Abstract

Background: Cerebral palsy is not only a serious neurodevelopmental disease causing significant morbidity in children, but also a traumatic experience leading to psychosocial trauma to the parents/caregivers of the affected children. It is usually caused by prenatal or early post-natal insults to the newborn brain which may be associated with some congenital syndromes like congenital heart disease with transposition of the viscera but rarely a heterotaxy syndrome, a condition characterized with congenitally abnormal arrangement of the thoracic and abdominal viscera. Method: We present a case report of a 12-month-old boy with neurodevelopmental delay, recurrent episodes of non-mucoid and non-bloody diarhoea, occasional constipation, bilious vomiting, abdominal distension and fever with associated cough and difficulty in breathing. Results: We discuss an unusual presentation of cerebral palsy and heterotaxy syndrome diagnosed clinically with supporting evidence from both laboratory and radiological tests. Cerebral palsy was diagnosed from the history of birth asphyxia, delayed developmental milestone, limb spasticity and low values for all sub-scores of Bayley-III scale. Heterotaxy syndrome was diagnosed from the radiologic evidence of dextrocardia, left-sided stomach, centrally located liver and malrotation of gut with volvulus. We also provide a brief literature review of the incidence and prevalence, causes and risk factors, classification, clinical presentation and associated co-morbidities of heterotaxy syndrome. Conclusion: Diagnosis of heterotaxy syndrome in a child with background cerebral palsy is a great challenge to both physicians and radiologists. This is more so in developing countries due to poor availability of good diagnostic apparatus, therefore, a high index of suspicion is needed. A clear understanding of the clinical features, comprehensive history taking and thorough physical examination are important in making prompt diagnosis. Timely and appropriate imaging is necessary to prevent delays in diagnosis and treatment which lead to poor outcomes.

Highlights

  • Cerebral palsy (CP) is a syndrome that is caused by congenital or acquired insults that occurs to the developing brain usually before birth or during the early stage of infancy resulting in motor impairment, abnormalities of communication, intellectual ability, and epilepsy[1]

  • CP was defined by The International Executive Committee for the Definition of Cerebral Palsy as a group of disorders of the development of movement and posture, causing activity limitations which are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain

  • The syndrome is a form of congenital heart disease (CHD), and the most serious of them, which occurs in approximately 0.8% of children with CHD and has very poor prognosis when associated with complex cardiac lesions [9,15,16]

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Summary

Introduction

Cerebral palsy (CP) is a syndrome that is caused by congenital or acquired insults that occurs to the developing brain usually before birth or during the early stage of infancy resulting in motor impairment, abnormalities of communication, intellectual ability, and epilepsy[1]. Cerebral palsy is a serious neurodevelopmental disease causing significant morbidity in children, and a traumatic experience leading to psychosocial trauma to the parents/caregivers of the affected children It is usually caused by prenatal or early post-natal insults to the newborn brain which may be associated with some congenital syndromes like congenital heart disease with transposition of the viscera but rarely a heterotaxy syndrome, a condition characterized with congenitally abnormal arrangement of the thoracic and abdominal viscera. Conclusion: Diagnosis of heterotaxy syndrome in a child with background cerebral palsy is a great challenge to both physicians and radiologists This is more so in developing countries due to poor availability of good diagnostic apparatus, a high index of suspicion is needed. And appropriate imaging is necessary to prevent delays in diagnosis and treatment which lead to poor outcomes

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