Neonatal herpes simplex virus encephalitis with intracranial bleeding in a newborn baby with concurrent rhesus incompatibility.

We report a baby with neonatal herpes simplex virus (HSV) encephalitis concurrent with Rrhesus (Rh) incompatibility. He was delivered by a Ggravida 2 mother with a history of miscarriage in her previous pregnancy at a gestation age of 4 months. She had Bblood group 0 and Rrhesus negative. The baby was noticed to have jaundice on day one1 of life accompanied by generalised petechiae on the face and upper chest. A full blood picture revealed severe anaemia and severe thrombocytopaenia and HSV 1/2 IgM was positive. MRI of the brain showed multiple extensive haemorrhagic lesions on the frontal-temporal regions.

Osteogenesis imperfecta type VIII: highlighting the need for genetic testing.

We report a severe form of osteogenesis imperfecta (OI) type VIII from a lower-middle income country. This is the first case report of this type in Tanzania. The term neonate was delivered normally via spontaneous vaginal delivery and presented at the neonatal unit with features of shortened limb girdles and macrocephaly. The long bones had multiple fractures. He was diagnosed clinically to have OI or a type of metaphysial dysplasia. A plain X-ray showed multiple fractures of the long bones. The eyes did not have blue sclerae. Clinically, the generic diagnosis of OI was made.Genetic testing revealed typical prolyl 3-hydroxylase 1 (P3HI) gene mutations and a variant coordinate NM_001243246.1:c.1095C>G p, indicating a severe, fatal form of autosomal-recessive OI type VIII which presents with white sclerae. This rare variant is described here for the first time in our setting. This case highlights the need for genetic testing.

Bilateral cryptophthalmos with overlapping features of Manitoba oculo-tricho-anal (MOTA) syndrome and Fraser syndrome 2.

A male baby with bilateral cryptophthalmos without eyebrows, distorted anterior hairline, bifid nasal tip, low-set ears, hypertelorism and low anorectal anomaly who was phenotypically diagnosed with Manitoba oculo-tricho-anal syndrome (mutation in FREM1 gene) had an overlapping genotypic diagnosis of autosomal recessive Fraser syndrome 2 because of the presence of a closely related mutation in FREM2 This heterozygous variant was likely to be sporadic. Another mutation was identified in the CEP85L gene indicating lissencephaly 10. This genetic condition has abnormal gyri pattern in the occiput area. This form of lissencephaly is characterised by phenotypic heterogeneity whereby some patients have only mild mental retardation, while others have a very complex clinical picture.In conclusion, this rare condition with the overlap of genetics between several conditions highlights the need for genetic testing even in an low middle income country (LMIC).

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.

Post-auricular teratoma in an HIV-exposed newborn.

Teratomas in the neonatal age group are mostly benign at first, and the common site is the sacrococcygeal region. They are rarely associated with HIV infection. We report a case of an HIV-exposed newborn with a congenital teratoma at the post-auricular site who developed an infection. Early intervention by total surgical resection will prevent complications such as infections and malignant transformation. A term baby was delivered spontaneously by an HIV-positive mother who was on her regular medications. Prenatal ultrasound carried out in the third trimester showed a cyst swelling on the right post-auricular region. Radiological imaging and the histopathological result revealed a congenital teratoma. A wide major excision with preservation of the facial nerve was performed at the age of 8 weeks. Post-auricular teratomas are the rarest anatomical location and the prevalence of malignant transformation from benign is very low. If left untreated, this tumour is associated with high mortality and malignant transformation rates. An early complete surgical excision allows a good result with a low risk of complications and recurrence.

Erythema multiforme in a young adult following COVID-19 infection and vaccination in Tanzania.

We report the case of a young female adult in her early 20s, who had COVID-19 infection for 8 weeks and COVID-19 vaccination 4 weeks prior to presentation with an extensive rash associated with erythema multiforme, resembling varicella zoster on initial presentation. After initial acyclovir therapy with no improvement, systemic corticosteroid treatment dramatically resolved the patient's skin rash.

Guillain-Barré syndrome associated with COVID-19 infection.

We are reporting a case of Acute Post-Infectious Flaccid paralysis also commonly known as Guillain-Barré Syndrome (GBS) in a patient with confirmed COVID-19 infection. GBS often occurs following an infectious trigger which induces autoimmune reaction causing damage to peripheral nerves. So far, only 8 cases have been described in association with COVID-19. This is the first to be described in Tanzania in an African Child, and probably the first in the continent. This report is presented for clinicians to be aware and for the medical fraternity to look into this unusual presentation which may shed some more light on possible pathways of the pathogenesis and clinical manifestations. We recommend that the presentation of GBS with acute respiratory distress should warrant extra precaution and a testing for COVID-19 especially when the symptoms of COVID-19 are protean.

Guillain- Barré syndrome associated with COVID-19 infection

We are reporting a case of Acute Post-Infectious Flaccid paralysis also commonly known as Guillain-Barré Syndrome (GBS) in a patient with confirmed COVID-19 infection. GBS often occurs following an infectious trigger which induces autoimmune reaction causing damage to peripheral nerves. So far, only 8 cases have been described in association with COVID-19. This is the first to be described in Tanzaniain an African Child, and probably the first in the continent. This report is presented for clinicians to be aware and for the medical fraternity to look into this unusual presentation which may shed some more light on possible pathways of the pathogenesis and clinical manifestations. We recommend that the presentation of GBS with acute respiratory distress should warrant extra precaution and a testing for COVID-19 especially when the symptoms of COVID-19 are protean

Risk factors and outcomes of fetal macrosomia in a tertiary centre in Tanzania: a case-control study.

BACKGROUND: Fetal macrosomia is defined as birth weight ≥4000 g. Several risk factors have been shown to be associated with fetal macrosomia. There has been an increased incidence of macrosomic babies delivered and the antecedent complications. This study assessed the risk factors, maternal and neonatal complications of fetal macrosomia in comparison with normal birth weight neonates. METHODS: A case-control study was conducted at the Muhimbili National Hospital (MNH) maternity and neonatal wards. Cases comprised of neonates with birth weight ≥4000 g; controls were matched for sex and included neonates weighing 2500-3999 g. Detailed clinical and demographic information and laboratory investigations which included blood glucose, hematocrit and plasma calcium were collected. The child was followed up to discharge/death. RESULTS: The prevalence of macrosomic babies was 2.3 % (103 out of 4528 deliveries). Mean birth weight of macrosomic babies was 4.2 ± 0.31 kg whereas in the controls it was 3.2 ± 0.35 kg. Maternal weight ≥80 kg, maternal age ranging between 30 and 39 years, multiparity, presence of diabetes mellitus, and gestational age ≥40 years, previous history of fetal macrosomia and delivery weight ≥80 kg were significantly associated with fetal macrosomia. Macrosomic infants were more likely to have birth asphyxia, shoulder dystocia, hypoglycemia, respiratory distress and perinatal trauma and increased risk of death compared to controls. Maternal complications such as postpartum hemorrhage, second degree perineal tears and prolonged labor occurred more frequently in the macrosomia group compared to controls (p-value <0.05), while shoulder dystocia, uterine rupture and maternal death were recorded only among the cases and none occurred in the controls. CONCLUSION: Fetal macrosomia was an important cause of maternal and neonatal morbidity at Muhimbili National Hospital. Presence of risk factors should alert the obstetrician to closely monitor these pregnancies and plan on appropriate mode of delivery. Macrosomic neonates should be routinely screened and appropriately managed for hypoglycemia.