Cloverleaf Skull Syndrome: A Rare Case Report

Abstract

Cloverleaf deformity is an extremely rare skull deformity that occurs when several joints (sutures) between a baby's skull bones begin to fuse too early. Also known as Kleeblattschädel syndrome, the cloverleaf deformity causes bulging at the front and sides of the skull, resembling a cloverleaf shape. Cloverleaf skull syndrome is an abnormal configuration of the calvaria classified as craniosynostosis, consisting of premature ossification of cranial sutures. It is a deformity characterized by a remarkable enlargement of the head, with a trilobed configuration of the frontal view, resembling a three leaved clover⁽¹⁾ The first report about such a syndrome in the literature occurred in 1973 and, over the years only some tens of cases have been documented worldwide⁽³⁾. The present report describes a case of a severe craniofacial condition that is known as "cloverleaf skull syndrome". At Physical examination multiple malformations were observed, with altered craniofacial configuration, low set ear, hypertelorism, exopthalmos, syndactyly, closed anterior fontanelle, high arched palate, bilateral CTEV. The vast majority of patients with kleeblattschädel have intellectual impairment and hydrocephalus. It is usually seen in thanatophoric dysplasia: classically type II only, severe Apert syndrome, severe Crouzon syndrome, Boston-type craniosynostosis & Carpenter syndrome.⁽⁵⁾Craniosynostosis has an  incidence of 1 in 2100 to 1 in 2500 live births with multi-suture craniosynostosis constituting about 5% of all the cases ^[9]. This condition is associated with a variety of presentations including optic atrophy, hydrocephalus, respiratory problems, cleft palate, and disorders of hearing and speech.^[10] These cases are very rare & so shall be reported promptly to deal with management part of this disease. An expert team of maxillofacial, plastic, and neurosurgeons is needed to handle such complex conditions which involve the correction of the bony deformity.