Acute soft head syndrome in a teenager with sickle cell anemia: A case report.

Key Clinical Message: Sickle cell disease (SCD) rarely presents with acute soft head syndrome (ASHS) often posing a diagnostic dilemma. Recovery is typically spontaneous, however, in the context of lack of awareness and limited brain imaging it could potentially lead to poor outcome. Abstract: ASHS is a rare complication of SCD, invariably occurring near puberty with hitherto elusive pathogenic mechanisms. ASHS often resolves spontaneously on conservative management, however, lack of awareness in the context of limited access to brain imaging could pose diagnostic challenges resulting in inappropriate management and untoward outcome. We present a case of a teenager who presented with subtle symptoms for which the diagnosis of sickle cell anemia (SCA) was delayed until he developed ASHS. LTM was a 16 years old boy with a history of recurrent joints pain since the age of 6 years, with a family history of SCA, but had initial negative sickling test. He presented with episodes of multiple joints pain, unprovoked scalp and left orbital swelling, low-grade fever and mild headache without any evidence for bleeding diathesis. The diagnosis of SCA was confirmed by hemoglobin electrophoresis. Computed tomography (CT) scan of the head revealed subgaleal heamatoma (SGH) and intraorbital haematoma without intracranial hemorrhage (ICH). He was managed conservatively with analgesics and hydration together with antibiotics for associated sepsis with complete resolution of clinical symptoms within 2 weeks. This case represents a rare scenario for a relatively mild SCA phenotype presenting with ASHS whose diagnosis poses an enigma in the resource-limited contex. It is therefore, prudent to recognize ASHS to avoid judicious interventions which could potentially result in untoward clinical outcome.

Ruptured bilateral brain arteriovenous malformations in a young woman with early pregnancy: a case report.

BACKGROUND: Brain arteriovenous malformations (AVMs) are rare congenital developmental vascular lesions, and often presents with symptoms upon rupture. The controversy exists as to whether pregnancy confers an increased risk of intracranial hemorrhage. The diagnosis of brain AVMs, in the absence of brain imaging, is challenging in resource-limited settings, particularly in sub-Saharan Africa. CASE PRESENTATION: A 22-year old black African woman, primigravida at 14 weeks of gestation, presented with a history of persistent throbbing headache which was treated at primary health care facilities with analgesics and anti-migraine medications without relief. She later developed severe headache 2 weeks prior to admission and one-day history of serial partial generalized tonic-clonic seizures which were followed by post-ictal confusion and persistent right upper limb weakness. Initial evaluation revealed her to be pregnant and she later underwent a brain magnetic resonance angiography (MRA) at a university teaching hospital which revealed bleeding bilateral parietal AMVs with intracerebral haematoma and associated perilesional vasogenic oedema. The patient was managed conservatively using antifibrinolytic drugs and prophylactic anti-seizure drugs. Seven months later, she underwent a control brain MRA which revealed resolution of intracranial haematoma and associated vasogenic oedema and had her seizures well controlled. The headache had subsided and the pregnancy was allowed to continue to term under close obstetric and neurological observation. On follow up visits she reported episodes of nasal bleeding which upon ENT examination revealed nasal AVMs, suggesting the diagnosis of hereditary hemorrhagic telangiectasia (HHT). CONCLUSION: AVMs are rare but should prompt suspicion in young patients with atypical Central Nervous System (CNS) manifestations without evident underlying causes.

Prevalence and factors associated with renal dysfunction among children with sickle cell disease attending the sickle cell disease clinic at a tertiary hospital in Northwestern Tanzania.

BACKGROUND: Little is known on how the interaction between Sickle Cell Disease (SCD) and renal insults caused by other coexisting conditions in Sub Saharan Africa such as urinary schistosomiasis, malnutrition and HIV affect the prevalence of renal dysfunction in children with SCD. OBJECTIVES: To determine the prevalence and factors associated with renal dysfunction among children with SCD aged 6 months to 12 years attended at a tertiary hospital in Northwestern Tanzania. METHODS: A cross sectional hospital-based study with a short follow up component of 3 months for 153 children with SCD was done to document demographics, clinical characteristics and features of renal dysfunction including urine dipstick albuminuria (>20mg/l) and eGFR (<60ml/ml/min/1.73m2). Other potential renal insults such as HIV infection and Schistosomiasis were also evaluated. RESULTS: At enrollment, 48/153(31.37%) children had renal dysfunction declining to 31(20.3%) at 3 months follow up. Acute chest syndrome (OR 3.04, 95% CI [1.08-8.96], p = 0.044), severe anemia (OR 0.44, 95% CI [0.26-0.76],p = 0.003), urinary schistosomiasis (OR 7.43, 95% CI [2.10-26.32] p<0.002) and acute malnutrition (OR 4.92, 95% CI [1.29-18.84], p = 0.020). were associated with renal dysfunction. CONCLUSION: Where prevalent, urinary schistosomiasis and acute malnutrition increase the risk for renal dysfunction in children with SCD. We recommend albuminuria routine screening in children with SCD especially those presenting with acute chest syndrome, severe anemia and features of acute malnutrition for early detection of renal dysfunction among children with SCD.