CASE 17455 Published on 22.10.2021

A rare cause of craniofacial asymmetry in a child

Section

Paediatric radiology

Case Type

Clinical Cases

Authors

Sandeep Singh Awal1,Sajal Agarwal2, Ramesh Giti3

1. Department of Radiology, Jeevandeep Diagnostics, Jamshedpur, India

2. Department of Radiology, G.G. Hospital, M.P. Shah Medical College, Jamnagar, India

3. Department of Radiology, Mashhad University of Medical Sciences, Mashhad, Iran

Patient

4 years, male

Categories
Area of Interest Bones, CNS ; Imaging Technique CT
Clinical History

A 4-year-old male patient with history of abnormal morphology of head and behavioural abnormalities. Birth history was unremarkable. No history of maternal or fetal distress during pregnancy.

On clinical examination, the patient showed signs of neurodevelopmental delay and asymmetrical head shape.

Imaging Findings

Performed frontal and lateral skull radiographs revealed marked prominence of convolutional markings throughout with ‘copper-beaten’ appearance [1].

Non-contrast computed tomography (NECT) performed with VRT (Volume Rendering Technique) revealed premature fusion of the right coronal suture. Flattening of the right frontal bone was seen with distorted skull. Left coronal suture, sagittal suture, and bilateral lambdoid sutures appear unfused.

Based on imaging findings, the diagnosis of Unilateral coronal craniosynostosis - anterior plagiocephaly was made.

Discussion

Background

Craniosynostosis is defined as premature fusion of cranial sutures, either individually or in combination [2]. During infancy and early childhood, normal sutures allow growth of brain and skull. The sutures close in chronological order as the cerebral growth slows down. Metopic suture is the first suture to close, followed by coronal, lambdoid and sagittal suture. Premature closure of any suture/sutures hampers optimal skull growth, leading to abnormal head/skull shape [3,4].

Craniosynostosis can be primary or secondary [1]. Causes of secondary craniosynostosis include haematological disorders like thalassemia and sickle cell disease, metabolic disorders like rickets and certain bone dysplasias [5]. Many syndromic associations have been reported including Crouzon syndrome, Apert syndrome, and Carpenter syndrome [6,7].

Imaging Perspective

Skull radiography may predict craniosynostosis by abnormal head shape [4]. However, CT with multiplanar reconstruction and 3D techniques like VRT is the ideal imaging modality to diagnose and delineate the extent of premature sutural fusion [7]. Coexisting abnormalities including hydrocephalus, gray matter abnormalities and corpus callosum defects may be encountered. MRI is useful in detecting coexisting abnormalities [4,7].

The imaging features of craniosynostosis depends on the type and extent of premature sutural fusion [4,7]:

  • Dolichocephaly (also known as scaphocephaly), is characterized by the premature fusion of sagittal suture of skull. The skull grows in the anteroposterior direction while the transverse growth is impeded. As a result, the skull is elongated in the anteroposterior direction, while the transverse diameter of skull is decreased.
  • Brachycephaly is a type of craniosynostosis in which there is premature fusion of the bi-coronal and/or bi-lambdoid sutures. This leads to widening of skull in the transverse direction.
  • Plagiocephaly is characterised by unilateral premature fusion of coronal and/or lambdoid suture.
  • Trigonocephaly is the premature closure of metopic suture resulting in a triangular skull pointing anteriorly.
  • Oxycephaly is the most severe form of craniosynostosis characterized by premature fusion of coronal, sagittal, lambdoid sutures resulting in conical appearance of the skull.
  • Cloverleaf skull (Kleeblattschädel) is a rare, severe form of craniosynostosis, caused by intrauterine premature closure of sagittal,
    coronal, lambdoid sutures resulting in cloverleaf morphology of skull.

Prognosis, treatment, or therapeutic options:

Prognosis depends on the type of craniosynostosis and the extent of sutural fusion. Severe forms can be treated surgically by cranioplasty [7].

Written informed patient consent for publication has been obtained.

Differential Diagnosis List
Unilateral coronal craniosynostosis - Anterior plagiocephaly
Positional plagiocephaly
Posterior plagiocephaly
Dolichocephaly
Brachycephaly
Trigonocephaly
Final Diagnosis
Unilateral coronal craniosynostosis - Anterior plagiocephaly
Case information
URL: https://www.eurorad.org/case/17455
DOI: 10.35100/eurorad/case.17455
ISSN: 1563-4086
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