Respiratory failure after scoliosis correction surgery in patients with Prader-Willi syndrome: Two case reports

BACKGROUNDSleep-disordered breathing, including hypoventilation and obstructive sleep apnea, is often observed in Prader-Willi syndrome (PWS). Particularly in adolescence, scoliosis causes a progressive restrictive pulmonary pattern, leading to hypoventilation, so timely corrective surgery is requir...

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Bibliographic Details
Published in:World journal of clinical cases 2021, Vol.9 (32), p.9960-9969
Main Authors: Yoon, Ju-Yul, Park, Sung-Hee, Won, Yu Hui
Format: Report
Language:English
Online Access:Get full text
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Summary:BACKGROUNDSleep-disordered breathing, including hypoventilation and obstructive sleep apnea, is often observed in Prader-Willi syndrome (PWS). Particularly in adolescence, scoliosis causes a progressive restrictive pulmonary pattern, leading to hypoventilation, so timely corrective surgery is required. However, the effect is controversial. In addition, since mental retardation of PWS, patient effort-based respiratory tests may be less reliable. So far, no studies have accurately reported on the comparison of respiratory function before and after corrective surgery, and appropriate respiratory function measurement method in PWS. CASE SUMMARYWe present two cases of adolescent PWS with typical characteristics, including obesity, mental retardation, and scoliosis. Two boys, aged 12 and 13, diagnosed with PWS, both underwent scoliosis correction surgery. Before and immediately after surgery, arterial blood tests showed no abnormalities and no respiratory symptoms occurred. However, after 6-7 mo, both patients complained of daytime sleepiness, difficulty sleeping at night, dyspnea on exertion, and showed cyanosis. Hypercapnia and hypoxia were confirmed by polysomnography and transcutaneous CO2 monitoring during sleep and were diagnosed with obstructive sleep apnea and alveolar hypoventilation. It was corrected by nighttime noninvasive ventilation application and normal findings of arterial blood gas were maintained after 6-8 mo follow-up. CONCLUSIONEven after scoliosis surgery, "periodic" monitoring of respiratory failure with an "objective" test method is needed for timely respiratory support.
ISSN:2307-8960
2307-8960
DOI:10.12998/wjcc.v9.i32.9960