Responding to the need: An evaluation of the subspecialty units in a pediatric surgical department in a limited resource setting using selected optimal resources for children's surgery strategies.

BACKGROUND: The Global Initiative for Children's Surgery group published the Optimal Resources for Children's Surgery (OReCS) document outlining the essential criteria and strategies for children's surgical care in low-resource settings. Limited data exist on subspecialties in pediatric surgery and their contribution to global surgery efforts. The study aimed to evaluate the development of subspecialty units within Chris Hani Baragwanath Academic Hospital (CHBAH) Department of Pediatric Surgery (DPS) from January 1, 2018 to December 31, 2021 using selected OReCS strategies for the improvement of pediatric surgery. METHODS: A retrospective descriptive research design was followed. The study population consisted of CHBAH PSD records. The following data were collected: number of patients managed in PSD subspecialty unit (the units) clinics and surgeries performed, number of trainees, available structures, processes and outcome data, and research output. RESULTS: Of the 17,249 patients seen in the units' outpatient clinics, 8275 (47.9%) burns, 6443 (37.3%) colorectal, and 2531 (14.6%) urology. The number of surgeries performed were 3205, of which 1306 (40.7%) were burns, 644 (20.1%) colorectal, 483 (15.1%) urology, 341 (10.6%) hepatobiliary, and 431 (12.8%) oncology. Of the 16 selected strategies evaluated across the 5 units, 94% were available, of which 16.4% was partly provided by Surgeons for Little Lives. Outcome data in the form of morbidity and mortality reviews for all the units is available, but there is no data for timeliness of care with waiting lists. There were 77 publications and 41 congress presentations. CONCLUSION: The subspecialty units respond to the global surgical need by meeting most selected OReCS resources in the clinical service provided.

A 7-year retrospective review of renal trauma in paediatric patients in Johannesburg.

BACKGROUND: South African data on paediatric patients with renal trauma that are usually managed conservatively is scarce. This study aimed to review a 7-year experience of paediatric renal trauma and management. METHODS: A retrospective review of all paediatric admissions with renal injury was conducted in the Department of Paediatric Surgery, University of the Witwatersrand, between 1 January 2012 and 31 December 2018. Data from medical records reviewed included patient age, gender, mechanism of injury, severity of injury, management and length of hospital stay. RESULTS: Thirty-one patients with renal injuries were identified, of which 30 had complete data. Of these cases, 26/30 (87%) sustained blunt renal injuries and 4/30 (13%) were penetrating. The median age at presentation was 6 years, and 60% were females. Three patients had isolated renal injuries, and 23 had concomitant injuries including hepatic (9), thoracic (8), splenic (5), head (4), facial (3) and ureteric (1). Twenty-three patients were managed non-operatively. Two required renal exploration with resultant nephrectomies and one haemodynamically unstable patient died preoperatively. Four patients required operative intervention for concomitant injuries with no renal exploration. Two patients required ureteric stenting. The median length of hospital stay was 7 days (Range: 4-11 days, IQR 7 days). CONCLUSION: Renal injuries in haemodynamically stable patients should be managed non-operatively. A 93% renal preservation rate was achieved in this cohort of patients with nephrectomy performed only in haemodynamically unstable patients with Grade V injuries, in keeping with international norms.

The Burden of Disease and Pathology at a Rapidly Expanding Tertiary Paediatric Surgical Unit in South Africa.

BACKGROUND: The precise burden of paediatric surgical care in South Africa is unknown. In the absence of epidemiological data, hospital-based study is a first step to gauge the burden and profile of paediatric surgical disease. We aim to describe the profile of pathology, pattern of referrals, and complications of paediatric surgical care at Chris Hani Baragwanath Academic Hospital (CHBAH). METHODS: A 1-year retrospective record review for the period 3/1/2019 to 1/1/2020 was conducted by evaluation of the morbidity and mortality databases of the Department of Paediatric Surgery (DPS). Number of admissions, consultations, complications, and surgeries performed were analysed and classified. RESULTS: A total of 11,932 unique patient encounters occurred. Emergencies (79%, 1841/2329) accounted for the majority of admissions. Trauma accounted for 49% (896/1841) of emergency admissions. Elective surgery constituted 52% (1202/2316) and emergency surgery 48% (1114/2316) of all procedures performed. The emergency department (55%, 1271/2329), outpatients department (19%, 447/2329), and peripheral hospitals (16%, 378/2329) were the source of the majority of admissions. A complication rate of 9% (208/2316) was observed. CONCLUSION: The high-volume subspecialist environment at CHBAH presents the ideal environment for delivery of specialist paediatric surgical services and training. Injury prevention, optimal use of existing resources, and additional physical, human and financial resources are required to meet the existing and predicted future burden of paediatric surgical disease.

Neonatal surgical outcomes: a prospective observational study at a Tertiary Academic Hospital in Johannesburg, South Africa.

PURPOSE: The neonatal period is the most vulnerable period for a child. There is a paucity of data on the burden of neonatal surgical disease in our setting. The aim of this study was to describe the frequency with which index neonatal surgical conditions are seen within our setting and to document the 30-day outcome of these patients. METHODS: This was a single-centre prospective observational study in which all neonates with paediatric surgical pathology referred to the paediatric surgical unit with a corrected gestational age of 28 days were included. RESULTS: Necrotising enterocolitis was the most frequent reason for referral to the paediatric surgical unit (n = 68, 34.34%). Gastroschisis was the most frequent congenital anomaly referred (n = 20, 10.10%). The overall morbidity was 57.58%. Surgical complications contributed to 18.51% of morbidities. The development of gram negative nosocomial sepsis was the most frequent cause of morbidity (n = 98, 50.78%). Mortality at 30 days was 21.74% (n = 40). Sepsis contributed to mortality in 35 patients (87.5%), 16 of which had gram negative sepsis. CONCLUSION: Gram-negative sepsis was a major contributing factor in the development of morbidity and mortality in our cohort. Prevention and improvement in infection control are imperative if we are to improve outcomes in our surgical neonates.

Correlation of white cell count and CRP in acute appendicitis in paediatric patients.

BACKGROUND: Ten per cent of children who present with abdominal pain at an emergency department are diagnosed with acute appendicitis. The diagnosis of which relies on clinical acumen, but addition of tests such as measurement of the white cell count (WCC) and C-reactive protein (CRP) are needed to decrease the morbidity associated with inappropriate surgical management. This study evaluates the sensitivity, specificity and accuracy of the WCC and CRP separately and when used in combination and evaluates whether an increased WCC and CRP are associated with complicated appendicitis. METHOD: A retrospective record review of all paediatric patients who underwent appendicectomy between June 2010 and December 2016 was conducted. Demographic data, the WCC, CRP and histology results were reviewed. RESULTS: 763 records were reviewed. The sensitivity and specificity of the WCC were 69.6% and 43.1% respectively and of the CRP were 95.4% and 24.5% respectively. The sensitivity was higher when both the CRP and WCC were elevated (97.47%). A normal WCC and CRP had a specificity of 98%, with an odds ratio of 8.69 of a patient not having appendicitis. There was a borderline significance between the WCC and the presence of acute appendicitis (p = 0.0494). The CRP was significant in patient with acute appendicitis (p < 0.0001). The WCC and CRP between uninflamed appendix specimens, uncomplicated appendicitis and complicated appendicitis was significant. CONCLUSION: Both increasing CRP and WCC correlates with an increased likelihood of the presence of complicated appendicitis. The chance of a patient having appendicitis with both normal WCC and CRP is low.

TGF-beta1 binding protein-like modules of fibrillin-1 and -2 mediate integrin-dependent cell adhesion.

Human fibrillin, a major component of the extracellular matrix, exists as two highly homologous forms (fibrillin-1 and -2). Several modules of fibrillin are homologous to TGF-beta1 binding protein. Two of these modules, D25 (the 25th module of fibrillin-1 and -2 D segment) and D12 (the 12th module of fibrillin-2 D segment) contain the cell adhesion motif arginyl-glycyl-aspartyl (RGD). The ability of RGD to mediate adhesion to D25-1 and D12-2 was investigated using bacterially expressed fusion proteins. Human skin fibroblasts and murine L-cells were used in microassays of cell attachment and cell spreading on fibrillin fusion-protein substrata. Dose-dependent experiments and competitive inhibition by soluble RGD-containing peptides demonstrated that D25-1 and D12-2 mediate RGD-dependent cell adhesion. These results provide evidence for a cell adhesion function of fibrillin-2. Inhibition with anti-integrin antibodies showed that alpha(v) and beta3 integrins mediate adhesion to D25-1, while alpha3, alpha(v) and beta1 are involved in adhesion to D12-2. Binding of different receptors may elicit distinct cell signalling supporting the hypothesis that fibrillin-1 and fibrillin-2 have distinct roles.

A novel de novo mutation in exon 14 of the fibrillin-1 gene associated with delayed secretion of fibrillin in a patient with a mild Marfan phenotype.

The Marfan syndrome, an autosomal dominant heritable disorder of connective tissue, is caused by mutations in the gene for fibrillin-1, FBN1. A novel FBN1 mutation was identified using temperature-gradient gel electrophoresis of a reverse-transcribed polymerase chain reaction product spanning exons 14 to 16. The mutation, G1760A, is predicted to result in the amino acid substitution C587Y and thus to disrupt one of the disulfide bonds of the calcium-binding epidermal growth factor-like module encoded by exon 14. C587Y was found to be a de novo mutation in a relatively mildly affected 15-year-old girl whose clinical phenotype was characterized mainly by ectopia lentis and thoracic scoliosis. Metabolic labeling of cultured dermal fibroblasts from the affected patient demonstrated delayed secretion of fibrillin with normal synthesis and no decrease in incorporation into the extracellular matrix compartment. Fibrillin immunostaining of confluent dermal fibroblast cultures revealed no visible difference between the patient's cells and control cells. Characterization of many different FBN1 mutations from different regions of the gene may provide a better understanding of clinical and biochemical genotype-phenotype relationships.

Endoscopic laser therapy for respiratory distress due to obstructive airway tumors.

Neodymium-YAG laser therapy for unresectable malignant airway obstructions has a promising future. Nine patients with moderate to severe respiratory distress all experienced rapid relief of dyspnea and substantial improvement in pulmonary function within hours to days after treatment with laser phototherapy. The quality of life and survival was improved in otherwise hopeless cases. A nonfatal case of pneumothorax was the only major complication.