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CASE REPORT
Year : 2019  |  Volume : 20  |  Issue : 2  |  Page : 103-105

Anesthetic management of a teen with MURCS variant with tetralogy of Fallot for vaginoplasty


Department of Anaesthesia, Jehangir Hospital, Pune, Maharashtra, India

Correspondence Address:
Dr. Avantika Sandeep Bhat
Kakade Nagar, A Building, S. No. 284, Tanaji Nagar, Chinchwad, Pune - 411 033, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/TheIAForum.TheIAForum_24_19

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MRKH (Mayer Rockytonsky Kuster Houster) Syndrome is a rare congenital disorder of reproductive system affecting females (Incidence is 1:4500 newborn girls). There are 2 types of this syndrome. Type 1 involves maldevelopment of only the reproductive system. Type 2 is also known as MURCS syndrome which involves reproductive, cardiovascular systems, renal agenesis, somatic, vertebral and hearing defects. The MURCS variant with congenital heart defects is very rare type. Morbidity and mortality of patients having corrected Tetralogy of Fallot (TOF) depends upon the severity of residual right ventricular outflow tract (RVOT) obstruction and the amount of pulmonary regurgitation. Successful anaesthetic management of such a case is similar to a tightrope walk. Acute kidney injury (AKI) to the single kidney and deranged coagulation profile were additional challenges that we addressed to during the conduct of this case.


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