PICU Clinical Pathway for Evaluation/Treatment of Children with Jeune Syndrome/Chest Wall Hypoplasia Undergoing VEPTR Implantation

Goals and Metrics
Patient Education
Provider Resources
Special Considerations
Child with Jeune Syndrome Admitted for Chest Wall Hypoplasia Undergoing VEPTR Implantation
Referring Patients, Physician Consultation & Care Prior to Transport
Hospital admission for Full Evaluation to Optimize Patient Status, Plan for Surgical Procedure & Post-operative Care
  • Consults on Admission
  • Preoperative Imaging
  • Labs
  • Refer to Jeune Syndrome Admission Order Set

Patient to OR for VEPTR Implantation

Intra-operative Management

Admit to PICU

Refer to IP Orthopaedic Jeune VEPTR Post Operative Management Order Set

Postoperative Day 0
  • Goals:
  • Maintain Analgesia
  • Maintain Invasive Ventilatory Support
Postoperative Day 1
  • Goals:
  • Maintain Analgesia – Begin PO Pain Medication if Possible
  • Mobilization
  • Maintain Wound Integrity
  • Assess Nutrition Readiness for Patients with Tracheostomy
  • Consider Readiness for Extubation
Postoperative Day 2
  • Goals:
  • Wean from PCA
  • Advance Nutrition
  • Maintain Mobility
  • Consider Readiness for Extubation
  • Begin Education for Discharge
Postoperative Day 3 and 4
  • Goals:
  • Advance Nutrition
  • Maintain Mobility
  • Enteral Pain Management
Postoperative Day 5
  • Goals:
  • First Dressing Change
  • Education for Managing Patient at Home
  • Baseline Respiratory Status
Discharge Education for Orthopaedic Care
  • Goals:
  • Baseline Pulmonary Status
  • Enteral Medication
  • Tolerating Baseline Enteral Feeds
  • Pass PT and OT Requirements
Posted: July 2019
Revised: June 2022
Authors: K. Devine, CRNP; L. McNeely, CRNP; J. Anari, MD; P. Cahill, MD; D. Davis, MD; O. Mayer, MD; I. Yuan, MD; A. Boyd, CRNP; D. Platenecky, DPT; J. Vicencio, DPT; L. Rhodes, RT; K. Semless, CRNP