Anaesthesia of an infant with Johanson-Blizzard Syndrome: A case report and review of literature

Dr. Smriti Sinha*, Dr. Joel Chakravarthy, Dr. Varghese K Zachariah

Anaesthesia of an infant with Johanson-Blizzard Syndrome: A case report and review of literature

Dr. Smriti Sinha*, Dr. Joel Chakravarthy, Dr. Varghese K Zachariah

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Dr. Smriti Sinha*, Dr. Joel Chakravarthy, Dr. Varghese K Zachariah, “Anaesthesia of an infant with Johanson-Blizzard Syndrome: A case report and review of literature”, ijmhs, vol. 10, no. 06, pp. 893–895, Jun. 2020.

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Abstract

We present an 8 months old infant with Johanson-Blizzaed syndrome (Autosomal
recessive disease) for postero sacral anorectal formation for imperforate anus who required
general anaesthesia. An anaesthetic plan for each individual should be personalized
as the phenotypic presentation varies over a spectrum of severe form to hypomorphic
form. The anaesthetic considerations included pancreatic exocrine insufficiency,
mental retardation, hearing impairment, growth delay, hypotonia, lax joints and difficult
airway due to short nose, absent ala nasi, receding chin, maxillary hypoplasia
and high arched palate. Pre-operative sedation was avoided because of hypotonia of
the pharyngeal muscles and difficult airway. Pancreatic enzymes replacement continued
with intraoperative sugar monitoring. To the best of our knowledge there have
been less than hundred cases reported of JB Syndrome, four cases have been reported
from India. There are very limited number of reported case describing anaesthetic
considerations of JB Syndrome.
Key words: Johanson-Blizzard syndrome–Anaesthetic management–Pancreatic exocrine
dysfunction

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Issue Vol. 10 No. 06 (2020): Innovative Journal of Medical and Health Science
Section Research Articles

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Anaesthesia of an infant with Johanson-Blizzard Syndrome: A case report and review of literature

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