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Trans-sellar–trans-sphenoidal encephalocele presenting as nasal obstruction in an infant: a case report and systematic review of existing reports

Authors:
Noufal Basheer at Malabar Institute of Medical Sciences Ltd.
Noufal Basheer
Aiswarya Raj at Amrita Vishwa Vidyapeetham
Aiswarya Raj
Jim Mathew
Jim Mathew
Jim Mathew
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Jacob Alapatt
Jacob Alapatt
Jacob Alapatt
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Abstract and Figures

Trans-sellar trans-sphenoidal encephalocele is a rare congenital anomaly, with only around 20 cases having been documented in literature around the world. Surgical repair of these defects in the pediatric population commonly uses either the transcranial or the transpalatal approach, with the choice of approach being individualized based on the clinical features, age, and associated defects present in the patient. Here, we document a case of a 4-month-old child who presented to us with nasal obstruction, who was diagnosed with this rare entity and successfully underwent a transcranial repair for the same. We also provide a systematic review of all existing case reports that have described this rare condition in the pediatric population, as weel as the different surgical approaches used in each case.
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Child's Nervous System (2023) 39:2237–2243
https://doi.org/10.1007/s00381-023-05956-7
CASE REPORT
Trans‑sellar–trans‑sphenoidal encephalocele presenting asnasal
obstruction inaninfant: acase report andsystematic review
ofexisting reports
NoufalBasheer1· AiswaryaRaj2· JimMathew1· JacobAlapatt1
Received: 19 February 2023 / Accepted: 7 April 2023 / Published online: 13 April 2023
© The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2023
Abstract
Trans-sellar trans-sphenoidal encephalocele is a rare congenital anomaly, with only around 20 cases having been documented
in literature around the world. Surgical repair of these defects in the pediatric population commonly uses either the transcra-
nial or the transpalatal approach, with the choice of approach being individualized based on the clinical features, age, and
associated defects present in the patient. Here, we document a case of a 4-month-old child who presented to us with nasal
obstruction, who was diagnosed with this rare entity and successfully underwent a transcranial repair for the same. We also
provide a systematic review of all existing case reports that have described this rare condition in the pediatric population, as
weel as the different surgical approaches used in each case.
Keywords Encephalocele· Trans-sellar· Trans-sphenoidal encephalocele· Nasal obstruction· Transcranial repair
Introduction
The incidence of encephalocele ranges from 1 in 3000 to
1 in 5000 thousand live births [1]. The incidence of basal
cephaloceles is thought to be 1 in 35,000 live births, making
them rare anomalies. Based on the location of the defect in
the bone, they have been further sub-classified as transethmoi-
dal, spheno-orbital, sphenomaxillary, and trans-sphenoidal
encephaloceles. Trans-sphenoidal encephaloceles are a rare
entity, accounting for 5% of all basal encephaloceles (1 in
700,000 live births), with only a handful of cases documented
around the world [2]. Trans-sellar trans-sphenoidal encepha-
locele presenting in the pediatric population is an extremely
uncommon presentation, being the least common subtype of
trans-sphenoidal encephaloceles, with fewer than 20 cases
being reported worldwide [3, 4]. Modern neurosurgery has
advanced to the point where surgical correction of a once
terrifying scenario is now conceivable, despite the fact that
it was once thought to be a major challenge with extremely
high fatality rates. We hereby present a case of a 4-month-old
child with this rare clinical presentation, who was successfully
operated on using the transcranial approach, along with a sys-
tematic review of existing case reports on this phenomenon.
Case report
We present a case of a 4-month-old child, born at term as
the first child of a non-consanguineous marriage. There was
no history of maternal exposure to drugs or radiation. An
antenatal scan at 5months of intrauterine gestation revealed
polyhydramnios and a cleft palate. He was born via Cesarean
section at term with a birth weight of 3.35kg, with midline
Highlights
• Trans sellar, trans sphenoidal encephaloceles are an extremly
rare entity, with very fewcases having been previously
reported around the world.
• The incidence is estimated to be1 in 700,000 live births.
• Surgical approaches for the repair of this defect includes
transcranial and trans-palatal approaches and an endoscopic
repair via the nasal cavity.
• This case report details the use of a transcranial approach, as well as
offers a systematic review of all previously reported cases in literature.
* Aiswarya Raj
raj.aiswarya97@gmail.com
Noufal Basheer
basheer.noufal@gmail.com
Jim Mathew
djangojimmathew@gmail.com
Jacob Alapatt
jacobpalapatt@gmail.com
1 Department ofNeurosurgery, Aster MIMS, Kozhikode,
Kerala, India
2 Department ofNeurology, Aster MIMS, Kozhikode, Kerala,
India
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