Necrotising enterocolitis in a middle-income country: Early onset and risk factors for mortality.

AIM: There is limited literature available about necrotising enterocolitis (NEC) in low- and middle-income countries. This study sought to determine the proportion, pattern and risk factors for mortality among very low birth weight (VLBW) neonates with NEC in a middle-income setting. METHODS: A retrospective observational cohort study was conducted on all infants with birth weights less than 1501 g admitted from 2018 to 2020 at Groote Schuur Hospital, Cape Town, South Africa. Data were obtained from the Vermont Oxford Network and hospital folders. RESULTS: A total of 104/1582 (6.6%) neonates were diagnosed with NEC with a median onset of 8 days of life. The mortality rate was 39.0%, compared to the all-cause mortality rate of 18.7% for VLBW neonates. Thirty-two (32.0%) neonates with NEC were transferred for surgery of whom 10 (31.3%) died. Small for gestational age (p = 0.13), NEC stage 2B or above (p = 0,002), a positive blood culture (p = 0.018), a raised C-reactive protein (p = 0.013), hyponatraemia (p = 0.004), anaemia requiring blood transfusion (p = 0.003) and thrombocytopenia requiring platelet transfusion (p = 0.033) were associated with mortality. A positive blood culture was found in 37.0% with a predominance of Klebsiella pneumoniae isolates. CONCLUSION: NEC has an early onset and a high mortality rate in our setting.

Neonatal Admission Temperature in Middle- and High-Income Countries.

BACKGROUND AND OBJECTIVES: Despite being preventable, neonatal hypothermia remains common. We hypothesized that the proportion of newborns with hypothermia on admission would be high in all settings, higher in hospitals in middle-income countries (MIC) compared with high-income countries (HIC), and associated with morbidity and mortality. METHODS: Using the Vermont Oxford Network database of newborns with birth weights 401 to 1500 g or 22 to 29 weeks' gestational age from 2018 to 2021, we analyzed maternal and infant characteristics, delivery room management, and outcomes by temperature within 1 hour of admission to the NICU in 12 MICs and 22 HICs. RESULTS: Among 201 046 newborns, hypothermia was more common in MIC hospitals (64.0%) compared with HIC hospitals (28.6%). Lower birth weight, small for gestational age status, and prolonged resuscitation were perinatal risk factors for hypothermia. The mortality was doubled for hypothermic compared with euthermic newborns in MICs (24.7% and 15.4%) and HICs (12.7% and 7.6%) hospitals. After adjusting for confounders, the relative risk of death among hypothermic newborns compared with euthermic newborns was 1.21 (95% confidence interval 1.09-1.33) in MICs and 1.26 (95% confidence interval 1.21-1.31) in HICs. Every 1°C increase in admission temperature was associated with a 9% and 10% decrease in mortality risk in MICs and HICs, respectively. CONCLUSIONS: In this large sample of newborns across MICs and HICs, hypothermia remains common and is strongly associated with mortality. The profound burden of hypothermia presents an opportunity for strategies to improve outcomes and achieve the neonatal 2030 Sustainable Development Goal.

Immediate skin-to-skin contact for low birth weight infants is safe in terms of cardiorespiratory stability in limited-resource settings.

Aim: To investigate the safety of skin-to-skin contact initiated immediately after birth on cardiorespiratory parameters in unstable low birth weight infants. Methods: A randomized clinical trial was conducted in tertiary newborn units in Ghana, India, Malawi, Nigeria and Tanzania in 2017-2020, in infants with birth weight 1.0-1.799 kg. The intervention was Kangaroo mother care initiated immediately after birth and continued until discharge compared to conventional care with Kangaroo mother care initiated after meeting stability criteria. The results of the primary study showed that immediate Kangaroo mother care reduced neonatal mortality by 25% and the results have been published previously. The post-hoc outcomes of this study were mean heart rate, respiratory rate, oxygen saturation during the first four days and the need of respiratory support. Results: 1,602 infants were allocated to control and 1,609 to intervention. Mean birth weight was 1.5 kg (SD 0.2) and mean gestational age was 32.6 weeks (SD 2.9). Infants in the control group had a mean heart rate 1.4 beats per minute higher (95% CI -0.3-3.1, p = 0.097), a mean respiratory rate 0.4 breaths per minute higher (-0.7-1.5, p = 0.48) and a mean oxygen saturation 0.3% higher (95% CI -0.1-0.7, p = 0.14) than infants in the intervention group. Conclusion: There were no significant differences in cardiorespiratory parameters during the first four postnatal days. Skin-to-skin contact starting immediately after birth is safe in low birth weight infants in limited-resource settings.

Knowledge of essential newborn care and neonatal danger signs amongst post-natal mothers in Dar es Salaam, Tanzania.

Background: Tanzania has high infant mortality. Essential newborn care (ENC) and neonatal danger indicators are vital for reducing neonatal morbidity and mortality. This study examined postnatal moms' knowledge of ENC and neonatal danger indicators in Dar es Salaam. Methods: Post-natal moms from four hospitals in Dar es Salaam, Tanzania, participated in a hospital-based, cross-sectional study employing sequential sampling. Interviewees completed a structured questionnaire. SPSS 20.0 was used for data analysis. We employed frequencies to summarize ENC and neonatal risk signs knowledge. When the pvalue was less than 0.05, statistical significance was assumed for the adjusted odds ratio. Results: There were 825 people registered. Most were married (71.8%) and had a primary education (59%). Over 85% of women visited the prenatal clinic at least four times, however only 33.1% received ENC education during ANC visits and 70.5% following birth. Nurses and midwives trained 86% of them. 64.2% of postnatal mothers were ENC-savvy. Fever and difficulty to breastfeed were the most prevalent neonatal risk signals. Participants who did not receive ENC education before delivery were 1.74 times more likely to have bad knowledge (AOR 1.74 [95% CI (1.22-2.49)], p0.002), while moms who did not obtain education after delivery were 4.2 times more likely to have poor knowledge (AOR 4.20 [95% CI 3.00-5.88]). Conclusions: Over 35% of postnatal mothers lacked ENC and newborn danger sign awareness. Prenatal and postnatal education increased maternal knowledge. Before and after delivery, ENC and neonatal risk indicators should be emphasized.

Aetiology, antimicrobial susceptibility and outcome of children with sepsis, admitted at Muhimbili National Hospital, Dar es Salaam.

Introduction: sepsis is defined as a systemic inflammatory host response syndrome (SIRS) to infection, commonly bacterial. The global prevalence of sepsis is 8.2% with a mortality rate of 25%, whilst in Tanzania the prevalence is 6.6%. Treatment of sepsis involves early initiation of antibiotics based on local sensitivity patterns. However, there is an increase in antimicrobial resistance to commonly used antibiotics. Hence to promote rational use of antibiotics, we aimed at establishing the etiology, local susceptibility patterns and outcome of children with sepsis aged 2 months to 15 years, admitted at Muhimbili National Hospital (MNH), Dar es Salaam. Methods: a hospital based prospective cross sectional study was conducted among 245 participants who were consecutively recruited. A standardized structured questionnaire was used to collect information. Blood cultures and complete blood counts were done. Antimicrobial susceptibility was also done on positive cultures using disc diffusion method. Data were analyzed using SPSS version 20. Frequencies and proportions were used to summarize categorical data, whilst median and interquartile range was used to summarize continuous data. Student T test was used to compare means of data which were normally distributed and the differences in proportions were tested using Chi square test or Fisher's exact test. A p value of = 0.05 was considered to be statistically significant. Results: there was predominance of male participants (67.5%) with a median age was 2 years and an interquartile range (IQR) 10 months to 4 years. Culture positive sepsis was detected among 29.8% of the participants, and the common Gram-positive bacterial isolates were S. aureus (39.7%) Coagulase Negative Staphylococcus (CoNS) (35.6%) and Gram-negative isolates were E. coli (12.3%), Klebsiella spp (6.8%) and Pseudomonas aeruginosa (5.5%). All bacteria showed a high resistance to ampicillin (80%- 100%) followed by ceftriaxone (40 - 70%). All Pseudomonas aeruginosawere 100% resistant to ampicillin, gentamycin and ceftriaxone but were sensitive to amikacin. There was less than 40% resistance to co-amoxiclav, meropenem, ciprofloxacin, amikacin, and clindamycin. The overall case mortality rate from sepsis was 9.4%. Among children discharged 59.3% had prolonged hospital stay of more than 7 days. Age group 1 to 5 years, prior use of antibiotics, tachycardia, and leukocytosis were significantly associated with high mortality. Conclusion: bacterial sepsis is prevalent at Muhimbili National Hospital contributing to 9.4% of mortality and a prolonged hospital stay of more than 7 days among 59.3% of the participants. Gram-positive bacteria were found to be predominant cause of sepsis, whereas both Gram-positive and Gram-negative bacteria had a high resistance to first and second line antimicrobials including: ampicillin, gentamycin, and ceftriaxone.

Magnitude and associated factors of anti-retroviral therapy adherence among children attending HIV care and treatment clinics in Dar es Salaam, Tanzania.

INTRODUCTION: The HIV pandemic continues to contribute significantly towards childhood mortality and morbidity. The up-scaling of the Anti-retroviral therapy (ART) access has seen more children surviving and sanctions great effort be made on ensuring adherence. Adherence is a dynamic process that changes over time and is determined by variable factors. This necessitates the urgency to conduct studies to determine the potential factors affecting adherence in our setting and therefore achieve the 90-90-90 goal of sustainable viral suppression. OBJECTIVES: To assess the magnitude and associated factors of ART adherence among children (1-14 years) attending HIV care and treatment clinics during the months of July to November 2018 in Dar es Salaam. METHODS: A cross-sectional clinic-based study, conducted in three selected HIV care and treatment clinics in urban Dar es Salaam; Muhimbili National Hospital (MNH), Temeke Regional Referral Hospital (TRRH), Infectious Disease Centre- DarDar Paediatric Program (IDC-DPP) HIV clinics during the months of July to November 2018. HIV-infected children aged 1-14 years who had been on treatment for at least six months were consecutively enrolled until the sample size was achieved. A structured questionnaire was used for data collection. Four-day self-report, one-month self-recall report and missed clinic appointments were used to assess adherence. Frequencies and percentages were used to describe categorical data. The odds ratio was used to analyse the possible factors affecting ART adherence Logistic regression models were used to determine the factors associated with ART adherence. Analysis was conducted using SPSS version 20.0 and p-value <0.05 were considered statistically significant. RESULTS: 333 participants were recruited. The overall good adherence (≥95%) was approximated to be 60% (CI-54.3-65.1) when subjected to all three measures. On multivariable logistic regression, factors associated with higher odds of poor adherence were found to be caregivers aged 17-25 years [AOR = 3.5, 95%CI-(1.5-8.4)], children having an inter-current illness [AOR = 10.8, 95%CI-(2.3-50.4)], disbelief in ART effectiveness [AOR = 5.495; 95%CI-(1.669-18.182)] and advanced clinical stage [AOR = 1.972; 95% CI-(1.119-3.484)]. The major reasons reported by caregivers for missing medications included forgetfulness (41%), high pill burden (21%), busy schedule (11%) and long waiting hours at the clinic (9%). CONCLUSION AND RECOMMENDATIONS: In the urban setting of Dar es Salaam, ART adherence among children was found to be relatively low when combined adherence measures were used. Factors associated with poor ART adherence found were younger aged caregivers, and child intercurrent illness, while factors conferring good adherence were belief in ART effectiveness and lower HIV clinical stage. More attention and support should be given to younger aged caregivers, children with concomitant illness and advanced HIV clinical stages. Educating caregivers on ART effectiveness may also aid in improving adherence.

Measuring coverage and quality of supportive care for inpatient neonatal infections: EN-BIRTH multi-country validation study.

Background: An estimated 7 million episodes of severe newborn infections occur annually worldwide, with half a million newborn deaths, most occurring in low- and middle-income countries. Whilst injectable antibiotics are necessary to treat the infection, supportive care is also crucial in ending preventable mortality and morbidity. This study uses multi-country data to assess gaps in coverage, quality, and documentation of supportive care, considering implications for measurement. Methods: The EN-BIRTH study was conducted in five hospitals in Bangladesh, Nepal, and Tanzania (July 2017-July 2018). Newborns with an admission diagnosis of clinically-defined infection (sepsis, meningitis, and/or pneumonia) were included. Researchers extracted data from inpatient case notes and interviews with women (usually the mothers) as the primary family caretakers after discharge. The interviews were conducted using a structured survey questionnaire. We used descriptive statistics to report coverage of newborn supportive care components such as oxygen use, phototherapy, and appropriate feeding, and we assessed the validity of measurement through survey-reports using a random-effects model to generate pooled estimates. In this study, key supportive care components were assessment and correction of hypoxaemia, hyperbilirubinemia, and hypoglycaemia. Results: Among 1015 neonates who met the inclusion criteria, 89% had an admission clinical diagnosis of sepsis. Major gaps in documentation and care practices related to supportive care varied substantially across the participating hospitals. The pooled sensitivity was low for the survey-reported oxygen use (47%; 95% confidence interval (CI) = 30%-64%) and moderate for phototherapy (60%; 95% CI = 44%-75%). The pooled specificity was high for both the survey-reported oxygen use (85%; 95% CI = 80%-89%) and phototherapy (91%; 95% CI = 82%-97%). Conclusions: The women's reports during the exit survey consistently underestimated the coverage of supportive care components for managing infection. We have observed high variability in the inpatient documents across facilities. A standardised ward register for inpatient small and sick newborn care may capture selected supportive care data. However, tracking the detailed care will require standardised individual-level data sets linked to newborn case notes. We recommend investments in assessing the implementation aspects of a standardised inpatient register in resource-poor settings.

Prevalence of iron deficiency and iron deficiency anaemia among children with congenital heart defects at tertiary hospitals in Dar es Salaam, Tanzania: a cross-sectional study.

Introduction: iron deficiency (ID) is the most prevalent nutritional problem worldwide with children being the most vulnerable. In children with congenital heart defect (CHD), ID may lead to iron deficiency anaemia (IDA) which carries a poor prognosis due to exacerbation of left ventricular dysfunction and heart failure. This study assessed the prevalence and factors associated with ID and IDA among children with CHD at Muhimbili National Hospital (MNH) and Jakaya Kikwete Cardiac Institute (JKCI) in Tanzania. Methods: a descriptive hospital-based cross-sectional study was conducted among 238 participants with echocardiography confirmed CHD presenting at MNH and JKCI. A structured questionnaire was used to collect demographic data and medical history. Anthropometric measurements were done and blood samples for evaluation of complete blood count, serum ferritin and C-reactive protein were collected. Descriptive statistics such as frequencies, percentages, median with interquartile range, were used to describe study participants. Comparison of continuous variables was performed using Student's t-test or Mann-Whitney U-test as appropriate and Chi-square (x2) test or Fisher's exact test for categorical variables to determine associations. Odds ratio (OR) with 95% confidence intervals (CI) were estimated to determine risk factors for iron deficiency and iron deficiency anaemia. All analyses were conducted using SPSS version 20 and p-value ≤0.05 was considered statistically significant. Results: characteristic of study participant majority 66.4% (n 158) were less than 60 month of age with nearly equal number of male 51.3%(122) to female 48.7% (n 116). The overall prevalence of anaemia among study participants was 47.5% (n 238) with mild, moderate and severe anaemia being 21.4%, 21.4% and 4.6% respectively. The prevalence of iron deficiency was 26.9% (n 64) and that of iron deficiency anaemia was 20.2% (n 48). Age below 5 years, cyanotic CHD, history of recent illness and less consumption of red meat were significantly associated with iron deficiency (ID) and iron deficiency anaemia (IDA). After controlling for independent variables, history of recent illness aOR 0.46, 95% CI 0.22-0.98 P 0.045 less frequent consumption of red meat aOR 0.11 95% CI 0.04-0.32 P <0.001 and cyanotic CHD aOR: 0.40, 95% CI 0.18-0.87; p 0.021, were associated with of iron deficiency similarly age below 5 years aOR 0.33 0, 95% CI 14-0.89 P 0.02 early weaning practices aOR 0.50 95% CI 0.23-0.97 P 0.050 less frequent consumption of red meat aOR 0.07 CI (0.02-0.24 p <0.01 were significantly associated with iron deficiency anaemia. Conclusion: nearly half of the children with CHD in this study had anaemia, more than a quarter had ID and one-fifth had IDA. Routine screening and management of both ID and IDA in children with CHD should be practised during weaning and throughout the childhood to prevent ventricular dysfunction further heart failure.

Immediate "Kangaroo Mother Care" and Survival of Infants with Low Birth Weight.

BACKGROUND: "Kangaroo mother care," a type of newborn care involving skin-to-skin contact with the mother or other caregiver, reduces mortality in infants with low birth weight (<2.0 kg) when initiated after stabilization, but the majority of deaths occur before stabilization. The safety and efficacy of kangaroo mother care initiated soon after birth among infants with low birth weight are uncertain. METHODS: We conducted a randomized, controlled trial in five hospitals in Ghana, India, Malawi, Nigeria, and Tanzania involving infants with a birth weight between 1.0 and 1.799 kg who were assigned to receive immediate kangaroo mother care (intervention) or conventional care in an incubator or a radiant warmer until their condition stabilized and kangaroo mother care thereafter (control). The primary outcomes were death in the neonatal period (the first 28 days of life) and in the first 72 hours of life. RESULTS: A total of 3211 infants and their mothers were randomly assigned to the intervention group (1609 infants with their mothers) or the control group (1602 infants with their mothers). The median daily duration of skin-to-skin contact in the neonatal intensive care unit was 16.9 hours (interquartile range, 13.0 to 19.7) in the intervention group and 1.5 hours (interquartile range, 0.3 to 3.3) in the control group. Neonatal death occurred in the first 28 days in 191 infants in the intervention group (12.0%) and in 249 infants in the control group (15.7%) (relative risk of death, 0.75; 95% confidence interval [CI], 0.64 to 0.89; P = 0.001); neonatal death in the first 72 hours of life occurred in 74 infants in the intervention group (4.6%) and in 92 infants in the control group (5.8%) (relative risk of death, 0.77; 95% CI, 0.58 to 1.04; P = 0.09). The trial was stopped early on the recommendation of the data and safety monitoring board owing to the finding of reduced mortality among infants receiving immediate kangaroo mother care. CONCLUSIONS: Among infants with a birth weight between 1.0 and 1.799 kg, those who received immediate kangaroo mother care had lower mortality at 28 days than those who received conventional care with kangaroo mother care initiated after stabilization; the between-group difference favoring immediate kangaroo mother care at 72 hours was not significant. (Funded by the Bill and Melinda Gates Foundation; Australian New Zealand Clinical Trials Registry number, ACTRN12618001880235; Clinical Trials Registry-India number, CTRI/2018/08/015369.).

Neonatal resuscitation: EN-BIRTH multi-country validation study.

BACKGROUND: Annually, 14 million newborns require stimulation to initiate breathing at birth and 6 million require bag-mask-ventilation (BMV). Many countries have invested in facility-based neonatal resuscitation equipment and training. However, there is no consistent tracking for neonatal resuscitation coverage. METHODS: The EN-BIRTH study, in five hospitals in Bangladesh, Nepal, and Tanzania (2017-2018), collected time-stamped data for care around birth, including neonatal resuscitation. Researchers surveyed women and extracted data from routine labour ward registers. To assess accuracy, we compared gold standard observed coverage to survey-reported and register-recorded coverage, using absolute difference, validity ratios, and individual-level validation metrics (sensitivity, specificity, percent agreement). We analysed two resuscitation numerators (stimulation, BMV) and three denominators (live births and fresh stillbirths, non-crying, non-breathing). We also examined timeliness of BMV. Qualitative data were collected from health workers and data collectors regarding barriers and enablers to routine recording of resuscitation. RESULTS: Among 22,752 observed births, 5330 (23.4%) babies did not cry and 3860 (17.0%) did not breathe in the first minute after birth. 16.2% (n = 3688) of babies were stimulated and 4.4% (n = 998) received BMV. Survey-report underestimated coverage of stimulation and BMV. Four of five labour ward registers captured resuscitation numerators. Stimulation had variable accuracy (sensitivity 7.5-40.8%, specificity 66.8-99.5%), BMV accuracy was higher (sensitivity 12.4-48.4%, specificity > 93%), with small absolute differences between observed and recorded BMV. Accuracy did not vary by denominator option. < 1% of BMV was initiated within 1 min of birth. Enablers to register recording included training and data use while barriers included register design, documentation burden, and time pressure. CONCLUSIONS: Population-based surveys are unlikely to be useful for measuring resuscitation coverage given low validity of exit-survey report. Routine labour ward registers have potential to accurately capture BMV as the numerator. Measuring the true denominator for clinical need is complex; newborns may require BMV if breathing ineffectively or experiencing apnoea after initial drying/stimulation or subsequently at any time. Further denominator research is required to evaluate non-crying as a potential alternative in the context of respectful care. Measuring quality gaps, notably timely provision of resuscitation, is crucial for programme improvement and impact, but unlikely to be feasible in routine systems, requiring audits and special studies.

"Every Newborn-BIRTH" protocol: observational study validating indicators for coverage and quality of maternal and newborn health care in Bangladesh, Nepal and Tanzania.

BACKGROUND: To achieve Sustainable Development Goals and Universal Health Coverage, programmatic data are essential. The Every Newborn Action Plan, agreed by all United Nations member states and >80 development partners, includes an ambitious Measurement Improvement Roadmap. Quality of care at birth is prioritised by both Every Newborn and Ending Preventable Maternal Mortality strategies, hence metrics need to advance from health service contact alone, to content of care. As facility births increase, monitoring using routine facility data in DHIS2 has potential, yet validation research has mainly focussed on maternal recall surveys. The Every Newborn - Birth Indicators Research Tracking in Hospitals (EN-BIRTH) study aims to validate selected newborn and maternal indicators for routine tracking of coverage and quality of facility-based care for use at district, national and global levels. METHODS: EN-BIRTH is an observational study including >20 000 facility births in three countries (Tanzania, Bangladesh and Nepal) to validate selected indicators. Direct clinical observation will be compared with facility register data and a pre-discharge maternal recall survey for indicators including: uterotonic administration, immediate newborn care, neonatal resuscitation and Kangaroo mother care. Indicators including neonatal infection management and antenatal corticosteroid administration, which cannot be easily observed, will be validated using inpatient records. Trained clinical observers in Labour/Delivery ward, Operation theatre, and Kangaroo mother care ward/areas will collect data using a tablet-based customised data capturing application. Sensitivity will be calculated for numerators of all indicators and specificity for those numerators with adequate information. Other objectives include comparison of denominator options (ie, true target population or surrogates) and quality of care analyses, especially regarding intervention timing. Barriers and enablers to routine recording and data usage will be assessed by data flow assessments, quantitative and qualitative analyses. CONCLUSIONS: To our knowledge, this is the first large, multi-country study validating facility-based routine data compared to direct observation for maternal and newborn care, designed to provide evidence to inform selection of a core list of indicators recommended for inclusion in national DHIS2. Availability and use of such data are fundamental to drive progress towards ending the annual 5.5 million preventable stillbirths, maternal and newborn deaths.

A qualitative study on the voluntariness of counselling and testing for HIV amongst antenatal clinic attendees: do women have a choice?

BACKGROUND: Mother-to-child transmission (MTCT) of the Human Immunodeficiency -Virus (HIV) is a serious public health problem, contributing up to 90% of childhood HIV infections. In Tanzania, the prevention-of-mother-to-child-transmission (PMTCT) feature of the HIV programme was rolled out in 2000. The components of PMTCT include counselling and HIV testing directed at antenatal clinic attendees. It is through the process of Provider Initiated Counseling and Testing (PITC) that counselling is offered participant confidentiality and voluntariness are upheld and valid consent obtained. The objective of the study was to explore antenatal clinic attendees' experiences of the concept of voluntariness vis- a- vis the implementation of prior counseling and subsequent testing for HIV under the PITC as part of their antenatal care. METHODS: In-depth interviews were conducted with17 antenatal clinic attendees and 6 nursing officers working at the Muhimbili National Hospital (MNH) antenatal clinic. The study data were analyzed using qualitative content analysis. RESULTS: Antenatal clinic attendees' accounts suggested that counselling and testing for HIV during pregnancy was voluntary, and that knowledge of their HIV status led them to access appropriate treatment for both mother and her newborn baby. They reported feeling no pressure from nursing officers, and gave verbal consent to undergo the HIV test. However, some antenatal clinic attendees reported pressure from their partners to test for HIV. Healthcare providers were thus faced with a dilemma of disclosure/ nondisclosure when dealing with discordant couples. CONCLUSION: Antenatal clinic attendees at MNH undertook the PITC for HIV voluntarily. This was enhanced by their prior knowledge of HIV, the need to prevent mother- to- child transmission of HIV, and the effectiveness of the voluntary policy implemented by nursing officers.

Class II pentalogy of Cantrell.

BACKGROUND: Pentalogy of Cantrell is a rare syndrome, first described by Cantrell and co-workers in 1958. The syndrome is characterized by the presence of five major congenital defects involving the diaphragm, abdominal wall, the diaphragmatic pericardium, lower sternum and various congenital intra-cardiac abnormalities. The syndrome has never been reported in Tanzania, although may have been reported from other African countries. Survival rate of the complete form of pentalogy of Cantrell is as low as 20%, but recent studies have reported normal growth achieved by 6 years of age where corrective surgeries were done; showing that surgical repair early in life is essential for survival. CASE PRESENTATION: The African baby residing in Tanzania was referred from a district hospital on the second day of life. She was noted to have a huge omphalocele and ectopia cordis covered by a thin membrane, with bowels visible through the membrane and the cardiac impulse visible just below the epigastrium. Despite the physical anomaly, she appeared to saturate well in room air and had stable vitals. Her chest X-ray revealed the absence of the lower segments of the sternum and echocardiography showed multiple intra-cardiac defects. Based on these findings, the diagnosis of pentalogy of Cantrell was reached. On her fifth day of life, the neonate was noted to have signs of cardiac failure characterized by easy fatigability and restlessness during feeding. Cardiac failure treatment was initiated and she was discharged on parents' request on the second week of life. Due to inadequate facilities to undertake this complex corrective surgery, arrangements were being made to refer her abroad. In the meantime, her growth and development was satisfactory until the age of 9 months, when she ran out of the medications and succumbed to death. Her parents could no longer afford transport cost to attend the monthly clinic visits, where the infant was getting free medication refill. CONCLUSIONS: The case reported here highlights that in resource limited settings; poor outcome in infants with complex congenital anomalies is a function of multiple factors. However, we believe that surgery would have averted mortality in this 9-month-old female infant. We hope to be able to manage these cases better in future following the recent establishment of cardiac surgery facilities at Muhimbili National Hospital.