Alobar holoprosencephaly with cebocephaly in a neonate: A rare case report from Northern Tanzania.

INTRODUCTION AND IMPORTANCE: Holoprosencephaly is a rare brain malformation consisting of impaired midline cleavage of the embryonic forebrain presenting with variable features of craniofacial dysmorphism. It affects 1 in 10,000 live births occurring more in females than males. We present a case of alobar HPE and aim to raise awareness on the importance of early prenatal detection and counselling. CASE PRESENTATION: We present a case of 3200-gram female baby, born by spontaneous vaginal delivery with APGAR scores of 5 and 6 in the first and fifth minute of life respectively. On admission, the baby was lethargic, had central and peripheral cyanosis, hypothermic with temperature of 32.1 °C, respiratory rate of 65 breaths/min, heart rate of 135 beats/min and oxygen saturation of 94% with an oropharyngeal airway and on oxygen support via a face mask. She had microcephaly, hypotelorism, and a small nose with a single imperforate nostril. She was diagnosed to have alobar holoprosencephaly with cebocephaly. A computed tomography scan of the brain revealed a cephalohematoma in the vertex and an intranasal soft tissue density lesion blocking the entrance measuring approximately 10 × 8.5 mm. Absence of the corpus callosum and septum pellucidum with a resulting monoventricle formed from the lateral ventricles, the fusion of the thalami and a sizeable arachnoid cyst involving the left cerebellar hemisphere were evident. She was started on IV antibiotics and IV fluids. Non-invasive airway management was opted for by the ENT team based on the condition of the baby. She succumbed to death 6 days post admission due to severe respiratory failure. CLINICAL DISCUSSION: The types of HPE are alobar, semi lobar, lobar and interhemispheric variants. Alobar HPE is the most severe form and is incompatible with life. Clinical presentation entails facial dysmorphism with features of hypotelorism, microcephaly and a blind ended nostril. Alobar and semilobar HPE can reliably be diagnosed with ultrasound during the first and second trimesters of pregnancy. Absence of choroid plexus and fused cortex are pathognomonic characteristic on ultrasound and CT scan respectively. CONCLUSION: Alobar holoprosencephaly is a rare brain malformation which is incompatible with life. Prenatal ultrasound screening of the foetus brain is essential and reliable in making a diagnosis. Absence of the "butterfly" sign in the foetal brain ultrasonography should raise a high index of suspicion for brain malformation with unfavourable outcome. Legal medical termination of pregnancy may serve as an early intervention.

Ileal Atresia in a 10-day-old male baby: A case report.

Ileal Atresia is noted to be the commonest cause of intestinal obstruction in neonates worldwide but still under diagnosed in Sub-Saharan countries with scarce data reported on its occurrence. It is likely under diagnosed due to low index of suspicion. Advancement in foetal ultrasound during prenatal period will increase index of suspicion and hence early diagnosis and correction. This is the first case report in our setup highlighting this condition. We present a case of a 10-day-old male baby referred to us due to vomiting since birth. Clinically was in distress with a palpable supraumbilical mass that was firm and non-tender. Abdominal ultrasound scan showed poor peristalsis and minimal dilation of bowels suggestive of partial intestinal obstruction. Abdominal X-ray showed dilated bowels with multiple air-fluid levels, empty rectum with features suggestive of intestinal obstruction. Patient was successfully operated but unfortunately on day 3 post-surgery succumbed.

Necrotizing fasciitis in a 5-week-old infant: An unusual presentation.

Necrotizing fasciitis is a rare and life-threatening soft-tissue infection characterized by rapidly spreading inflammation and subsequent necrosis of the fascial planes and surrounding tissue. These children should be managed promptly with broad-spectrum antibiotics and possible surgical debridement. Here, we present a 5-week-old female infant who presented with fever and ulcer on right lower jaw. She had a sudden onset of darkening of the right lower jaw, which progressed to reddish discoloration, and then an ulcer developed in the middle of this area of discoloration. She had a tender swelling extending from the right pre-auricular area to the right upper neck, crossing the right jaw and extending toward the right cheek and maxillary area. Swelling had areas of hyperpigmentation with an ulcer measuring 2 by 3 cm at the center of the lesion, oozing purulent fluid, and Staphylococcus aureus was cultured. The surgeons conducted incision and debridement in theater after suspecting necrotizing fasciitis of the jaw and the baby was kept on intravenous antibiotics, antiretroviral therapy and was dressed daily. The child fared well thereafter.

Ectopia cordis: A case report of pre-surgical care in resource-limited setting.

INTRODUCTION AND IMPORTANCE: Ectopia cordis is a rare congenital malformation of thoracic midline fusion that presents as location of the heart outside the open chest cavity. This presents as a surgical emergency and demands early and specialized intervention. Particularly in resource-limited settings, where prenatal ultrasonography screening is not done, these children are often born in facilities without the capability of managing such conditions definitively, necessitating them to be referred to a specialized centre. At lower health facilities, the challenge is in ensuring that the child is kept stable and protected from infection until they can reach a centre with the facilities required for care. This report describes the management give to such a child until they were successfully handed over to a cardiac institute. CASE PRESENTATION: We present a newborn male baby delivered at term to a mother from a low socio-economic background with his heart and abdominal viscera outside the thoracic and abdominal cavity. Despite presenting at a centre without cardiac surgery facilities or cardiologists, they were sustained until referral. CLINICAL DISCUSSION: Ectopia cordis is a rare congenital anomaly characterized by defect in the fusion of the anterior chest wall resulting in the abnormal extra-thoracic location of the heart. Five types exist; cervical type with worst prognosis, attempts can be made to re-locate the heart and close the thoracic defect surgically. CONCLUSION: Even with limited resources, it is possible to provide the basic care necessary to sustain a child with this complex anomaly until definitive management can be provided.

Effectiveness of FIBEROPTIC phototherapy compared to conventional phototherapy in treating HYPERBILIRUBINEMIA amongst term neonates: a randomized controlled trial.

BACKGROUND: Neonatal jaundice is one of the most common problems in neonates. Effective treatment of jaundice requires therapeutic intervention with high quality phototherapy. Over recent years, several studies reported fiberoptic phototherapy to be less effective than conventional phototherapy in term neonates. Our study aimed to compare the effectiveness of fiberoptic phototherapy with a larger illuminated area and higher irradiance to conventional phototherapy methods. METHODS: This was a randomized controlled trial conducted at the Kilimanjaro Christian Medical Centre (KCMC). A total of 41 term neonates, less than 7 days of age with unconjugated hyperbilirubinemia were randomized. Thirteen (13) neonates were allocated to receive fiberoptic phototherapy, 13 to blue light conventional phototherapy and 15 to white light conventional phototherapy. Effectiveness was assessed by comparing the duration of phototherapy, bilirubin reduction rate and side effects of treatment. The data was analyzed with the independent t-test. RESULTS: The mean overall bilirubin reduction rate was comparable in the fiberoptic phototherapy group (0.74%/h) and the blue light conventional phototherapy group (0.84%/h), with no statistically significant difference (p-value 0.124). However, white light conventional phototherapy had a significantly lower mean overall bilirubin reduction rate (0.29%/h) as compared to fiberoptic phototherapy (p-value < 0.001). The mean treatment duration of phototherapy was 69 h, 68 h and 90 h in the fiberoptic, blue light conventional and white light conventional phototherapy groups respectively. Side effects such as loose stool and skin rash were noted in some participants who received conventional phototherapy. No side effects of treatment were noted in the fiberoptic phototherapy group. CONCLUSION: The effectiveness of fiberoptic PT and blue light conventional PT were comparable in terms of bilirubin reduction rate and treatment duration, whereas fiberoptic phototherapy was more effective than white light conventional PT, with a significantly higher bilirubin reduction rate and shorter treatment duration. Fiberoptic phototherapy may mitigate side effects caused by conventional phototherapy. TRIAL REGISTRATION: The Pan African Clinical Trial Registry, PACTR202004723570110 . Registered 22nd April 2020- Retrospectively registered.

Neonatal Polycystic Kidney Disease in a One-Day-Old Baby: A Case Report.

BACKGROUND: Polycystic kidney disease in neonates is a rare genetic disease which can be either autosomal dominant or autosomal recessive with each presenting at a certain period in life. They can both be diagnosed before or after birth using fetal ultrasound. This is a case of a five-hour-old baby with suspected polycystic kidney disease in a tertiary hospital in northern Tanzania. CASE PRESENTATION: We present a case of a five-hour-old female baby referred to us with a complaint of non-progressive abdominal distension since birth. The birth weight was 2.4 kilograms with a good APGAR score. Clinically, the baby had palpable kidneys bilaterally, widened anterior fontanelle communicating with the posterior as well as rocker bottom feet. Her abdominal ultrasound showed bilaterally enlarged echogenic kidneys with loss of cortico-medullary differentiation and multiple tiny cystic spaces. An echocardiogram showed patent ductus arteriosus and moderate tricuspid regurgitation with mild pulmonary regurgitation. The patient was started on medication but unfortunately on day two post admission the baby succumbed. CONCLUSION: Neonatal polycystic kidney disease is associated with high morbidity and mortality rates. It may not be as rare as previously reported. Minimal to no awareness exists on the condition or its effects in our setup due to underdiagnosis and neither availability of neonatal screening nor availability of genetic analysis. It is likely underdiagnosed due to a lack of skills in fetal ultrasounds and no neonatal ICU to care for these babies. Increased awareness will increase the index of suspicion. This is the first case report in our setup highlighting this condition.

Juvenile Polyposis Syndrome in a Young Girl from Northern Tanzania.

Introduction. Juvenile polyposis syndrome is a rare autosomal dominant disorder in children characterized by multiple polyps in the gastrointestinal tract. A variety of clinical features manifest, including prolapse of a polyp or entire rectum, gastrointestinal bleeding, anaemia, and intussusception. This condition if left unmanaged promptly leads to fatal complications including the development of cancer of the bowel. Case Presentation. A 13-year-old girl with a history of mass protrusion per anus associated with bloody diarrhea. Colonoscopy showed multiple polyps in her large bowel. She underwent total colectomy with ileorectal anastomosis and did clinically well post surgery with no complications. Conclusion. Juvenile polyposis syndrome is an inherited condition with significant morbidity and a high risk of colon malignancy. It is important for early screening and diagnosis and hence management in its early stages as there are no specific standard guidelines for children.

Isolated Pulmonary Hydatid Cyst: A Rare Presentation in a Young Maasai Boy from Northern Tanzania.

INTRODUCTION: Hydatidosis is a parasitic manifestation caused by Echinococcus granulosus. It is characterized by cystic lesions in the liver and lungs. Diagnosis is based on typical history and radiological measures. CASE PRESENTATION: A four-year-old boy presented with a one-year history of dry cough and difficulty in breathing which was of gradual progression. Computed tomography of the chest revealed a large 11.7 cm × 8.6 cm × 11.0 cm cyst in the right hemithorax. The patient underwent thoracotomy and recovered well post procedure. CONCLUSION: This case report highlights that large hydatid cysts can be surgically removed with good outcome and the importance of realizing that the disease is a burden to the public health and is much neglected.

Neuropsychiatric symptoms in Tanzanian HIV-infected children receiving long-term efavirenz treatment: a multicentre, cross-sectional, observational study.

BACKGROUND: Efavirenz is commonly prescribed for children with HIV infection, yet little is known about risks of neuropsychiatric side-effects. We aimed to compare competence (social involvement, activities, and school performance) and psychopathology (internalising and externalising problems), cognitive performance (intelligence and working memory), and adherence in Tanzanian children on an efavirenz-based versus a non-efavirenz-based regimen. METHODS: In this multicentre, cross-sectional, observational study, we included consecutive children (aged 6-12 years) with HIV infection, on combination antiretroviral therapy (cART) for at least 6 months, and with viral loads of less than 1000 copies per mL from HIV care clinics of three primary health facilities and three referral hospitals in Moshi, Kilimanjaro, Tanzania. Children with acute illnesses, medication switch in the 6 months before the study visit, and any history of brain injury or developmental delay before cART initiation were excluded. All interviews and assessments were done by trained local research nurses under the supervision of a medical doctor. The primary outcomes, competence and psychopathology, were measured with the Child Behavior Checklist. We used ANCOVA to assess differences between groups. This study is registered with ClinicalTrials.gov, number NCT03227653. FINDINGS: Between June 19, 2017, and Dec 14, 2017, 141 children were analysed, of whom 72 (51%) used efavirenz-based cART and 69 (49%) used non-efavirenz-based cART. After controlling for age, sex, and clinical and demographic confounders, we observed lower competence (adjusted mean difference -2·43 [95% CI -4·19 to -0·67], p=0·0071), largely driven by lower school performance scores (adjusted mean difference -0·91 [-1·42 to -0·40], p=0·00055), in the efavirenz group than in the non-efavirenz group. More total (adjusted mean difference 5·96 [95% CI -1·12 to 13·04], p=0·098) and internalising (adjusted mean difference 2·00 [-0·29 to 4·29], p=0·086) behavioural problems were seen in the efavirenz group than in the non-efavirenz group, although these findings were non-significant. No differences were found in externalising problems (adjusted mean difference 0·78 [95% CI -1·55 to 3·11], p=0·51). INTERPRETATION: Our results suggest that treatment with efavirenz in children is associated with a mild increase in neuropsychiatric symptoms, especially in children who receive doses higher than or equal to the WHO recommended doses for efavirenz. Clinical awareness and adequate follow-up of neuropsychiatric symptoms in efavirenz in children remain warranted. FUNDING: Aidsfonds, Radboud University Medical Center.

Prevalence and genotypes of Rotavirus among children under 5 years presenting with diarrhoea in Moshi, Tanzania: a hospital based cross sectional study.

BACKGROUND: Diarrhoea is a main cause of morbidity and mortality in children under 5 responsible for approximately four billion cases and 1.1 million deaths annually. In developing countries, it causes two million deaths each year. The major causative organism responsible is Rotavirus which is responsible for one-third of hospitalizations with approximately 40% mortality. RESULTS: The prevalence of Rotavirus infection was 26.4% (73/277). The predominant strain of Rotavirus found was G1 21/73 (53.8%), followed by G8 9/73 (23.1%), G12 5/73 (12.8%), G9 3/73(7.7%) and G4 1/73 (2.6%). All serotypes identified were in children who had completed Rotavirus vaccination except for one who had G8 in whom the vaccine was introduced after they had completed immunizations. CONCLUSION: The overall prevalence of rotavirus has reduced from 33.2% in 2009 to 26.4% in 2016. We have found G1 to be the predominant serotype as well as other circulating serotypes namely G4, G8, G9 and G12. Despite a reduction in prevalence, there is a need for further rotavirus surveillance in the region.

Prevalence, severity and early outcomes of hypoxic ischemic encephalopathy among newborns at a tertiary hospital, in northern Tanzania.

BACKGROUND: Hypoxic Ischemic Encephalopathy (HIE) remains a problem of great concern worldwide especially in developing countries. The occurrence of a neurological syndrome can be an indicator of insult to the brain. We aimed to determine the prevalence, HIE proportions, neurological signs and early outcomes of newborns that developed birth asphyxia at KCMC Tanzania. METHODS: A prospective study was conducted at KCMC from November 2014 to April 2015 among newborns with birth asphyxia. Sarnat and Sarnat score was used to assess newborns immediately after birth to classify HIE and were later followed daily for 7 days or until discharge. RESULTS: Of the 1752 deliveries during the study period, 11.5% (n = 201) had birth asphyxia. Of the 201 newborns, 187 had HIE. Of these 187 with HIE; 39.0% had moderate HIE and 10.2% had severe HIE according to the Sarnat and Sarnat classification. Neurological signs that were observed during the study period were; weak/absent reflexes (46.0%), hypotonia (43.3%) and lethargy (42.2%). Mortality was 9.1% among the 187 newborns with HIE. Mortality was higher among newborns with severe HIE 84.2% (16/19) compared to those with moderate HIE 1.4% (1/73). On the 7th day after delivery, 17.1% (32/187) of the newborns did not show any change from the initial score at delivery. CONCLUSION: Prevalence of birth asphyxia is high in our setting and most of the newborns (49%) end up with moderate/severe HIE. Good obstetric care and immediate resuscitation of newborns are vital in reducing the occurrence of HIE and improving the general outcome of newborns.

Juvenile dermatomyositis in a 4-year-old Kenyan girl.

To our knowledge, this is the first case report of juvenile dermatomyositis (JDM) in Tanzania. It demonstrates that the characteristic cutaneous findings of JDM may easily be overlooked, especially on dark skin, and the difficulty of clinical management in resource-constrained settings.

Diagnosis of human fascioliasis in Arusha region, northern Tanzania by microscopy and clinical manifestations in patients.

BACKGROUND: Human fascioliasis (HF) is a zoonotic disease that has been identified in many countries worldwide. This report concerns the identification and clinical management of cases of human fascioliasis in the suburbs of Arusha city, northern Tanzania in 2013. Fascioliasis is included among the WHO's Neglected Tropical Diseases as a plant transmitted trematode infection. Human fascioliasis has not been described before in the East Africa region, including Tanzania. METHODS: Patients presenting at a primary healthcare centre in Arusha Region, northern Tanzania provided fresh stool samples for routine ova and parasite screening (saline and iodine preparations). Subsequent stool samples were preserved in 5 % formalin in saline and subjected to ether sedimentation for examination. RESULTS: Out of 1460 patients, 305 (21 %) were diagnosed positive for fascioliasis based on the demonstration of brownish, oval eggs with inconspicuous opercula in stool. Two distinct egg sizes were identified; large 170-212.5 by 115-150 µm (mean 194.5 by 130.5 µm) and smaller eggs 120-150 by 87.5 - 112.5 µm (mean 138.8 by 101 µm). Clinically, patients presented with fever (39 - 40 °C) and abdominal pain. Some patients had pruritis around the mouth and their lips were swollen. 3 patients were treated and cured with single dose Triclabendazole. The remaining 302 patients were treated with Nitazoxanide and 122 (40 %) were cleared of infection with a single course. Snails of the genus Lymnaea were found in the surroundings. CONCLUSIONS: This report serves to remind medical professionals in East Africa that HF is a probable differential diagnosis in patients presenting with similar symptoms. It is possible to diagnose fascioliasis by light microscopy although specific antigen tests are required for confirmation. Human fascioliasis however, has not been described or reported in Tanzania before and begs further investigation.