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CC BY 4.0 · Indian Journal of Neurosurgery
DOI: 10.1055/s-0046-1815923
Clinical Images

Bullhorn Appearance of Skull in a Follow-Up Case of Craniosynostosis

Authors

  • Abhijit Acharya 

    1   Department of Neurosurgery, Institute of Medical Sciences & SUM Hospital, Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India

  • Ashok Kumar Mahapatra

    1   Department of Neurosurgery, Institute of Medical Sciences & SUM Hospital, Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India

  • Souvagya Panigrahi

    1   Department of Neurosurgery, Institute of Medical Sciences & SUM Hospital, Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India

  • Rama Chandra Deo 

    1   Department of Neurosurgery, Institute of Medical Sciences & SUM Hospital, Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India

  • Amresh Chandra Bal

    2   Department of Anaesthesia, Institute of Medical Sciences & SUM Hospital, Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India
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Abstract

Craniosynostosis is a craniofacial developmental anomaly resulting from the premature fusion of single or multiple skull sutures. It can have a syndromic (approximately 25%) or non-syndromic association (approximately 75%). Premature fusion of sutures can lead to developmental delays and cosmetic abnormalities of the calvarium. Their management involves surgical removal of the fused suture lines. Surgical correction, however, can lead to several complications such as hematomas, dural tear, and cerebrospinal fluid (CSF) leaks, etc. We present an 8-month-old child who presented with clinical features of non-syndromic craniosynostosis. He underwent “Pi” stripping 3 months ago. On follow-up, he developed two symmetrical bilateral globular swellings over the coronal sutures, 2 months after the first surgery. The swellings gave the appearance of a “bull horn.” Imaging with a CT brain was suggestive of hydrocephalous. A low-pressure ventriculoperitoneal shunt was placed. The postoperative period was uneventful, with resolution of the swellings and improvement in the craniofacial cosmetic appearance.


Keywords

craniofacial development - craniosynostosis - hydrocephalus - ventriculoperitoneal shunt

Introduction

Craniosynostosis is a congenital developmental abnormality of the skull resulting from premature fusion of the suture lines, which can occur as early as the intrauterine period. The “ideal age” for skull suture fusion varies as per suture, but general ranges have been described: the metopic suture fuses between 3 and 9 months, the anterior fontanelle closes by 18 to 24 months, the coronal and sagittal sutures fuse around 24 to 26 years, and the lambdoid suture closes around 26 to 27 years. Complete closure of some sutures may continue into adulthood and even old age, with the squamosal suture fusing around 60 years.[1−3] The presence of sutures helps in the expansion of the skull in parallel with brain growth. Premature fusion of one or multiple sutures can restrict the growth of the corresponding part of the brain, potentially leading to developmental delays. Craniosynostosis may involve a single fused suture or multiple sutures. Multiple fused sutures involvement is often associated with syndromes such as Aperts, Cruzons, Pfeiffers, etc. Syndromic craniosynostosis involves approximately 25% of cases, whereas non-syndromic craniosynostosis accounts for around 75%.[4]


Case Presentation

We present an 8-month-old child who demonstrated clinical features of non-syndromic craniosynostosis involving the bilateral coronal and sagittal sutures. He underwent “Pi” stripping 3 months ago. The surgery was done for early fusion of the coronal and sagittal sutures, associated with delayed development and early closure of anterior fontanelle. The stripping was done in the shape of a “pi” involving removal of the bilateral coronal sutures along with bilateral parasagittal strips of bone. The lambdoid suture was not removed during the first surgery. On follow-up, two symmetrical bilateral globular swelling developed over the coronal sutures, 2 months after the first surgery. The swellings gave the appearance of a “bull horn” ([Fig. 1A]). On Imaging with a CT scan of the brain, findings were suggestive of hydrocephalous with gross dilatation of the bilateral frontal horns ([Fig. 1B]). A low-pressure ventriculoperitoneal shunt was placed from the left posterior aspect at keen's point. The postoperative period of the patient was uneventful. A postoperative CT scan of the brain demonstrated the shunt in situ, with resolution of the swellings and hydrocephalous ([Fig. 2A]). There was also notable cosmetic improvement in the craniofacial features ([Fig. 2B]).

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Fig. 1 (A) Bullhorn appearance of the patient after cranial stripping. (B) CT showing hydrocephalous with frontal horn dilatation. CT, computed tomography.
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Fig. 2 (A) CT brain was suggestive of shunt in situ with a resolution of swelling and hydrocephalous. (B) Cosmetic improvement of the craniofacial features. CT, computed tomography.

Discussion

Hydrocephalus is a known complication that can occur after craniosynostosis surgery. The incidence of craniosynostosis is approximately 1 in 2,500 live births. The incidence of hydrocephalus following craniosynostosis surgery varies widely, ranging from 4 to 40%, depending on the series and the type of craniosynostosis.[3] The exact cause remains unknown; however, associations have been reported with genetic factors such as FGFR1, FGFR2, FGFR3 mutations, as well as mutations in SMAD6, TCF12, ERF, and MSX2 in non-syndromic cases.[5] The pathogenesis of hydrocephalus following craniosynostosis surgery is multifactorial and not yet fully understood. Possible mechanisms include impaired cerebrospinal fluid absorption due to blood or debris in the subarachnoid space; venous hypertension caused by jugular foramen stenosis or other venous outflow obstruction; and cranial vault remodeling that alters CSF dynamics and increases intracranial pressure. Risk factors for developing hydrocephalus after craniosynostosis surgery include: syndromic craniosynostosis (e.g., Apert or Crouzon), involvement of multiple sutures, previous cranial surgery, and intraoperative complications (e.g., excessive blood loss or venous injury). Close monitoring and follow-up are crucial for early detection and management of hydrocephalus in these patients. The long-term prognosis for patients with craniosynostosis who undergo shunt placement is generally good, however, it depends on several factors, including the type and severity of craniosynostosis, the presence of other health conditions, and the effectiveness of the shunt. Key factors influencing prognosis include shunt function and the need for revisions, as shunt malfunction or infection may occur and require additional surgery; cranial growth and development with ongoing monitoring of cranial growth and cognitive development being crucial; and associated conditions, as the presence of other health issues, such as syndromic craniosynostosis, can impact prognosis. Outcomes include high survival rates, with most patients reaching adulthood. Many patients achieve normal or near-normal cognitive development, although some may experience residual neurological issues, such as seizures or visual problems.[6] [7] Depending on the sutures involved craniosynostosis can be morphologically divided into anterior (coronal) and posterior (lambdoid) plagiocephaly, dolichocephaly (sagittal), and other forms. Infants are evaluated using CT brain imaging which is considered the gold standard. They can identify the fused suture (single or multiple), the type of craniosynostosis, and associated intracranial abnormalities, such as hydrocephalous. SPECT is necessary to evaluate the perfusion of the cerebral parenchyma. Management includes an endoscopic approach, typically performed between 3 and 6 months of age, and an open surgical approach, usually undertaken between 6 months and 1 year of age. These patients should undergo surgery before 1 year of age to allow ample time for brain growth and to reduce the risk of disability. Surgical complications occur in approximately 1 to 1.5% of cases. Common complications include hematoma formation, dural tears, and CSF leaks.[8] In our case, the patients developed hydrocephalous 2 months after cranial stripping, presenting with two symmetrical, bilateral, globular swellings over the coronal sutures. He was managed with a low-pressure shunt placed via a posterior approach despite higher dilation of frontal horns. This process was selected to reduce complications, as frontal shunt placement may result in shunt hardware exposure, making the posterior approach potentially more appropriate.


Conclusion

The key teaching point is the need for vigilance for hydrocephalus during follow-up in patients with craniosynostosis. For long-term care, a regular follow-up with a multidisciplinary team, including neurosurgery, craniofacial surgery, and developmental pediatrics, is essential to monitor shunt function, cranial growth, and overall development.



Conflict of Interest

None declared.

Acknowledgment

The authors acknowledge SOA University for their unconditional support.


Address for correspondence

Abhijit Acharya, MCh Neurosurgery
Department of Neurosurgery, Institute of Medical Sciences & SUM Hospital, Siksha ‘O’ Anusandhan University
BBSR, Odisha 751030
India   

Publication History

Article published online:
21 January 2026

© 2026. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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Zoom
Fig. 1 (A) Bullhorn appearance of the patient after cranial stripping. (B) CT showing hydrocephalous with frontal horn dilatation. CT, computed tomography.
Zoom
Fig. 2 (A) CT brain was suggestive of shunt in situ with a resolution of swelling and hydrocephalous. (B) Cosmetic improvement of the craniofacial features. CT, computed tomography.