Indian Journal of Clinical Anaesthesia 2023;10(3):318–321
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Indian Journal of Clinical Anaesthesia
Journal homepage: www.ijca.in
Case Report
A case report on anaesthetic challenges in Patau syndrome: Navigating
craniofacial and cardiac defects
Abha Singh
1
, Pragya Shukla
1,
*, Amrita Rath
1
, Shashi Prakash
1
, Sanjay Bhaskar
1
1
Dept. of Anaesthesiology, Institute of Medical Sciences, BHU, Varanasi, Uttar Pradesh, India
ARTICLE INFO
Article history:
Received 30-01-2023
Accepted 04-07-2023
Available online 07-09-2023
Keywords:
Atrial septal defect
Craniofacial defect
Difficult airway
Esophageal dilator
Limited resources
Patau syndrome
ABSTRACT
The anaesthetic management of paediatric patients with Patau syndrome presents unique challenges,
particularly when combined with craniofacial and cardiac defects and limited airway resources. This case
report highlights our experience in managing a syndromic child with a difficult airway in a resource-
constrained environment. The inability to secure the airway adequately can pose significant perioperative
risks. In this case, we successfully utilized an oesophageal bougie as an alternative technique to secure the
airway. This report emphasizes the importance of adapting to limited resources and employing innovative
approaches to ensure optimal patient care in challenging situations.
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1. Introduction
Patau syndrome is the third most common autosomal
trisomy disorder, after Trisomy 21 and Trisomy 18, with
a prevalence rate of 8 to 15 per 100,000 live births.
1
Patau syndrome phenotype is characterized by multiple
craniofacial and congenital organ malformations leading
to difficult ventilation and intubation.
2
Cleft palate is
reported in 75% of cases and is associated with increased
risk of pulmonary aspiration and infection.
2
This risk
further increases with age due to development of thoracic
kyphoscoliosis resulting in reduced lung volumes and
ineffective cough.
3,4
As the paediatric patients have poor
functional residual capacity, unsuccessful intubation can
lead to hypoxemia, bradycardia and even cardiac arrest.
5
The airway devices for difficult airway in paediatric patients
are often restricted due to unavailability and smaller sizes,
hence a well formulated strategies are often required.
5,6
* Corresponding author.
E-mail address: drabhasingh1990@yahoo.in (P. Shukla).
2. Case Report
A 2 years 6 months old male child weighing 8.5 kg, a
known case of Patau syndrome (Trisomy 13) was planned
to undergo laparoscopic orchiopexy surgery for bilateral
non-palpable undescended testis. The physical examination
revealed craniofacial anomalies like short-webbed neck,
micrognathia, bulbous nose, prominent occiput, sharp
canines, incisors tooth decay and limb defects like swollen
palms and soles with clenched fingers and toes (Figures 1
and 2). The birth history suggested preterm assisted vaginal
delivery at 36 weeks of gestational age, with 1.2 kg
birth weight. Although baby cried immediately after birth
but, was dyspnoeic and shifted to ventilator in Neonatal
Intensive Care Unit for 2 weeks due to Respiratory distress
syndrome (RDS). The boy had a history of weak cry
with poor rooting, suckling reflexes, feeding difficulty,
regurgitation of milk through the mouth and nose till
the age of 2 years, without any obvious oropharyngeal
deformity. The general examination revealed delayed
developmental milestones, poor nutritional status, small
scrotal sac, normal intelligence quotient (IQ) for age with
https://doi.org/10.18231/j.ijca.2023.065
2394-4781/© 2023 Author(s), Published by Innovative Publication. 318