Prader-Willi syndrome with Oculocutaneous Albinism: Anaesthetic Implications and Management
Prader-Willi syndrome is a rare congenital disorder characterized by obesity, hypotonia, hypogonadism and developmental delay. The genetic disorder is caused by microdeletion on chromosome 15 on the paternal chromosome. The syndrome is a 2-stage disorder with hypotonic early infantile phase and...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
JCDR Research and Publications Pvt. Ltd.
2014-04-01
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| Series: | National Journal of Laboratory Medicine |
| Subjects: | |
| Online Access: | http://www.njlm.net/articles/PDF/2007/4-%206780_F(H)_PF1(PAG)_PFA(AK)_PF2(AGAK).pdf |
| Summary: | Prader-Willi syndrome is a rare congenital disorder
characterized by obesity, hypotonia, hypogonadism and
developmental delay. The genetic disorder is caused
by microdeletion on chromosome 15 on the paternal
chromosome. The syndrome is a 2-stage disorder with
hypotonic early infantile phase and an obese childhood
phase. These individuals have abnormal physiologic
response to hypoxia and hypercapnia, narrow oropharyngeal
space, thick secretions and exaggerated response to
sedatives posing increased anaesthetic risk. We describe
the anaesthetic management of a patient with PraderWilli syndrome where in laparoscopic orchidopexy was
performed successfully under general anaesthesia. |
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| ISSN: | 2277-8551 2455-6882 |