Esophageal atresia with tracheoesophageal fistula: two case reports.

BACKGROUND: The incidence of esophageal atresia with tracheoesophageal fistula is 1 out of 3000-5000 live births. Its incidence in lower middle income countries is not known. The infants usually present with excessive secretions or choking while feeding and are at risk for aspiration. The outcome of these infants in lower middle income countries is not encouraging due to delays in referral, sepsis at presentation requiring preoperative stabilization, postoperative complications such as anastomosis leaks, pneumonia, and pneumothorax. CASE PRESENTATION: We present two African babies who were term infants at age 2 days (male) and 5 days (female) with diagnosis of esophageal atresia and tracheoesophageal fistula. The 5-day-old infant required preoperative stabilization due to sepsis and delayed surgery with a poor postoperative outcome. The 2-day-old infant was preoperatively stable and had a good postoperative outcome. The challenges faced in management of these two cases have been highlighted. CONCLUSION: Outcome of infants with esophageal atresia and tracheoesophageal fistula in lower middle income countries is not encouraging due to delays in referral and poor postoperative healing attributed to sepsis and recurrent pneumothorax. Timely referral, preoperative condition of the infant, and timely management has shown to be a contributory factor for an improved outcome.

Late diagnosis of congenital diaphragmatic hernia: a case report.

BACKGROUND: Congenital diaphragmatic hernia beyond the neonatal period is not uncommon. Its diagnosis in infancy and early childhood poses a challenge owing to different clinical presentation ranging from gastrointestinal to respiratory symptoms. These neonates are usually misdiagnosed as having pneumonia until radiological imaging picks up the defect during routine scan for worsening respiratory symptoms. In high-income countries, the survival rate for these patients has been reported to be high, while in Sub-Saharan Africa the survival rate is still low due to delayed diagnosis, delayed referral, and hence delayed management. CASE REPORT: We present an African male baby from non-consanguineous parents, 6 weeks old, diagnosed with congenital diaphragmatic hernia at 6 weeks of age after failure to respond to antibiotics for suspected pneumonia. Despite attempts at management, he died at 5 weeks post surgery. CONCLUSION: Our case emphasizes the importance of early clinical suspicion and early detection for a differential diagnosis of congenital diaphragmatic hernia in infants who present with respiratory symptoms not responding to antibiotics or recurrent pneumonia, and improving the availability of imaging in primary care facilities to diagnose such defects early and manage them accordingly.

Caudal regression syndrome without maternal diabetes mellitus.

Caudal regression is a rare complex disorder impacting the formation of the caudal segment of the spine and spinal cord. We report a preterm newborn baby who was referred to us due to respiratory distress syndrome and bilateral knee contracture. A clinical examination and a radiographic skeletal survey revealed a short spinal cord with complete agenesis of the lumbar, sacrum and coccygeal spine, and hypoplastic iliac bones with bilateral knee contractures. The mother did not have diabetes. The long-term outcome is not well-known in our set-up.

Neurocristic cutaneous hamartoma of the scalp with disseminated melanocytic nevi.

We report a newborn with neurocristic cutaneous hamartoma of the scalp. He was delivered at term via caesarean section due to a previous scar and presented at the neonatal unit on the fifth day with giant congenital nevi on the scalp and disseminated melanocytic nevi throughout the body. The MRI scan of the brain showed a defect at the occipital region with herniation of the occipital lobes and ventricles through the defect, with infratentorial brain parenchyma exhibiting normal signal return and intact cerebellum. The initial diagnosis was a giant haemangioma, which has ruptured, and possible cytomegalovirus infection, causing 'blueberry muffin' syndrome. On follow-up, the hamartoma/haemangioma-like mass regressed, and a large well-demarcated melanotic patch on scalp and large encephalocele were seen. This infant is now being scheduled for neurosurgical intervention.

Spectrum of paediatric rheumatic disorders at a tertiary hospital in Tanzania.

BACKGROUND: Paediatric rheumatic disorders are common in children and result in significant impairment in quality of life, morbidity and mortality. There is limited information on the burden of these disorders in lower income countries especially in sub-Saharan Africa. Few case reports have documented presence of paediatric rheumatic disorders in Tanzania. This study was conducted to determine the spectrum of rheumatic disorders among children at Muhimbili National Hospital (MNH). METHODS: This was a retrospective study conducted among children who were attended at MNH between January 2012 and August 2019. Paediatric patients seen in the out-patient clinics and those admitted in the wards were eligible. All patients with diagnosis of rheumatic disorders were identified from admission books and outpatient clinic logbooks, and later data were collected from their case notes and were recorded in clinical research forms. Collected information included age, sex, clinical features and laboratory tests results. RESULTS: A total of 52 children with mean age of 9.5 ± 4.3 years, 12 (40.4%) participants were aged above 10 years and 32 (61.5%) were females. Frequently reported clinical presentations were joint pain 44 (84.6%), joint swelling 34 (65.4%), fever 24 (46.2%) and skin rashes 21(40.4%). Juvenile idiopathic arthritis (JIA) was the predominant diagnosis reported in 28 (53.8%) participants followed by juvenile systemic lupus erythematosus 8 (15.4%), mixed connective tissue diseases 4 (7.7%) and juvenile dermatomyositis 4 (7.7%). Antinuclear antibody test was performed in 16 participants it was positive in 9 (56.2%). Nine participants were tested for anti-double stranded DNA test and 5 (55.6%) were positive for this test. C-reactive protein was tested in 46 participants out of which 32 (69.6%) had elevated levels. HIV was tested in 24 (46.2%) participants and results were negative. Thirty-five out of 52 (67.3%) participants had anaemia. Predominant drugs used for treatment of JIA include prednisolone and methotrexate. CONCLUSIONS: Paediatric rheumatic disorders are not uncommon in Tanzania-and were noted to affect more female children in this study. Predominant conditions included juvenile idiopathic arthritis (JIA), juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM).

Acute kidney injury among critically ill neonates in a tertiary hospital in Tanzania; Prevalence, risk factors and outcome.

BACKGROUND: Neonatal acute kidney injury contributes to high mortality in developing countries. The burden of neonatal AKI is not known in Tanzania despite having high neonatal mortality. This study was conducted to determine the burden of AKI among critically ill neonates admitted at Muhimbili National Hospital. METHODS: This was a cross-sectional study conducted in the neonatal ward at the MNH. Eligible critically ill neonates were recruited consecutively between October 2017 and March 2018. Data was collected using a standardized structured questionnaire. Blood specimen was drawn to measure baseline creatinine at admission, 48th hour, 72nd hour and 14th day. Data was analysed using SPSS version 20.0 Univariate analysis was done using chi-square to determine the association between categorical variables and multivariate logistic regression was performed to determine predictors of AKI. RESULTS: A total of 378 critically ill neonates were recruited, 31.5% had AKI and independent predictors of AKI were noted to be neonatal sepsis (aOR 2.237, 95%CI 1.3-3.6, P = 0.001), severe pneumonia (aOR3.0, 95%CI 1.0-9.3, P = 0.047) and use of gentamycin (aOR6.8, 95%CI 1.3-9.3, P = 0.02). Complete resolution of renal dysfunction at the fourteenth day was seen in 83.1% of the neonates while 16.9% had persistence of renal dysfunction. Ultrasound scan were performed among 105 participants with AKI revealed increased echogenicity, mild hydronephrosis and ectopic kidneys in 25 (23.8%), 4 (3.8%) and 2 (1.9%) respectively. In-hospital mortality was significantly higher among neonates with AKI (70.6%) as compared to those without (29.4%) p< 0.001. CONCLUSIONS: AKI was noted in a third of critically ill neonates, with neonatal sepsis, severe pneumonia and use of gentamycin as independent predictors of AKI. Neonates who suffered AKI had twice as much mortality as compared to those without.