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NAPNAP Annual Conference March 30, 2019 Kapiolani Cleft and Craniofacial Center Joan Meister, MD

Craniofacial Care Through the Spectrum. NAPNAP Annual Conference March 30, 2019 Kapiolani Cleft and Craniofacial Center Joan Meister, MD Helen Lau, CPNP Diane Lynne Ching, MD.

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NAPNAP Annual Conference March 30, 2019 Kapiolani Cleft and Craniofacial Center Joan Meister, MD

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  1. Craniofacial Care Through the Spectrum NAPNAP Annual Conference March 30, 2019 Kapiolani Cleft and Craniofacial Center Joan Meister, MD Helen Lau, CPNP Diane Lynne Ching, MD

  2. No financial interest/arrangement or affiliation with one or more organizations that could be perceived as a real or apparent conflict of interest in the context of the subject of this presentation CREATING A HEALTHIER HAWAIʻI

  3. Objectives • Identify prenatal concerns for the expectant family • Identify 3 areas of concern for the baby with CL+ CP • Illustrate needs through the years for CL+ CP patient • Identify 3 tips to decrease risk for positional plagiocephaly CREATING A HEALTHIER HAWAIʻI

  4. Kapi‘olani Cleft and Craniofacial Center • Multidisciplinary team established in 2007 by 2 neonatologists to provide comprehensive care to cleft patients • American Cleft Palate and Craniofacial Association (ACPA)- approved team • 755 active patients (Nov 28, 2007 with 6 patients) • Cared for 1200 patients to date CREATING A HEALTHIER HAWAIʻI

  5. Treating patients with Cleft and Craniofacial Conditions • Cleft lip and cleft palate • Pierre Robin Sequence • 22q11.2 deletion syndrome • Craniosynostosis • Hemifacial Microsomia/Goldenhar • Microtia • Treacher Collins • Other conditions • Velopharyngeal dysfunction and submucous cleft palate CREATING A HEALTHIER HAWAIʻI

  6. Specialists • Audiologist • Craniofacial surgeon • Genetics Team – geneticist and/or genetic counselor • Occupational therapist • Oral surgeon • Orthodontist • Speech pathologist • Pediatric dentist • Plastic surgeonPediatrics Team – pediatricians and pediatric nurse practitioner • Behavioral health nurse practitioner • Social Worker By Referral: • Otolaryngologist (ENT) • Ophthalmologist • Dietitian • Pulmonologist • Neurosurgeon CREATING A HEALTHIER HAWAIʻI

  7. Babycenter.com CREATING A HEALTHIER HAWAIʻI

  8. In the Beginning… • Cleft lip – forms at about 7 weeks of pregnancy from LMP • Cleft palate –forms at 11 weeks from LMP • Risk Factors • Heavy alcohol consumption • Smoking • Certain prescription medications for acne and epilepsy medication • Topiramate & valproic acid (epilepsy), prednisone & amoxicillin (acne) https://www.jabfm.org/content/29/2/254.full https://cleftline.org/wp-content/uploads/2012/03/GEN-01.pdf CREATING A HEALTHIER HAWAIʻI

  9. Prenatal concerns • Guilt • Caused by certain foods • Full moon • Family genetics • Growth and Development • “Disabled” • Worried that they may not be able to talk or eat or hear • Teasing and getting along at school • Future QOL • Types of bottles to order • What to expect (+ or – CP) • Adjustment/coping • Typically sad that the normal baby is not “normal” anymore • How to tell family and friends • Finances • Work schedules CREATING A HEALTHIER HAWAIʻI

  10. Classification – Cleft lip • Cleft lip • Unilateral, bilateral or median/midline • Complete or incomplete • Example: “Right unilateral complete cleft lip” vs “Right unilateral incomplete cleft lip” • Cannot always tell prior to delivery but family will be counselled on types of CL CREATING A HEALTHIER HAWAIʻI

  11. What to do now that baby is born? Cleft Lip • Continue with normal newborn nursery baby care • Order newborn baby screening tests as usual including hearing screen • Nurse/feed as normal (breastfeeding or OTC bottle ok) • If a wide cleft lip, may be candidate for lip taping through KCCC • Some babies will have difficulty feeding CREATING A HEALTHIER HAWAIʻI

  12. Postnatal concerns • Immediately after birth Cleft palate or small jaw • Cleft palate surgery • Surgery to “break” the jaw • Trach • Breathing at night • Bringing home a new baby • Other defects/syndromes • Treat the baby or the defect • Surgery • Pain, length of hosp stay, timing • Miss the cleft lip • When—3-6 months of age CREATING A HEALTHIER HAWAIʻI

  13. Lip Taping • To Tape or Not to Tape, that is the question. • Approximate tissue • May be added stressor for the family • Cut and measure tape • Monitor cheeks for rash • ~ 3 months taping • Cost to the clinic (6 pks steri-strips, thin hydrocolloid dressing) • Dynacleft ($325-$425) – taping for 3 months CREATING A HEALTHIER HAWAIʻI

  14. PCP’s office • Routine WCCs • Pre-op H and P for surgery clearance at age 3-5 months old within 1 month of surgery • Support and education CREATING A HEALTHIER HAWAIʻI

  15. Soft tissue clefts in the Tessier Classification system Bone, soft tissue and skin can be affected http://oralmaxillo-facialsurgery.blogspot.com/2010/05/congenital-malformations-nose.html CREATING A HEALTHIER HAWAIʻI

  16. Boney cleft/soft tissue cleft https://upload.wikimedia.org/wikipedia/commons/1/12/Picture_Tessier_classification.jpg CREATING A HEALTHIER HAWAIʻI

  17. Dr. Paul Tessier • French general surgeon • Worked in the area of cleft lip repair in the 1940s • His work essentially created the field of craniofacial surgery • In the 1960’s and 1970’s, he developed techniques used in craniofacial surgery now • Surgeons performing procedures within the craniofacial specialty are either trained by Dr. Tessier or by surgeons he trained • Hence the Tessier Classification https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2740534/ CREATING A HEALTHIER HAWAIʻI

  18. Clinical Picture – midline cleft lip • Cleft palate or submucous cleft palate? Feeding difficulties? • Encephalocele in the palate? • Cleft nose? • Hyperteloric (wide spaced eyes?) • Vision concerns? • Endocrine concerns? CREATING A HEALTHIER HAWAIʻI

  19. Classification - Cleft palate • Cleft Palate • Complete or incomplete • Bilateral or unilateral complete CLCP • Submucous Cleft Palate • Defect in the palate muscle or the bone • May not be obvious • Intact mucosa • Clinical findings • Bifid uvula, notch at end of the hard palate, bluish tint (to mucosa) zona pellucida CREATING A HEALTHIER HAWAIʻI

  20. http://slideplayer.com/slide/5258425/16/images/16/Classification+of+Cleft+Palate.jpghttp://slideplayer.com/slide/5258425/16/images/16/Classification+of+Cleft+Palate.jpg CREATING A HEALTHIER HAWAIʻI

  21. What to do now that baby is born? Cleft Palate • Normal newborn nursery baby care • Newborn hearing screen and routine tests • Monitor jaw size, tongue size and breathing • Micrognathia and snoring or pauses in breathing/apnea? • Ok to attempt breastfeeding but unlikely to exclusively provide sufficient volume and calories to sustain good weight gain • Feeding consult CREATING A HEALTHIER HAWAIʻI

  22. Nursery Course—Cleft palate • May need longer stay to assure comfort and efficacy of feeding plan • May consider fortification of breastmilk/higher calorie formula • Bottle/nipple needs may change as baby gets stronger • Family should keep log of feeding volumes and durations • Expect nasal regurgitation CREATING A HEALTHIER HAWAIʻI

  23. PCP’s office • Frequent weight checks • Expect requests from KCCC for referrals to see feeding, peds, plastics, ENT, and hearing • Expect to schedule an H and P clearance for CP surgery about age 10 mon- 14 mons old to be completed within 1 mon of surgery • Referral to EIS • Reinforcement of consistent follow up and care CREATING A HEALTHIER HAWAIʻI

  24. KCCC • Coordinate with feeding therapist for an appt to check weight and feeding within a week in KCCC • Route note to PCP’s office after visit • Discuss timeline for repair • Cleft lip – 3-5 months old • Cleft palate 10-14 months old • Submit authorization for coverage of additional bottles • Ongoing monitoring of hearing and speech • Access to RD, SW, BH, genetics, DDS, orthodontist • Plastic surgeon will perform the cleft lip/palate surgery CREATING A HEALTHIER HAWAIʻI

  25. Bottles • Pigeon Bottle • Haberman Bottle (Medela) • Dr. Brown’s Specialty bottle CREATING A HEALTHIER HAWAIʻI

  26. Surgery Cleft Lip • Same day surgery ~ 3 hours long • Antibiotics and pain medications • No change in feeding practice Cleft Palate • 3-4 hr surgery • Overnight observation • Antibiotics and pain medications • Full Liquids Soft diet • Limitations • No straws, no brushing upper teeth, no S.T. CREATING A HEALTHIER HAWAIʻI

  27. Surgery • Use of “no-no’s” • Rationale for dietary restrictions post palate repair • Education for post-op behavior of patient • Education regarding post op visits and potential common complications CREATING A HEALTHIER HAWAIʻI

  28. Infancy and Toddler • Feeding/weight gain/proper use of oral muscles • Monitor for reflux • Encouragement of language opportunities and speech development • Vigilance with ear health and hearing • Importance of consistent speech therapy • Normal child—not “sick role” CREATING A HEALTHIER HAWAIʻI

  29. Childhood • Potential for palate revisions after age 3 “speech surgery” • Prolonged orthodontic treatments and interventions • Continued need for speech therapy • Vigilance with ear health and hearing • Social Emotional growth and confidence CREATING A HEALTHIER HAWAIʻI

  30. School Age • Continue to monitor hearing and speech • Follow up with Otolaryngology as needed • Snoring? CLCP higher risk for sleep disordered breathing • Behavioral Health • Consider impact of school transitions • Orthodontics evaluation and possible need for appliance to expand the maxilla in preparation for alveolar bone graft • May need PCP to request for medical authorization for orthodontics CREATING A HEALTHIER HAWAIʻI

  31. http://www.odlortho.com/product/fan-rpe/ CREATING A HEALTHIER HAWAIʻI

  32. School Age • Alveolar bone graft Surgery • Timing based on dental development • Bone harvested from the Iliac crest to stabilize the maxilla from 2 pieces to 1 piece • Surgeon can be craniofacial or oral surgeon CREATING A HEALTHIER HAWAIʻI

  33. Pre-operative course • H & P clearance from PCP within 1-2 weeks of surgery • Access to Child Life Services for child and family • Consent for surgery within 2 months of surgery • May be referred for a cone beam CT • Post operative • Overnight stay • Wound care of the graft site and donor site • Pain management • Diet restrictions • Oral hygiene • Shower/bath restrictions • Activity restrictions and no contact sports x 6 wks • May be referred for a cone beam CT to evaluate bony fill • Discharge home: mouthwash, antibiotics, analgesics CREATING A HEALTHIER HAWAIʻI

  34. School Age (cont.) • VPI (velopharyngeal insufficiency) and need for secondary palatal surgery? • Fancy word for disorder of the structure of the soft palate to close against the posterior pharyngeal wall during speech. • Proper closure closes off the airflow through the nose to be directed into the oral cavity for speech production CREATING A HEALTHIER HAWAIʻI

  35. Nasopharyngoscopy • Nasopharyngoscopy is the gold standard for direct assessment of VPI • Speech therapy, Otolaryngologist, plastic surgeon • Helps to determine treatment plan for VPI • If secondary palatal surgery is recommended, will need pre-op H & P and plan for post op care as before CREATING A HEALTHIER HAWAIʻI

  36. Adolescence • Monitor hearing and speech • Oral surgery evaluation to monitor jaw growth and pre-surgical planning if patient is a candidate for orthognathic surgery • Social Work • Discussion during early adolescence to encourage advocating for self, asking questions about own health, transition to adult care • Access to Child Life Services to address pre-surgical anxiety CREATING A HEALTHIER HAWAIʻI

  37. Orthognathic Surgery • Work up with oral surgeon • Timing and social impact • Cost and insurance coverage CREATING A HEALTHIER HAWAIʻI

  38. Head Shape • Just like the earth—the head should be round not flat. • Normal variants exist • When to be concerned • Is it all just appearance CREATING A HEALTHIER HAWAIʻI

  39. Normal Skull Sutures CREATING A HEALTHIER HAWAIʻI

  40. Plagiocephaly CREATING A HEALTHIER HAWAIʻI

  41. Craniosynostosis CREATING A HEALTHIER HAWAIʻI

  42. Classification • Abnormal Headshape • Craniosynostosis vs Positional Plagiocephaly • Coronal • Unilateral vs Bilateral/bicoronal • Sagittal • Lambdoid (rare) • Occipital flatness • Positioning of the ears from the top view • Affect the forehead • Torticollis? CREATING A HEALTHIER HAWAIʻI

  43. Synostosis vs Positional Plagiocephaly • Open vs closed AFOF, ridging? Pulled up eyebrow/eye? Forehead bulging, bitemporal pinching? HC small? Long AP? Short AP? Syndromic in appearance? Growth and development delays? • If HC big and increasing headsize, should consider referral to neurosurgery rather than KCCC to look at underlying cause of macrocephaly • Likely not craniosynostosis and may require a brain MRI CREATING A HEALTHIER HAWAIʻI

  44. Referral -plagiocephaly • When was it noticed • Acitivity level and positioning of baby • Ear position and sutures/fontanelles open • Birth history; multiple gestation; uterus • Imaging CREATING A HEALTHIER HAWAIʻI

  45. Referral - plagiocephaly • Positioning – tummy time, decrease time in carseat/swing • Torticollis? If so, refer to EIS and/or PT • Helmet therapy for plagiocephaly https://www.aans.org/Patients/Neurosurgical-Conditions-and-Treatments/Positional-Plagiocephaly CREATING A HEALTHIER HAWAIʻI

  46. Questions or Referrals? • Contact: • Helen Lau, CPNP • Joan Meister, MD • Diane Lynne Ching, MD • 808.983.8500 x1 • www.kapiolani.org/cleft CREATING A HEALTHIER HAWAIʻI

  47. References ACPA Family Services. Retrieved March 14, 2019 from www.cleftline.org. American Association of Neurological Surgeons, Positional Plagiocephaly. Retrieved March 14, 2019 from https://www.aans.org/Patients/Neurosurgical-Conditions-and-Treatments/Positional-Plagiocephaly Anderson, I.C., Sedaghat, A.R., McGinley, B.M., Redett, R.J., Boss, E., and Ishman, S. L. (2011). Prevalence and Severity of Obstructive Sleep Apnea and Snoring in Infants With Pierre Robin Sequence. The Cleft Palate-Craniofacial Journal. 48(5):614-8. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/20815709 Beck, S., and Marcus, C. (2009) Pediatric Polysomnography. Sleep Med Clin. 4(3): 393-406. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2739664/

  48. References Chan, J., Edman, J., and Koltai, P. (2004). Obstructive Sleep Apnea in Children. American Family Physician. 69(5):1147-1155. Retrieved from https://www.aafp.org/afp/2004/0301/p1147.html Lam, D., Jensen, C. Mueller, C. Starr, J. Cunningham, M. and Weaver, M. (2010). Pediatric sleep apnea and craniofacial anomalies: a population-based case-control study.Laryngoscope. 120(10): 2098-105. Retrieved from https://onlinelibrary.wiley.com/doi/abs/10.1002/lary.21093 MacLean, J.E.,Fitzsimons, D.,Fitzgerald, D.A., andWaters, K.A. (2002). The spectrum of sleep-disordered breathing symptoms and respiratory events in infants with cleft lip and/or palate. Archives of Disease in Childhood. 97(12):1058-63. http://www.ncbi.nlm.nih.gov/pubmed/23042804

  49. References Section on Pediatric Pulmonology, Subcommittee on Obstructive Sleep Apnea Syndrome. (2002). Clinical Practice Guideline: Diagnosis and Management of Childhood Obstructive Sleep Apnea Syndrome. Pediatrics. 109 (4): 704-71

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