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Transsphenoidal encephalocele, colpocephaly and corpus callosum agenesis in a midline cleft lip and palate patient: A very rare case

Authors:
Indranil Dutta at Medical College Kolkata
Indranil Dutta
GuruaribamNilamani Sharma
GuruaribamNilamani Sharma
GuruaribamNilamani Sharma
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KhungdombamPalin Singh
KhungdombamPalin Singh
KhungdombamPalin Singh
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Letters to Editor
Transsphenoidal
encephalocele,
colpocephaly and corpus
callosum agenesis in a
midline cleft lip and palate
patient: A very rare case
Sir,
A very few case reports were done in the literature about
transsphenoidal encephalocele (TSE) with colpocephaly
and corpus callosum agenesis in a midline cleft lip and
palate patient. No such case report was made from India.
Basal encephalocele (BE) occurs due to herniation of neural
elements including pituitary gland or optic apparatus,
through a defect in the skull base. TSE represents <5% of all
BEs with an estimated incidence of 1 in 700,000 live births.[1]
Clinical presentation is most often insidious except
in those cases where a large herniation protrudes
through the epipharynx. They are often associated with
hypertelorism, broad nasal root, cleft lip/palate, optic
nerve anomalies and agenesis of the corpus callosum.[2]
In this case, a 10‑year‑old boy presented with speech
problem and mild difficulty in breathing due to midline
cleft lip and palate defect – Tessier cleft 0. At birth, his
growth parameters were appropriate for age. On facial
examination, broad nasal alar base and midline cleft lip
repair scar mark were present. Systemic examination was
unremarkable. The cleft lip was operated at 6 months of
age, and no intraoral mass was seen that time.
This time intraoperatively, we found a pulsatile,
normal mucosal colour, compressible mass protruding
from nasal cavity and clearly visible through the cleft
palate [Figure 1].
To know the reason of that mass, magnetic resonance
imaging (MRI) of the brain was carried out. MRI report
showed features suggestive of corpus callosum agenesis,
TSE, colpocephaly, absence of septum pellucidum and
dilated and high‑riding third ventricle [Figure 2].
Neurosurgical opinion was sought and asked the patient
to review with endocrinologist in view of the high risk of
hypothalamopituitary dysfunction.
To correct the speech problem and to protect the TSE,
secondary incomplete midline cleft palate was repaired
by pushback technique left untouched TSE. This
encephalocele was planned for operation in his second
decade of life through transnasal approach.
Post‑operative follow‑up period patient was relieved
from breathlessness and improvement of speech as
compared to earlier. Patient and his guardians are happy
with the outcome.
In conclusion, the presence of midline craniofacial anomalies
in association with symptoms of nasal obstruction,
persistent rhinorrhoea, visual deficits and endocrine
dysfunction should alert the surgeons/physicians to the
possible presence of BE. Plastic surgeons may encounter
this surprise finding during cleft palate repair and should
be aware of the plan of action in case of such discovery.
Guardians of the patient should keep him away from
contact sports and be aware of avoid nasal suctioning.
Indications for surgical intervention include significant
airway obstruction, repeated meningitis, recurrent
rhinorrhoea, progressive loss of vision due to TSE or
BE. Endoscopic route is preferred because of the high
1911, 1963. p. 17, 304, 456, 519.
2. Parsons RW. Scar prognosis. Clin Plast Surg 1977;4:181-6.
3. Hoffman MD, Bielinski KB. Surgical pearl: The hybrid mattress
suture. J Am Acad Dermatol 1997;36:773-4.
4. Wu W, Chavez-Frazier A, Migden M, Nguyen T. The
buried half horizontal, half vertical mattress suture: A novel
technique for wound edges of unequal lengths. Dermatol Surg
2016;42:1391-3.
How to cite this article: Mehrotra BS, Sharma M. The buried
hybrid mattress suture: A novel technique. Indian J Plast Surg
2018;51:332-4.
© 2019 Indian Journal of Plastic Surgery | Published by Wolters Kluwer - Medknow
Access this article online
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Website:
www.ijps.org
DOI:
10.4103/ijps.IJPS_24_18
This is an open access journal, and articles are distributed under the terms of
the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License,
which allows others to remix, tweak, and build upon the work non-commercially,
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Indian Journal of Plastic Surgery Volume 51 Issue 3 September-December 2018 334
Published online: 2019-07-26
Letters to Editor
Figure 1: Intraoral examination reveals secondary cleft palate and the
pulsatile mass
Figure 2: Transsphenoidal herniation of meninges with cerebrospinal uid and
part of basifrontal cortex suggestive of transsphenoidal encephalocele
rate of post‑operative hypothalamopituitary dysfunction
following transcranial approach.[3] If neurosurgeons want
to intervene at the same time of cleft palate repair, through
wide gap of cleft palate approach would be a better option
or cleft palate repair may be postponed till TSE repair.
Treatment options[4] include
1. Early management of elevated intracranial pressure
2. To prevent sac rupture, urgent closure of skin defects
and/or removal of non‑functional extracerebral tissue
with watertight dural closure
3. Bone correction–frontal remodelling, nasal bone grafting
with attention to prevention of “long‑nose” deformity
4. Correction of telecanthus with the transnasal wiring.
Correction of dystopia and hypertelorism can be
performed in the same stage.
Declaration of patient consent
These authors certify that they have obtained all
appropriate patient consent forms. In the form, the legal
guardian has given his consent for images and other
clinical information to be reported in the journal. The
Guardian understands that names and initials will not be
published and due efforts will be made to conceal patient
identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conicts of interest
There are no conflicts of interest.
Indranil Dutta, Guruaribam Nilamani Sharma,
Khungdombam Palin Singh
Department of Plastic Surgery, Shija Hospitals and Research
Institute, Imphal West, Manipur, India
Address for correspondence:
Dr. Indranil Dutta,
Quarter No. 11, Doctor’s Quarter, Shija Hospitals and Research
Institute, Langol, Imphal West - 795 004, Manipur, India.
E-mail: drindranil035@gmail.com
REFERENCES
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Intrasphenoidal meningo-encephalocele: Report of two rare
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2. Chandran S, Raturi S, Pillay HM, James TE. Transsphenoidal
encephalocele masquerading as nasal mass in a 2-year-old boy.
BMJ Case Rep 2014;2014. pii: bcr2013201426.
3. Monteiro M, de Albuquerque AC, Nobre MC, Veloso AT,
Mendes VA, de Souza Filho LD, et al. Transspheinoidal
transpalatal meningoencephalocele. Arq Neuropsiquiatr
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4. Tsutsumi K, Asano T, Shigeno T, Matsui T, Ito S, Kaizu H,
et al. Transcranial approach for transsphenoidal encephalocele:
Report of two cases. Surg Neurol 1999;51:252-7.
How to cite this article: Dutta I, Sharma GN, Singh KP.
Transsphenoidal encephalocele, colpocephaly and corpus callosum
agenesis in a midline cleft lip and palate patient: A very rare case.
Indian J Plast Surg 2018;51:334-5.
© 2019 Indian Journal of Plastic Surgery | Published by Wolters Kluwer - Medknow
Access this article online
Quick Response Code:
Website:
www.ijps.org
DOI:
10.4103/ijps.IJPS_118_18
This is an open access journal, and articles are distributed under the terms of
the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License,
which allows others to remix, tweak, and build upon the work non-commercially,
as long as appropriate credit is given and the new creations are licensed under
the identical terms.
Indian Journal of Plastic Surgery Volume 51 Issue 3 September-December 2018335
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