic vasoconstriction from unopposed α-adrenergic activity and hence critical circulation impairment to the bowel in the 2 children.

A review of primary and secondary burn services in the Western Cape, South Africa.

BACKGROUND: In 2011, the Department of Health of the Western Cape Province, South Africa, requested a review of current burn services in the province, with a view to formulating a more efficient and cost-effective service. This article considers the findings of the review and presents strategies to improve delivery of appropriate burn care at primary and secondary levels. METHODS: Surveys were conducted at eight rural and urban hospitals, two outreach workshops on burn care, four regional hospitals and at least 60 clinics in Cape Town and in the Western Cape as far as Ladismith. A survey on community management of paediatric burns was also included in the study. RESULTS: The incidence of burns was highest in the winter months, more than half of those affected were children, and the majority of burns were scalds from hot liquids. Most burn injuries managed at primary level were minor, with 75% of patients treated by nurse practitioners and discharged. The four regional secondary hospitals managed the majority of moderate to severe burns. There is room for improvement in terms of treatment facilities and consumables at all levels, regional hospitals being particularly restricted in terms of outdated equipment, a shortage of intensive care unit beds, and difficulties in transferring patients with major burns to a burns unit when indicated. CONCLUSION: The community management of paediatric burns was satisfactory, although considerable delays in transfer and insufficient pain control hampered appropriate care. A great need for ongoing education at all levels was identified. Ten strategies are presented that could, if implemented, lead to tangible improvements in the management of burn patients at primary and secondary levels in the Western Cape.

Local anesthetic wound infusion versus standard analgesia in paediatric post-operative pain control.

INTRODUCTION: Local anesthetic wound infusion has shown promising results in adults. Its use in children is limited to some centers and there are only a few prospective trials in this group of patients. METHODS: Sub-fascial continuous local anaesthetic wound infusion (CLAWI) (0.2% Bupivacaine) plus intravenous paracetamol and rescue intravenous morphine was compared to: (a) Epidural bupivacaine (EPI) plus paracetamol and rescue intravenous morphine for patients undergoing laparotomy. (b) Intravenous morphine and paracetamol (standard post-operative analgesia-SAPA) in children undergoing Lanz incision laparotomy for complicated appendicitis. 'InfiltralLong', PANJUNK(®) catheters were placed sub-fascially after peritoneal closure for post-operative bupivacaine infusion. Pain scores were recorded regularly by the same blinded pain specialist. The primary outcomes were pain control and total morphine. The secondary outcomes were time to full feeds, mobilization requirement for urinary catheter and complications. RESULTS: Sixty patients (18 laparotomy-CLAWI, 17 laparotomy-EPI and 12 appendectomy-CLAWI, and 13 appendectomy-SAPA) were analyzed. The average pain score was 2.5 (1-4) in the CLAWI groups, 3.0 (1-5) in the EPI group and 3.5 (2-5) in the SAPA group. Morphine requirements were markedly less for CLAWI. SAPA and EPI groups required urinary catheters for longer and took longer to mobilize (average 4 days compared to 2 days for CLAWI). There were no wound or bupivacaine complications in the CLAWI group. CONCLUSION: Continuous subfascial bupivacaine infusion is reliable, safe and effective in paediatric post-operative pain control with considerably reduced opiate requirements.

A review of community management of paediatric burns.

This study was a component of a broader review to evaluate burn care in South Africa. A prospective audit of 353 children with thermal injuries admitted to the Red Cross War Memorial Children's Hospital in Cape Town was performed during 2012/2013. The audit was based to assess the adherence of initial burn management to the provincial policy guidelines on the clinical management of the burn wound. The community management of each patient prior to admission to a burns centre was assessed for the following: basic demographics, emergency home management, wound cover, analgesia and transport to medical facilities. Their ages ranged from 1 month to 14 years. The average total body surface area [TBSA] was 15% [1-86%]. Most of the injuries were due to hot water accidents [78.5%] followed by flame burns (9%), direct contact and electricity burns. Two hundred and twenty five children [63%] received first aid measures at home, including cooling with water [166] ice [30] and a cooling agent. No cooling was instituted in 130 and 65% of the patient's wounds were cooled for 10 min or less. Eighty percent proceeded to the referral centre or burns unit without their wounds being covered; with only 19 patients having any medical type of dressing available at home. Two hundred and ninety five children [83.6%] received pain medication prior to admission at the burns unit. Of the 316 patients not directly attending the burns unit, 137 received i.v. fluids of which 95 had burns greater than 10% TBSA. None of the patients were in shock on admission and all i.v. lines were functioning. Forty-four children with burns greater than 10% did not receive i.v. fluids. The audit identified six factors that were inadequately addressed during the pre-admission period: first aid, cooling of the wound, early covering of the wound, resuscitation, pain management and transfer. If these could be readdressed, basic burn care would be substantially improved in the study area.

Traditional burn care in sub-Saharan Africa: a long history with wide acceptance.

Burns are very common in sub-Saharan Africa and are considered to be a major health care problem. The management of burns in many African countries is challenged by limited financial resources, inaccessible health care facilities, lack of trained professionals and superstition. These limitations are related to the many burned patients seeking treatment from traditional healers. The use of traditional remedies, plant and animal products are seen as an important aspect of burn management as it is both an affordable and respected treatment modality. Despite its popularity, the use of traditional burn care remedies is faced with many challenges as little research has been done on its effectiveness, dosage and adverse reactions. This paper reviewed the traditions and customs associated with traditional burn care as well as the use of plant, animal and mineral products used by traditional healers.

The risks of gastrointestinal injury due to ingested magnetic beads.

Accidental ingestion of foreign bodies is a common problem in children. Magnetic bead toys are hazardous, having potentially lethal consequences if ingested. These magnets conglomerate in different segments of bowel, causing pressure necrosis, perforation and/or fistula formation anywhere along the gastrointestinal tract. A clinical diagnostic pitfall is that the appearance on the initial abdominal radiograph may be misinterpreted by the uninitiated as a single metallic object without any intervening intestinal wall. Symptoms do not occur until complications have developed, and even then, unless magnet ingestion is suspected, treatment may initially be mistakenly expectant, as with any other foreign body. After observing a case of multiple magnet ingestion that led to the rapid onset of small-bowel inter-loop fistulas and peritonitis, we attempted to reproduce the likely sequence of events in a laboratory setting using fresh, post-mortem porcine bowel as an animal model and placing magnetic toy beads within the bowel lumen. Pressure-induced perforation appeared extremely rapidly, replicating the operative findings in two of our cases. We propose that if magnet ingestion is suspected, early endoscopic or surgical retrieval is mandatory. Appropriate, rapid surgical intervention is indicated. Laparoscopy offers a minimally invasive therapeutic option.

Burn care in South Africa: a micro cosmos of Africa.

Burn injuries in Africa are common with between 300,000 and 17.5 million children under 5 years sustaining burn injuries annually, resulting in a high estimated fatality rate. These burns are largely environmentally conditioned and therefore preventable. The Western Cape Province in South Africa can be regarded as a prototype of paediatric burns seen on the continent, with large numbers, high morbidity and mortality rates and an area inclusive of all factors contributing to this extraordinary burden of injury. Most of the mechanisms to prevent burns are not easily modified due to the restraint of low socio-economic homes, overcrowding, unsafe appliances, multiple and complex daily demands on families and multiple psycho-social stressors. Children <4 years are at highest risk of burns with an average annual rate of 6.0/10,000 child-years. Burn care in South Africa is predominantly emergency driven and variable in terms of organization, clinical management, facilities and staffing. Various treatment strategies were introduced. The management of HIV positive children poses a problem, as well as the conflict of achieving equity of burn care for all children. Without alleviating poverty, developing minimum standards for housing, burn education, safe appliances and legislation, we will not be able to reduce the "curse of poor people" and will continue to treat the consequences.

Intussusception as a presenting feature of Burkitt lymphoma: implications for management and outcome.

PURPOSE: Intussusception is a recognised but unusual presenting feature of Burkitt lymphoma. We sought to identify the clinical features associated with intussusception in this setting, and assess the outcome following protocol directed chemotherapy. METHODS: A retrospective case note review was performed on patients treated for Burkitt lymphoma at our institution between 1976 and 2010. Cases presenting with intussusception were identified from hospital records and oncology database. RESULTS: Fourteen of the 210 children seen with a diagnosis of Burkitt lymphoma during the study period (6.7%) developed intussusception. Median age was 6.1 years (range 2.5-10.9). Twelve patients presented with recurrent abdominal pain, and two patients with a jaw mass associated with endemic Burkitt lymphoma. Nine patients underwent a right hemicolectomy with ileo-colic anastomosis, and five had segmental small-bowel resections. Three patients had bone marrow involvement at diagnosis, two of whom died. All patients received chemotherapy. Median follow-up was 6.07 years (range 0.1-28.8). CONCLUSIONS: Small bowel lymphoma should be considered in children presenting with intussusception above the normal infantile peak age range. The presentation is often insidious, and complete obstruction may not be apparent. However, when surgically resected, the majority can achieve a good outcome with additional chemotherapy.

Neonatal laparoscopy.

Until recently minimally invastive surgery was not performed in neonates. This was because of their small size and distinct physiological characteristics, neonates were not operated on using minimally invasive surgery until recently. Since the introduction of fine laparoscopic instruments, improvements in the surgical techniques and anaesthetic adjustments for laparoscopy, more complex operations are now being performed in neonates. While certain operations such as laparoscopic pyloromyotomy have become routine in many centres, some others require significant infrastructure and experience. Advantages of minimally invasive surgery seen in older children and adults, such as shortened hospital stay and less pain, also apply to neonates. There is no doubt that minimally invasive surgery for neonates is still in its infancy, and for many neonatal conditions requiring surgery, the benefits of minimally invasive surgery need to be established with well-designed studies.