Bilateral Cerebrovascular Accident in Pediatric Sickle Cell Anemia: A Cascade of Complications – A Case Report

doi:10.21203/rs.3.rs-7842997/v1

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Case Report

Bilateral Cerebrovascular Accident in Pediatric Sickle Cell Anemia: A Cascade of Complications – A Case Report

https://doi.org/10.21203/rs.3.rs-7842997/v1

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Background: Bilateral cerebrovascular accident (CVA) in pediatric sickle cell anemia (SCA) is an exceptionally rare neurological complication that amplifies disease burden. While unilateral ischemic stroke is a recognized manifestation of sickle cell vasculopathy, bilateral infarction is scarcely reported and often catastrophic. This case contributes novel clinical and rehabilitative insights into the multidimensional management of such presentations in a low-resource African setting.

Case presentation: A nine-year-old boy of Igbo ethnicity (HbSS) presented with three days of weakness and low-grade fever. During exchange blood transfusion he developed recurrent generalized tonic seizures, loss of consciousness, and subsequent quadriparesis. Cranial computed tomography revealed a large subacute left frontal infarct and chronic right frontal, parietal, and temporal infarcts, confirming bilateral ischemic CVA. Management included resuscitation with intravenous adrenaline, hydrocortisone, and phenobarbital; maintenance anticonvulsants (carbamazepine and levetiracetam); oxygen and antibiotics; and nutritional/hematinic support. Physiotherapy began on day 19, emphasizing spasticity reduction, joint mobility, trunk control, and caregiver training. After six physiotherapy sessions the child had modest improvements resolution of ankle clonus, mild reduction in upper-limb tone, and improved joint mobility, yet remained quadriparetic at discharge. He was conscious and seizure-free for 72 hours before discharge and referred for outpatient neurorehabilitation; the caregiver demonstrated competence in the home program.

Conclusion: This case highlights that early, context-sensitive rehabilitation even when limited by medical instability can preserve joint integrity, modulate tone, and empower caregivers, thereby improving quality of survival in pediatric SCA with stroke. The report documents the rare occurrence of bilateral CVA in pediatric SCA and supports integrating physiotherapy into acute multidisciplinary care.

Neurology

Pediatrics

Sickle cell anemia

Bilateral cerebrovascular accident

Pediatric cerebrovascular accident

Physiotherapy rehabilitation

Low-resource setting

Sickle cell anemia (SCA) is among the most prevalent inherited hemoglobinopathies worldwide, with Sub-Saharan Africa bearing the heaviest burden of disease [1]. Advances in neonatal screening, hydroxyurea therapy, and transcranial Doppler monitoring have transformed survival outcomes [2]. Yet, despite these gains, children with SCA remain vulnerable to recurrent vaso-occlusive crises, chronic anemia, infections, and, most alarmingly, cerebrovascular complications [3, 4]. Stroke, though relatively infrequent compared to other complications, is one of the most devastating, capable of leaving enduring cognitive, motor, and psychosocial impairments [5].

Bilateral Cerebrovascular Accident: An Unusual Catastrophe

Ischemic stroke in children with SCA is well-documented, with prevalence estimates ranging from 11% to 13% by the age of 20 years [6]. Classically, such strokes are unilateral and attributed to progressive vasculopathy of major cerebral arteries [7, 8]. However, bilateral cerebrovascular accident (CVA) remains exceedingly rare, representing a catastrophic event with compounded neurological sequelae. Recent reports indicate that strokes may even occur without cerebral arteriopathy, suggesting alternative or overlapping pathophysiological mechanisms [9, 10]. The presence of bilateral infarcts in a child not only magnifies the clinical burden but also challenges conventional assumptions about disease progression in SCA [2].

Beyond Survival: The Role of Rehabilitation

Stroke in pediatric SCA is not a single event but the beginning of a disabling trajectory. Survivors often face spasticity, weakness, and loss of functional independence, which can persist into adolescence and adulthood [11]. Rehabilitation particularly physiotherapy is critical to restoring motor control, preserving joint integrity, and enhancing quality of life [12, 13]. Evidence supports the role of structured physical activity in improving muscle strength and gross motor performance in children with SCA [14]. Yet, in many low-resource settings, access to such interventions is inconsistent, and the functional deficits following pediatric stroke remain under-addressed [15, 16].

Rationale for This Case Report

This report describes the case of a nine-year-old Igbo boy with SCA who experienced a cascade of complications beginning with bilateral CVA, complicated by recurrent seizures, quadriparesis, and functional decline. Such a presentation is exceptionally rare and underscores the fragile balance in managing pediatric SCA. Beyond documenting the unusual neurological trajectory, this case emphasizes the educational value of vigilant transfusion monitoring, timely diagnosis, and early rehabilitation integration in multidisciplinary care. It contributes to the scarce literature on bilateral pediatric CVA in SCA and highlights the dual challenges of acute stabilization and long-term functional recovery in low-resource contexts.

Patient Information

The patient was a nine-year-old Igbo boy from Abakpa Nike, Enugu State, Nigeria, who presented to the Department of Hematology and Oncology on 1 July 2025 with a three-day history of weakness and fever. He had been previously diagnosed with sickle cell anemia (HbSS genotype) one year earlier following an episode of cerebrovascular accident (CVA) managed at the University of Nigeria Teaching Hospital, Enugu. He is the first of three children in a monogamous family. His mother had been absent for three months prior to presentation, and he lived under the care of his paternal aunt, aged 43 years, who was a trader. His father, aged 35 years, worked with the Public Complaints Commission and provided intermittent support. The family’s socioeconomic background was modest, and the child was enrolled in a local primary school prior to his illness. There was no known family history of hematologic disorders or stroke, and immunization status was reportedly up to date. The patient had not been on routine follow-up at the sickle cell clinic at the time of presentation.

Clinical Findings on Initial Presentation

At presentation, the patient’s primary complaints were generalized weakness and intermittent low-grade fever for three days. The progressive weakness limited daily activity; the patient spent most of the day lying down. The fever was described as on and off, with partial relief after administration of paracetamol. There was no initial history of seizures at initial presentation. On physical examination, the patient appeared pale, mildly febrile (37.7°C), and warm to touch, with no signs of dehydration or respiratory distress. His weight was 26 kg, and vital signs were otherwise within acceptable limits for age. There was no cyanosis or jaundice at the time of admission. Neurological examination revealed that the patient was conscious and alert, with Glasgow Coma Scale (GCS) of 15/15. Pupils were equal and reactive to light. There was hypertonia of the left upper and lower limbs, while the right limbs demonstrated normal tone. Ankle clonus was present on the left side. Deep tendon reflexes were brisk on the left and normal on the right. There was no cranial nerve deficit noted at this stage, and sensation was intact. Systemic examination findings were otherwise unremarkable: respiratory examination showed good bilateral air entry without adventitious sounds, and the abdomen was soft with mild epigastric tenderness but no organomegaly. Cardiovascular and musculoskeletal examinations were within normal limits. In summary, the initial clinical impression was that of a known sickle cell anemia patient presenting with left-sided upper motor neuron signs following a suspected recurrent cerebrovascular event.

Early Admission Phase (1–2 July 2025)

Initial evaluation revealed mild anemia and hypertonia of the left limbs. He commenced routine hematologic care, and exchange blood transfusion (EBT) was planned to optimize oxygen-carrying capacity. However, approximately 20 minutes into the EBT, the child developed severe abdominal pain, restlessness, and generalized tonic seizures, followed by loss of consciousness and absent peripheral pulses. Immediate resuscitation was instituted with intravenous adrenaline, hydrocortisone, and diazepam, followed by intravenous phenobarbital to abort status epilepticus. After 10 seizure episodes, his GCS dropped to 7/15, prompting transfer to the Pediatric Intensive Care Unit (PICU) for advanced monitoring.

Intermediate Course (3–8 July 2025)

In the PICU, he remained unconscious (GCS 10/15), with sluggish pupillary reaction and intermittent tonic seizures involving the right upper and lower limbs. Laboratory investigations revealed hypokalemia (K⁺ 2.9 mmol/L), which was corrected with intravenous potassium chloride infusion. Despite stabilization, he developed recurrent febrile spikes and required antibiotic therapy with intravenous ceftriaxone, antipyretics, and tepid sponging.

Neurological Imaging and Progression (9–12 July 2025)

A cranial CT scan demonstrated a large subacute ischemic infarct in the left frontal lobe and a chronic infarct involving the right frontal, parietal, and temporal lobes with cystic encephalomalacia, dystrophic calcification, and cortical atrophy, consistent with bilateral cerebrovascular accident. The patient’s condition further deteriorated with increasing respiratory distress, jaundice, and hepatomegaly.

Late Course (13–31 July 2025)

Between 13 and 31 July, the child experienced recurrent seizures, despite anticonvulsant therapy with carbamazepine and diazepam. Physiotherapy commenced on 19 July, focusing on pain relief, spasticity reduction, and maintenance of joint range. At this time, he exhibited spasticity of both upper limbs and the left lower limb, tightness of the right tendoachilles, and marked functional dependency. Over six therapy sessions, the patient showed slight improvement in tone and ankle clonus but remained quadriparetic and unable to perform basic activities of daily living. On 31 July 2025, the caregiver requested discharge. The patient was referred to the Pediatric Physiotherapy Outpatient Unit for continued rehabilitation, with follow-up scheduled for 12 August 2025, see Table 1.

Table 1
Summary Timeline of Clinical Course
Date	Event / Review	Clinical Findings	Interventions / Actions	Impression / Remarks
1 July 2025	Admission to Hematology/Oncology Unit	Weakness ×3 days, low-grade fever; pale, mildly febrile (37.7°C); hypertonia of left UL & LL; ankle clonus + on left	Baseline assessment; plan for exchange blood transfusion (EBT)	Recurrent CVA in a known SCA patient
2 July 2025	During EBT	Developed abdominal pain → generalized tonic seizure → unresponsive (GCS 3/15), absent peripheral pulse	IV adrenaline, IV hydrocortisone, IV diazepam; seizure persisted → IV phenobarbital; urgent RBC 236 mg/dL	Status epilepticus; post-ictal state; possible transfusion reaction
3 July 2025	Head of Ward Review (HOWR)	Unconscious, GCS 10/15; pupils mid-sized, sluggish reaction	Continued PICU care	Repeated CVA; ? transfusion-induced anaphylactic shock
4 July 2025	PICU Review / SROC	Persistent seizures involving right UL & LL; serum K⁺ = 2.9 mmol/L	4.5 mmol IV KCl infused over 2 hours	Hypokalemia corrected; continue anticonvulsants
5–6 July 2025	Ongoing management	Febrile spikes (37.8–38.2°C)	Paracetamol, tepid sponging	Supportive therapy
7 July 2025	Pediatric Neurology Review	Still unconscious (GCS 10/15); seizure 2 days earlier; intermittent tonic seizures	Carbamazepine 100 mg, IV ceftriaxone 200 mg, omega-3, multivitamin, folic acid	Recurrent CVA with seizure disorder
8 July 2025	HOWR	Two seizure episodes (30 s each); K⁺ = 4.2 mmol/L; mild tachypnea (38 cpm)	Stopped KCl; continued IV ceftriaxone, PCM, IV fluids	Seizures controlled; mild respiratory distress
9–11 July 2025	Radiological evaluation	—	—	Awaiting CT scan
12 July 2025	CT Scan Result	Large subacute ischemic stroke (left frontal lobe); chronic infarcts (right frontal, parietal, temporal); cystic encephalomalacia, cortical atrophy	—	Bilateral cerebrovascular accident confirmed
13–15 July 2025	Systemic deterioration	Increasing respiratory distress, jaundice, hepatomegaly, intermittent seizures	O₂ therapy (4–5 L/min), PCM, diclofenac suppository, tepid sponging	Bilateral CVA with jaundice and hepatomegaly
16 July 2025	Neurosurgery Review	—	Recommended neurology follow-up and transfusion	Multidisciplinary care
19 July 2025	First Physiotherapy Review	Inability to move UL & LL; spasticity of left elbow; TA tight on right; poor grip on left	Soft tissue mobilization (STM), passive movements (PM), stretches, patella mobilization, caregiver education	Quadriparesis secondary to bilateral CVA in SCA
20 July 2025	Physiotherapy Ward Round	Unstable; therapeutic positioning and caregiver education only	—	Deferred active therapy
21 July 2025	Ophthalmology Review	Unco-operative for dilated fundoscopy	—	Nil contributory findings
22 July 2025	HOWR	One seizure (2–3 min) involving left UL; spontaneous abortion	Diazepam 5 mg IV	Recurrent focal seizure
23 July 2025	Physiotherapy Session	Impaired speech; trunk stiffness; hypertonia ULs > LLs	Scapular/shoulder mobilization, tonic inhibitory positioning, trunk mobilization, SPS to ULs	Slight tone modulation achieved
24 July 2025	HOWR	Three seizures (2 min each); persistent fever, weight loss	Diazepam 5 mg, PCM; continued antibiotics	Ongoing seizure disorder
25–28 July 2025	Supportive care	—	Continued anticonvulsants, IV fluids	Clinical stabilization
29 July 2025	HOWR	One seizure (3–5 min); stretching of limbs	Diazepam 10 mg IV	Seizure aborted
31 July 2025	Discharge request	Generalized hypertonia; no ankle clonus; bilateral synergy patterns; hepatomegaly 6 cm below RCM	Referred for outpatient physiotherapy; continued Keppra, carbamazepine, folic acid, multivitamins	Persistent quadriparesis; follow-up on 12 Aug 2025

Laboratory Investigations

Baseline hematologic tests revealed hemoglobin concentration of 7.2 g/dL, packed cell volume (PCV) of 21%, and white blood cell count of 13.6 × 10⁹/L. Hemoglobin electrophoresis confirmed HbSS genotype, with HbS = 84.7%, HbF = 12.2%, and HbA₂ = 3.1%, consistent with sickle cell anemia. Peripheral blood film demonstrated normochromic normocytic red cells with sickled forms. Biochemical analyses showed mild hypokalemia (K⁺ = 2.9 mmol/L) on the third day of admission, which was corrected with intravenous potassium chloride infusion. Serum sodium (Na⁺ = 137 mmol/L), chloride (Cl⁻ = 101 mmol/L), urea (4.6 mmol/L), and creatinine (0.9 mg/dL) remained within normal limits. Liver enzymes were mildly elevated, with AST = 41 IU/L and ALT = 35 IU/L. Total bilirubin was 2.8 mg/dL, with a mild increase in the conjugated fraction, in keeping with hemolytic jaundice secondary to sickling. Malaria parasite test was negative, and blood culture yielded no growth.

Neuroimaging

A cranial computed tomography (CT) scan performed on 12 July 2025 demonstrated a large subacute ischemic infarct in the left frontal lobe, with loss of gray-white matter differentiation and sulcal effacement. There was also evidence of a chronic infarct involving the right frontal, parietal, and temporal lobes, associated with cystic encephalomalacia, dystrophic calcification, and cortical atrophy. These findings were consistent with a bilateral cerebrovascular accident (CVA) - a rare manifestation in pediatric sickle cell anemia. No intracerebral hemorrhage or space-occupying lesion was identified.

Differential Diagnosis

The primary differential diagnoses considered at presentation included: 1) Recurrent ischemic stroke secondary to sickle cell vasculopathy, supported by the patient’s known HbSS genotype and prior history of CVA. 2) Transfusion-related anaphylactic reaction, given the temporal relationship between exchange transfusion and seizure onset. 3) Metabolic seizure due to electrolyte imbalance, particularly hypokalemia, though this was corrected without full neurological recovery. Infectious causes such as meningitis and cerebral malaria were ruled out as blood culture and malaria parasite tests were negative, and there were no meningeal signs or cerebrospinal fluid abnormalities. Metabolic stroke was also considered but deemed unlikely following correction of electrolyte imbalance without neurological recovery. Hence, the overall clinical, laboratory, and imaging findings supported a diagnosis of bilateral ischemic cerebrovascular accident secondary to sickle cell anemia.

Therapeutic Intervention

The patient underwent comprehensive acute medical and multidisciplinary management following the onset of generalized seizures during exchange transfusion on 2 July 2025. Emergency resuscitation with intravenous adrenaline, hydrocortisone, diazepam, and phenobarbital successfully controlled the status epilepticus after multiple episodes. Maintenance anticonvulsants (carbamazepine and levetiracetam) were instituted alongside oxygen therapy, intravenous ceftriaxone, and antipyretics. Hypokalemia (2.9 mmol/L) was promptly corrected with intravenous potassium chloride, and nutritional supplementation with folic acid, omega-3 fatty acids, and multivitamins was provided. Despite stabilization, the patient developed recurrent low-grade fever, hepatomegaly, and jaundice, prompting continued input from pediatric neurology, neurosurgery, and intensive care teams to guide neuroprotective management.

Physiotherapy was initiated on 19 July 2025, two weeks after admission, focusing on tone reduction, maintenance of joint mobility, and caregiver education for home-based care. Interventions included positioning, passive and assisted range-of-motion exercises, soft tissue mobilization, tonic inhibitory techniques, and trunk mobilization for postural control. Six physiotherapy sessions were completed between 19 and 29 July, limited by intermittent medical instability and recurrent seizures. By discharge on 31 July 2025, the patient demonstrated mild improvement in upper limb tone, resolution of ankle clonus, and preserved joint mobility but remained quadriparetic. A structured home exercise program and outpatient follow-up were prescribed to continue neurorehabilitation and support functional recovery see Table 2.

Table 2
Summary of Therapeutic Interventions and Clinical Response
Phase	Date	Interventions	Clinical Response / Outcome
Acute Phase	2 July 2025	Exchange transfusion → seizures, loss of consciousness	Resuscitated with IV adrenaline, hydrocortisone, diazepam; transferred to PICU
	3–6 July 2025	IV phenobarbital, oral carbamazepine, potassium correction	Seizure frequency reduced; vital signs stabilized
	7–12 July 2025	IV ceftriaxone, PCM, O₂ therapy, hydration, nutritional support	Fever resolved; hepatomegaly noted; CT pending
	12 July 2025	CT confirmed bilateral ischemic infarcts	Final diagnosis established; physiotherapy consult requested
Rehabilitation Phase	19 July 2025	Initial physiotherapy evaluation and caregiver training	Hypertonia and tight tendo-Achilles identified; poor grip; dependent for ADLs
	20 July 2025	Positioning, STM, passive ROM, stretches	Tone slightly reduced; tolerated partially
	23 July 2025	Scapular/shoulder mobilization, trunk mobility, tonic inhibitory positioning	Improved relaxation; decreased upper-limb tone
	24 July 2025	Patella mobilization, ROM to lower limbs, continued STM	Mild improvement in joint mobility
	26 July 2025	Reinforcement of caregiver home routine	Caregiver demonstrated correct positioning and ROM
	29 July 2025	Review session; medical instability recurred	Minimal functional progress; spasticity persisted
Discharge Phase	31 July 2025	Home exercise program and outpatient referral	Conscious, quadriparetic; referred for continued neurorehabilitation

Baseline vs. Discharge Functional Status

This case underscores the intricate challenges of managing bilateral cerebrovascular accident in pediatric sickle cell anemia, a rare but devastating complication. The integration of timely medical stabilization with early physiotherapy intervention yielded measurable improvement in tone, range of motion, and seizure control within a constrained clinical window. Though gross motor recovery remained limited due to recurrent seizures and systemic instability, the physiotherapy program achieved crucial secondary prevention goals including contracture avoidance, maintenance of joint mobility, and caregiver empowerment. These outcomes reflect the potential of multidisciplinary, context-sensitive rehabilitation in improving survival quality for children with complex sickle cell neurologic complications see Table 3.

Table 3
Baseline vs Discharge Physiotherapy Assessment
Parameter	Baseline (19 July 2025)	At Discharge (31 July 2025)
Level of consciousness	Drowsy; intermittent response (GCS 12/15)	Fully conscious; alert and interactive
Tone	Global hypertonia (ULs > LLs)	Mild reduction in UL tone; generalized spasticity persists
Ankle clonus	Present on left	Absent bilaterally
ROM (Elbow/Wrist)	Limited and painful	Mildly improved; less discomfort
Grip strength	Poor on left, fair on right	Fair bilaterally
Functional ability	Unable to roll, sit, or perform ADLs	Partial head/trunk control; dependent for mobility
Seizure frequency	Recurrent (≥ 10 episodes/day)	Nil in last 72 hours before discharge
Hepatomegaly	4 cm below RCM	6 cm below RCM, non-tender
Overall impression	Unstable, quadriparetic	Stable, quadriparetic, improved tone and responsiveness

Bilateral cerebrovascular accident in a child with sickle cell anemia represents one of the most severe yet under-documented neurological complications of the disease. Beyond its rarity, such a presentation exposes the fragile balance between hematologic stability, neurovascular integrity, and rehabilitative opportunity in pediatric SCD. The present case highlights how complex systemic and iatrogenic factors can converge to produce a catastrophic neurological outcome, while also demonstrating the rehabilitative potential of early physiotherapy even within the constraints of a low-resource setting. The occurrence of bilateral infarcts, however, suggests a more diffuse mechanism involving microvascular occlusion, hypoxia, and impaired autoregulation rather than focal stenosis. Recent studies have described stroke in SCD without demonstrable cerebral arteriopathy, supporting a paradigm of multifactorial vascular dysfunction [9]. Wambaka et al. further observed that even with improved transcranial doppler velocities following hydroxyurea therapy, incident strokes persist, indicating that the risk extends beyond measurable flow abnormalities [2]. In this patient, the bilateral pattern likely reflected a cascade of overlapping insults: pre-existing sickle vasculopathy, severe anemia, and possible transfusion-induced hypoxic or anaphylactic stress. Such complexity underscores the unpredictability of cerebrovascular events in pediatric SCD and the necessity for vigilant monitoring during transfusion-based interventions.

The clinical course typified the challenges of stroke recognition and management in low-resource environments. Neuroimaging confirmation was delayed by logistical barriers, allowing the ischemic process to evolve unchecked. Meanwhile, recurrent seizures, fluctuating metabolic indices (notably hypokalemia), and persistent fever compounded the neurological injury. These difficulties mirror findings from the National Academies of Sciences, which highlighted delayed access to diagnostic and supportive technologies as major determinants of poor outcomes in African SCD populations [3]. Despite these barriers, coordinated input from hematology, neurology, neurosurgery, and physiotherapy helped stabilize the child and initiate rehabilitative care. This multidisciplinary model, though often difficult to maintain in overstretched systems, remains the cornerstone of comprehensive stroke management.

Rehabilitation forms the bridge between survival and functional reintegration following pediatric stroke. In SCD, this bridge is often neglected, resulting in long-term disability even among survivors. Systematic reviews by Marchese et al. and Rock et al. confirm that structured physiotherapy programs improve motor performance, muscle properties, and quality of life in children with SCD [11, 12]. Likewise, Reader et al. reported that consistent outpatient physical-therapy engagement predicts better independence in adolescence and early adulthood [15].

In the current case, early physiotherapy initiated two weeks post-admission focused on tone reduction, joint preservation, and caregiver empowerment. Although gross motor function remained limited due to ongoing seizures, meaningful gains were recorded in joint mobility and tone modulation. More importantly, caregiver training ensured therapy continuity after discharge, an essential adaptation in settings where inpatient rehabilitation is brief. These outcomes reaffirm the principle that rehabilitation, even when constrained, is therapeutic, not merely supportive.

The case illuminates broader systemic issues in sub-Saharan Africa: delayed presentation, inconsistent follow-up, and insufficient integration of physiotherapy into hematology care. Structured transcranial Doppler screening, early neurorehabilitation referral, and family-centered education could reduce recurrent strokes and improve quality of life [17]. In line with the National Academies’ blueprint for sickle cell care, rehabilitation should be embedded in every stage of disease management, from acute stabilization to community reintegration [3].

Limitations and Reflections

This single case cannot establish causality but provides a vivid educational model of the clinical and functional continuum in pediatric SCD stroke. The absence of follow-up imaging and standardized motor assessments reflects local systemic constraints rather than research oversight. Nevertheless, the detailed physiotherapeutic documentation contributes rare practical evidence to the literature on early rehabilitation following bilateral CVA in SCD.

This case underscores that recovery in pediatric sickle cell stroke extends beyond medical stabilization to encompass functional reclamation through early, context-sensitive rehabilitation. Even minimal physiotherapy input, when combined with caregiver education, can preserve musculoskeletal integrity and foster meaningful progress. In resource-limited systems, integrating physiotherapy into the acute and chronic management of sickle cell disease is not optional; it is essential to improving long-term survival quality and reducing the global burden of post-stroke disability.

Patient’s Caregiver Perspective

The child’s caregiver expressed gratitude for the care received and particularly appreciated the inclusion of physiotherapy, which helped her understand how to assist her nephew at home. She reported that the exercises and positioning techniques taught during hospitalization made daily care easier and reduced her anxiety about handling the child’s limbs. Although she acknowledged that recovery had been slow, she believed that the home-based program offered hope and a clear path toward gradual improvement. She also highlighted the need for ongoing community support for families managing children with chronic complications of sickle cell disease.

ADLs	Activities of Daily Living
ALT	Alanine Aminotransferase
AST	Aspartate Aminotransferase
CT	Computed Tomography
CVA	Cerebrovascular Accident
EBT	Exchange Blood Transfusion
GCS	Glasgow Coma Scale
Hb	Hemoglobin
HbA₂	Hemoglobin A₂
HbF	Fetal Hemoglobin
HbS	Sickle Hemoglobin
HbSS	Homozygous Sickle Cell Genotype
IV	Intravenous
K⁺	Potassium Ion
LL	Lower Limb
Na⁺	Sodium Ion
PCM	Paracetamol
PCV	Packed Cell Volume
PICU	Pediatric Intensive Care Unit
ROM	Range of Motion
SCA	Sickle Cell Anemia
STM	Soft Tissue Mobilization
TA	Tendo-Achilles
UL	Upper Limb
UNTH	University of Nigeria Teaching Hospital

Ethics approval and consent to participate

Ethical approval for this case report was obtained from the Health Research Ethics Committee of the University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu State, Nigeria (Approval No.: UNTHREC/2025/10/608). All procedures were conducted in accordance with the institutional ethics framework and the 2013 revision of the Declaration of Helsinki.

Informed consent

Written informed consent for participation and publication was obtained from the patient’s father and aunt. In line with ethical publication practices, the aunt specifically withheld consent for the use or publication of the child’s photographs or any identifiable visual materials; hence, no images are included in this report. A copy of the written consent is available for review by the Editor-in-Chief of Journal of Medical Case Reports.

Availability of data and materials

All relevant data generated or analyzed during this case are included in this published article. Additional anonymized information can be made available from the corresponding author upon reasonable request.

Conflicting interests

The author declares no complicting interests related to this publication.

Funding

This case report received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. All clinical management and documentation were institutionally supported.

Authors’ contributions

Adaeze Imelda Onyekwelu conceptualized the report, performed the physiotherapy management, compiled and interpreted the data, and drafted the manuscript.
Acknowledgements

The author acknowledges the contributions of the medical and physiotherapy teams of the Departments of Hematology, Oncology, and Pediatric Physiotherapy at the University of Nigeria Teaching Hospital, Enugu, for their multidisciplinary input in the patient’s care. Special appreciation goes to the child’s caregiver for her cooperation, dedication, and willingness to engage in the home rehabilitation program.

Authors’ information

Adaeze Imelda Onyekwelu, BMR (PT), is a physiotherapist and lecturer at the Department of Physiotherapy, David Umahi University of Health Sciences, Uburu, Ebonyi State, Nigeria, with clinical interests in pediatric and neurorehabilitation physiotherapy.

Egesa WI, Nakalema G, Waibi WM, Turyasiima M, Amuje E, Kiconco G et al (2022) Sickle Cell Disease in Children and Adolescents: A Review of the Historical, Clinical, and Public Health Perspective of Sub-Saharan Africa and Beyond. Int J Pediatr 2022:3885979. 10.1155/2022/3885979
Wambaka B, Mpungu A, Mboizi V, Kalibbala D, Nambatya G, Murungi S et al (2025) Incident Stroke in Pediatric Sickle Cell Anemia Despite Overall Improved Transcranial Doppler Velocity in a Ugandan Hydroxyurea Trial: Antecedent and Ongoing Risks. medRxiv. ;2025.01.28.25320389. 10.1101/2025.01.28.25320389
National Academies of Sciences, Engineering, and, Health M, and Medicine Division; Board on Population Health and Public Health Practice; Committee on Addressing Sickle Cell Disease (2020) : A Strategic PlanBlueprint for Action; Martinez RM, Osei-Anto HA, McCormick M, editors. Addressing Sickle Cell Disease: A Strategic PlanBlueprint for Action. Washington (DC): National Academies Press (US); Available from: https://www.ncbi.nlm.nih.gov/books/NBK566466/
Tebbi CK (2022) Sickle Cell Disease: A Review. Hemato 3(2):341–366. 10.3390/hemato3020024
Light J, Boucher M, Baskin-Miller J, Winstead M (2023) Managing the Cerebrovascular Complications of Sickle Cell Disease: Current Perspectives. J Blood Med 14:279–293. 10.2147/JBM.S383472
Adams RJ, McKie VC, Brambilla D, Carl E, Gallagher D, Nichols FT et al (1998) Stroke Prevention Trial in Sickle Cell Anemia. Controlled Clin Trials 19(1):110–129. 10.1016/S0197-2456(97)00099-8
Bernaudin F, Verlhac S (2022) Detection and Management of Cerebral Vasculopathy. In: IntechOpen; 10.5772/intechopen.105099
Zedde M, Quaresima M, Capodanno I, Grisendi I, Assenza F, Napoli M et al (2024) Neurovascular Manifestations of Sickle Cell Disease. Hemato 5(3):277–320. 10.3390/hemato5030023
Linguet SL, Verlhac S, Missud F, Holvoet-Vermaut L, Brousse V, Ithier G et al (2024) Stroke without Cerebral Arteriopathy in Sickle Cell Disease Children: Causes and Treatment. Haematologica 109(10):3346–3356. 10.3324/haematol.2023.283773
Nichols FTIII (2025) Stroke Associated with Sickle Cell Disease. In: Lewis SL (ed) MedLink Neurology. MedLink, LLC, San Diego. [updated 2025; cited 2025 Oct 10]. Available from: www.medlink.com
Marchese V, Rock K, Harpold A, Salazar A, Williams M, Shipper AG (2022) Physical Impairment and Function in Children and Adolescents with Sickle Cell Disease: A Systematic Review. Arch Phys Med Rehabil 103(6):1144–1167e2. 10.1016/j.apmr.2021.08.022
Rock K, Ho S, Gray VL, Addison O, York T, Wells DK et al (2023) Muscle Properties, Gross Motor Performance, and Quality of Life in Children with Sickle Cell Disease. Pediatr Phys Ther 35(4):450–456. 10.1097/PEP.0000000000001037
Callaway D, Chawla A, Sprinz P (2020) Physical Therapy in Pediatric and Young Adult Patients with Sickle Cell Disease: Assessing Potential Benefits and Barriers. J Pediatr Hematol Oncol 42(8):463–466. 10.1097/MPH.0000000000001888
Pinto DMR, do Sacramento MS, Santos PHS, Silva WS, de Oliveira EC, Gardenghi G et al (2021) Physical Exercise in Sickle Cell Anemia: A Systematic Review. Hematol Transfus Cell Ther 43(3):324–331. 10.1016/j.htct.2020.06.018
Reader B, Benedict J, Rospert A, Lemanek K, Creary S (2025) Describing Outpatient Physical Therapy Use Among Adolescents and Young Adults with Sickle Cell Disease: A Single-Center Retrospective Study. Pediatr Blood Cancer 72(8):e31809. 10.1002/pbc.31809
Millis RM, Baker FW, Ertugrul L, Douglas RM, Sexcius L (1994) Physical Performance Decrements in Children with Sickle Cell Anemia. J Natl Med Assoc 86(2):113–116
Noronha SA, Sadreameli SC, Strouse JJ (2016) Management of Sickle Cell Disease in Children. South Med J 109(9):495–502. 10.14423/SMJ.0000000000000523
Kossorotoff M, Brousse V, Grevent D, Naggara O, Brunelle F, Blauwblomme T et al (2015) Cerebral Haemorrhagic Risk in Children with Sickle-Cell Disease. Dev Med Child Neurol 57(9):830–837. 10.1111/dmcn.12571

The authors declare no competing interests.

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Bilateral Cerebrovascular Accident in Pediatric Sickle Cell Anemia: A Cascade of Complications – A Case Report

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Abstract

BACKGROUND

Bilateral Cerebrovascular Accident: An Unusual Catastrophe

Beyond Survival: The Role of Rehabilitation

Rationale for This Case Report

CASE PRESENTATION

Patient Information

Clinical Findings on Initial Presentation

Early Admission Phase (1–2 July 2025)

Intermediate Course (3–8 July 2025)

Neurological Imaging and Progression (9–12 July 2025)

Late Course (13–31 July 2025)

Laboratory Investigations

Neuroimaging

Differential Diagnosis

Therapeutic Intervention

Baseline vs. Discharge Functional Status

DISCUSSION

Limitations and Reflections

CONCLUSION

Patient’s Caregiver Perspective

Abbreviations

Declarations

References

Additional Declarations

Supplementary Files

Status:

Version 1