Craniosynostosis v4.0: Diagnostic Phase Approval & Citation Summary of Version Changes Explanation of Evidence Ratings DIAGNOSTIC PHASE Guidelines for surgical procedure and timeframe Inclusion Criteria · Posterior vault distraction osteogenesis · Child with Craniosynostosis (PVDO), early infancy AND · Fronto-orbital advancement, 9-12 months of · Candidate for Cranial Vault age Expansion · Posterior vault switch cranioplasty, 6-9 months of age Exclusion Criteria · Fronto-orbital advancement 9-15 months of age · Children with diagnosis other than · Modified pi, 4-6 mos Craniosynostosis or without · Posterior cranial vault expansion Cranial Vault Expansion · Endoscopic strip craniectomy ≤ 4 months of age Initial Assessment · Ensure patient meets inclusion criteria AND surgery is recommended Off · Determine surgical procedure No · Submit surgical planning sheet Pathway · If PVDO, then also schedule hardware removal date 8-10 weeks post-op Yes Further assessments · If murmur, ECHO and consider ! cardiology consult Evaluate · If personal or family history of Murmur? bleeding disorder, obtain PT, PTT, Yes Atrial septal defects Bleeding disorder? INR, Von Willebrand antigen, stored should be treated prior MRSA? specimen and hematology to surgery given the high consultation risk for venous air embolism · If positive MRSA history, obtain with cranial vault expansio. *PFOs MRSA screening swab are common and do not require treatment No Ensure appropriate evaluations have been initiated · Complete Phase 1 of CIS ! PowerPlan and Checklist 1 Obtain a cervical CT in patients with syndromic synostosis given the higher risk of cervical anomalies To Pre-Surgical Phase For questions concerning this pathway, Last Updated: March 2019 contact: [email protected] Next Expected Review: March 2024 © 2019 Seattle Children’s Hospital, all rights reserved, Medical Disclaimer Craniosynostosis v4.0: Pre-Surgical Phase Approval & Citation Summary of Version Changes Explanation of Evidence Ratings PRE-SURGICAL PHASE Guidelines for surgical procedure and timeframe Inclusion Criteria · Posterior vault distraction osteogenesis (PVDO), early infancy · Child with Craniosynostosis AND · Fronto-orbital advancement, 9-12 months of age · Candidate for Cranial Vault · Posterior vault switch cranioplasty, 6-9 months of age Expansion · Fronto-orbital advancement 9-15 months of age Exclusion Criteria · Modified pi, 4-6 mos · Children with diagnosis other than Posterior cranial vault expansion · Craniosynostosis or without · Endoscopic strip craniectomy ≤ 4 months of age Cranial Vault Expansion Pre-surgical Assessment · Ensure patient meets inclusion Off criteria AND surgery is No recommended Pathway · Confirm planned surgical procedure Yes ! All patients must complete an evaluation in PASS clinic Patients with a bleeding disorder must be cleared by Hematology; Review PASS assessment; Review MRSA screening Ensure appropriate evaluations have been completed · Order blood To Holding · Obtain consents: Pre-operative teaching Area Phase · Blood consent and prepare for OR · Neurosurgery consent · Plastic Surgery consent · Complete Phase 2 of CIS PowerPlan and Checklist 2 For questions concerning this pathway, Last Updated: March 2019 contact: [email protected] Next Expected Review: March 2024 © 2019 Seattle Children’s Hospital, all rights reserved, Medical Disclaimer Craniosynostosis v4.0: Holding Area Phase Approval & Citation Summary of Version Changes Explanation of Evidence Ratings HOLDING AREA PHASE ! Order antibiotics early Vancomycin must be initiated 2 hours prior to incision, if used No, reschedule Off Ready for surgery surgery Pathway by Anesthesia evaluation? Yes Order FFP and peri-operative antibiotics · Cefazolin first-line · Clindamycin if history of cefazolin allergy, severe penicillin reaction, or history of MRSA sensitive to clindamycin · Vancomycin if history of MRSA resistant to clindamycin or with unknown sensitivities Ensure appropriate To Transfer pre-surgical preparation Phase · Complete Phase 3 of CIS PowerPlan and Checklist 3 For questions concerning this pathway, Last Updated: March 2019 contact: [email protected] Next Expected Review: March 2024 © 2019 Seattle Children’s Hospital, all rights reserved, Medical Disclaimer Craniosynostosis v4.0: Transfer Phase Approval & Citation Summary of Version Changes Explanation of Evidence Ratings PICU TRANSFER AND SURGICAL UNIT TRANSFER PHASES Continue peri-operative antibiotics · Cefazolin first-line · Clindamycin if history of cefazolin allergy, severe penicillin reaction, or history of MRSA sensitive to clindamycin · Vancomycin if history of MRSA resistant to clindamycin or with unknown sensitivities Complete surgery · Complete Phase 4 of CIS PowerPlan and Checklist 4 to track intra-operative activities for ICU handoff Transfer to ICU Post-operative monitoring · Jackson-Pratt drain · Hemodynamic stability · Continue peri-operative antibiotics · Encourage oral intake · Pain control No Prepare patient for floor transfer · Discontinue foley catheter To Discharge · Discontinue arterial catheter Transfer to floor Phase · Establish oral pain control regimen · Establish oral feedings · Complete Phase 5 of CIS PowerPlan and Checklist 5 For questions concerning this pathway, Last Updated: March 2019 contact: [email protected] Next Expected Review: March 2024 © 2019 Seattle Children’s Hospital, all rights reserved, Medical Disclaimer Craniosynostosis v4.0: Discharge Phase Approval & Citation Summary of Version Changes Explanation of Evidence Ratings DISCHARGE PHASE Receive patient on floor Discharge Planning · Remove Jackson-Pratt drain · Ensure clinic follow up established · Establish oral pain control · Encourage oral feeding · Discharge teaching Evaluate readiness for discharge · Complete Phase 6 of CIS PowerPlan and Checklist 6 Discharge Criteria · Temperature <38 C for last 24 hours · Pain controlled without IV meds > 4hrs · Tolerating enteral feeding · Evaluation of periorbital edema by ARNP or MD · Jackson-Pratt drain discontinued Discharge Instructions · Keep the incision clean with soap and water · Provide family with handout on incision care, discharge instructions, follow up plan · Call for: nausea or vomiting; fever >101.5 F; increased swelling, redness, or drainage from the incision; decreased level of consciousness or increased irritability For questions concerning this pathway, Last Updated: March 2019 contact: [email protected] Next Expected Review: March 2024 © 2019 Seattle Children’s Hospital, all rights reserved, Medical Disclaimer Craniosynostosis v4.0: First Year Follow-up Approval & Citation Summary of Version Changes Explanation of Evidence Ratings Follow-up For the First Year after Cranial Vault Reconstruction Based on Type of Intervention Inclusion Criteria Types of Interventions · Child with Craniosynostosis AND · Single Suture Synostosis · Candidate for Cranial Vault · Coronal Expansion · Crouzon/Pfeiffer Exclusion Criteria · Apert · Children with diagnosis other than Craniosynostosis or without Cranial Vault Expansion Initial post-op visits with PS Fronto-Orbital Posterior switch Modified Pi and Distraction Osteogenesis Endoscopic Advancement cranioplasty Posterior Vault of the cranial vault 1 week after hardware placement · Incision check 14-21 days (once helmet · Lateral skull film prior to has been fit) clinic visit on same day · Incision check · Assess helmet fit End of activation phase ARNP check in with (typically 12-16 days) Hanger 2-4 weeks post-op · Lateral skull film prior to Incision exam clinic visit on same day Evaluate at end of 1st helmet · Removal of activation · Incision check arms · Assess helmet fit Operative ARNP check · Order CT in with · Hardware removal Hanger · Immediate post-op CT · Same day discharge Evaluate at end of 2nd helmet, 3rd needed? 2-4 weeks post-op · Incision check rd · Incision exam · Assess for 3 helmet? Follow path for surgical Additional YES procedure surgery? above NO 1 year follow-up with full team · Shape assessment – 3dMD image · Developmental assessment · Clinical assessment for large, palpable skull defects · Screen: hearing, OSA, vision, concerns for pain, medical and dental home Single Suture Crouzon/ Coronal Apert Synostosis Pfeiffer For questions concerning this pathway, Last Updated: March 2019 contact: [email protected] Next Expected Review: March 2024 © 2019 Seattle Children’s Hospital, all rights reserved, Medical Disclaimer Craniosynostosis v4.0: Long Term Follow-up Sagittal, lambdoid, Metopic Approval & Citation Summary of Version Changes Explanation of Evidence Ratings Single Suture Craniosynostosis: Sagittal, Lambdoid, Metopic Inclusion Criteria · Child with Craniosynostosis AND · Candidate for Cranial Vault Expansion Exclusion Criteria · Children with diagnosis other than Craniosynostosis or without Cranial Vault Expansion Sagittal Lambdoid Metopic synostosis synostosis synostosis Post-operative Offer genetic consultation Follow-up 2 Years Post-op · Shape Assessment with clinical exam and 3DMD imaging · Clinical assessment for large (>3x3 cm), palpable skull defects · Assess development · Assess for features of a syndrome · Assess for symptoms of pain · LAMBDOID: Consider a HASTE MRI to assess for a chiari malformation Follow-up at 5-6 Years of age · Shape Assessment with clinical exam and 3DMD imaging · Clinical assessment for large (>3x3 cm), palpable skull defects · Assess development/learning · Assess