RESEARCH SUPPORT, NON-U.S. GOV'T
Add like
Add dislike
Add to saved papers

Mortality and life-threatening events after vertical expandable prosthetic titanium rib surgery in children with hypoplastic chest wall deformity.

BACKGROUND: Severe complex hypoplastic chest wall and spine deformity with thoracic insufficiency syndrome may be associated with premature death secondary to multisystem involvement. The vertical expandable prosthetic titanium rib (VEPTR) reconstructs the chest wall to address the thoracic insufficiency seen in this patient population. The purpose of this study is to report the mortality and life-threatening adverse events in a cohort of subjects with severe hypoplastic chest wall deformity, specifically with Jeune syndrome and Jarcho-Levin syndrome, after treatment with the VEPTR device.

METHODS: A cohort of 43 patients with either Jeune syndrome or Jarcho-Levin syndrome was extracted from a multicenter Investigational Device Exemption study of 214 patients who had surgery with the VEPTR device. Of the 43 patients, 19 had Jeune syndrome and 24 had Jarcho-Levin syndrome. Fatal and life-threatening adverse events were analyzed, including an assessment of time postsurgery, etiology, and the relationship of the adverse event to the surgical procedure.

RESULTS: Four (9%) of the 43 patients died during the follow-up period. All 4 (100%) of these patients had Jeune syndrome. After the initial surgery, 2 died within 6 months, and the remaining 2 within 1 year. Causes of death included respiratory complications in 2, renal failure in 1, and liver failure in 1. An additional 4 (9%) of the 43 patients had life-threatening adverse events. Two (50%) patients were diagnosed with Jeune syndrome and the remaining 2 (50%) with Jarcho-Levin syndrome. Two of the 4 life-threatening events were related to the surgery perioperatively: necrotizing enterocolitis and acute respiratory distress syndrome. The other two events included cardiac arrest secondary to central line placement and Demerol overdose.

CONCLUSIONS: Surgeons should be aware of the potential for fatality and life-threatening events that occur in patients with hypoplastic chest wall and spine deformity undergoing surgical treatment. The majority of these events occur within 1 year of surgery.

LEVEL OF EVIDENCE: Therapeutic study, Level IV (case series, no comparison group).

Full text links

For the best experience, use the Read mobile app

Group 7SearchHeart failure treatmentPapersTopicsCollectionsEffects of Sodium-Glucose Cotransporter 2 Inhibitors for the Treatment of Patients With Heart Failure Importance: Only 1 class of glucose-lowering agents-sodium-glucose cotransporter 2 (SGLT2) inhibitors-has been reported to decrease the risk of cardiovascular events primarily by reducingSeptember 1, 2017: JAMA CardiologyAssociations of albuminuria in patients with chronic heart failure: findings in the ALiskiren Observation of heart Failure Treatment study.CONCLUSIONS: Increased UACR is common in patients with heart failure, including non-diabetics. Urinary albumin creatininineJul, 2011: European Journal of Heart FailureRandomized Controlled TrialEffects of Liraglutide on Clinical Stability Among Patients With Advanced Heart Failure and Reduced Ejection Fraction: A Randomized Clinical Trial.Review

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app

Read by QxMD is copyright © 2021 QxMD Software Inc. All rights reserved. By using this service, you agree to our terms of use and privacy policy.

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app