Yoon JY, Park SH, Won YH. Respiratory failure after scoliosis correction surgery in patients with Prader-Willi syndrome: Two case reports. World J Clin Cases 2021; 9(32): 9960-9969 [PMID: 34877337 DOI: 10.12998/wjcc.v9.i32.9960]
Corresponding Author of This Article
Yu Hui Won, MD, PhD, Professor, Department of Physical Medicine and Rehabilitation, Jeonbuk National University Medical School, 20 Geonjiro, Jeonju 54907, Jeonbuk, South Korea. wonyh@jbnu.ac.kr
Research Domain of This Article
Pediatrics
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Nov 16, 2021; 9(32): 9960-9969 Published online Nov 16, 2021. doi: 10.12998/wjcc.v9.i32.9960
Respiratory failure after scoliosis correction surgery in patients with Prader-Willi syndrome: Two case reports
Ju-Yul Yoon, Sung-Hee Park, Yu Hui Won
Ju-Yul Yoon, Sung-Hee Park, Yu Hui Won, Department of Physical Medicine and Rehabilitation, Jeonbuk National University Medical School, Jeonju 54907, Jeonbuk, South Korea
Sung-Hee Park, Yu Hui Won, Research Institute of Clinical Medicine of Jeonbuk National University - Biomedical Research Institute of Jeonbuk National University Hospital, Jeonju 54907, Jeonbuk, South Korea
Author contributions: Won YH and Park SH contributed to the conception and design of the study or acquisition of data, or analysis and interpretation of data; Won YH and Yoon JY contributed to the drafting of the article or revising it critically for important intellectual content; and all authors provided final approval of the version to be submitted.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Yu Hui Won, MD, PhD, Professor, Department of Physical Medicine and Rehabilitation, Jeonbuk National University Medical School, 20 Geonjiro, Jeonju 54907, Jeonbuk, South Korea. wonyh@jbnu.ac.kr
Received: June 14, 2021 Peer-review started: June 14, 2021 First decision: July 26, 2021 Revised: August 3, 2021 Accepted: September 15, 2021 Article in press: September 15, 2021 Published online: November 16, 2021 Processing time: 148 Days and 10.4 Hours
Abstract
BACKGROUND
Sleep-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 SUMMARY
We 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.
CONCLUSION
Even after scoliosis surgery, “periodic” monitoring of respiratory failure with an “objective” test method is needed for timely respiratory support.
Core Tip: We describe two cases of adolescent Prader-Willi syndrome (PWS) with typical characteristics, such as obesity, mental retardation, and scoliosis, in which respiratory failure with severe desaturation and hypercapnia occurred after scoliosis correction surgery. Respiratory failure was confirmed and diagnosed by polysomnography (PSG) and transcutaneous CO2 monitoring and successfully treated with overnight noninvasive ventilation. These cases highlight the need for objective respiratory tests and timely respiratory support through perioperative and periodic pulmonary check-ups in PWS patients with cognitive decline. Guidelines for full PSG studies, including CO2 monitoring, should be framed to detect hypoventilation within a certain time post-surgery for scoliosis.
