While on call

25yo G1 P1 delivers a full-term infant after 12hrs of uneventful labor. The infant was found to have a cleft lip and palate. How should the care for this infant, and his mother, be?

Outline

Cleft Lip
Unilateral, Bilateral
Etiology, Epidemiology, pathophysiology,

treatment

Cleft Palate
Etiology, Epidemiology, pathophysiology,

treatment Operation technique

Incidence of Cleft Lip and Palate

1/750-1000 live births (2nd to club foot)

45% CL/P; 15% CL; 40% CP Males CL/P; females CL

2nd baby 1/30 (genetic counseling)

Aetiology
Genetic all forms of inheritance have been described , with chromosomal abnormalities (esp.trisomy 13 and 18) in 12% Environmental viral? steroids, anticonvulsants 5% Syndromes - occurs as part of >100 syndromes - 50% CP and 15 % CL/P have associated anomalies

Embryology of Cleft Lip

Weeks 4-7 frontonasal,maxillar y, and mandibular processes form the face Left CL due to failure of fusion of median nasal and maxillary processes

Prenatal Diagnosis by U/S at 12 weeks a good thing ?

Unilateral incomplete

Unilateral complete

Bilateral complete

Incomplete cleft palate

Unilateral complete lip and palate

Bilateral complete

Unilateral Cleft Lip

Complete

Incomplete

Bilateral Cleft Lip

Incomplete

Complete

Sequence of Interventions
Prenatal-1st few weeks plastic surgeon and nurse/coordinator; ?orthodontist 3 months hearing test 3-6 months lip repair +/ 10-18 months palate repair +/ 2-3 years -dental and speech evaluations

Sequence of Interventions (cont.)

5 years initial team conference 7-9 years team reassessment re lip and nose revision, orthodontia and bone graft, speech and pharyngoplasty 9-14 years orthodontia +/- bone graft Adolescence - ?lip and nose revision , team conference if necessary

The Neonatal Period

Pediatrician:
directs care
establishes feeding complete clefts preclude feeding
breast feeding not

possible a soft, large bottle with large hole is required a palatal prosthesis may be required

Presurgical Intervention

The Neonatal Period

Presurgical Orthodontics (Baby Plates)

Molds palate into

more anatomically correct position decreases tension may improve facial growth Grayson, presurgical nasal alveolar molding (PSNAM)

Cleft Lip Repair

Old rule of 10s Hb, age, weight Fetal or newborn risks Muscle and vertical height Nasal tip - when?

Techniques Cleft Lip Repair

Surgical Techniques

Cleft Lip Repair

unilateral rotation-advancement flap developed by Millard complications
dehiscence

- infection thin white roll - excess tension

Nasal Tip Revisions - Unilateral

Nasal Tip Revisions Bilateral

Pierre Robin Sequence

Micrognathia Glossoptosis ?horseshoe cleft palate 17% non- syndromic 34% Stickler (eyes, joints); 11% VCF Positioning > tongue-lip adhesion, floor of mouth release, tracheostomy or mandibular distraction

Objectives of Palate Repair

Normal speech Preservation of facial growth Preservation of hearing Separation of oral/nasal cavities Normal dental occlusion Normal swallowing

Embryology of Cleft Palate

weeks 8-11 Premaxilla from median nasal processes Palatal processes from maxillary processes fuse with premaxilla and nasal septum from anterior to posterior

Types of Palatal Clefts

Submucous Soft Unilateral bilateral

Eustachian Tube Dysfunction

Cleft Palate Team

Coordinator - ?nurse Plastic surgeon Otolaryngologist and Audiologist Speech Pathologist Dentist and Orthodontist Maxillofacial Surgeon

Cleft Palate Repair

V-Y Pushback Two Flap Palatoplasty Furlow Palatoplasty

Cleft Palate Repair

V-Y Pushback

Two uni-pedicled flaps (greater palatine artery) and one or two anteriorly based pedicled flaps Posterior flaps rotated in a V-Y advancement technique increasing the length of the palate Nasal mucosa not closed Improved speech results compared with bipedicled techniques Indicated for incomplete clefts

Cleft Palate Repair

Schweckendicks Primary Veloplasty

Incisions made in soft palate Muscle bundles released from the posterior hard palate and rotated Reconstruction of levator sling Closure of mucosal layers separately

Cleft Palate Repair

Furlow Palatoplasty

Lengthens the soft palate Reconstructs the muscle sling. Also commonly used to correct velopharyngeal insufficiency in patients with submucous cleft palate Speech outcomes are improved compared with other palatoplasty techniques.

Cleft Palate Repair

Complications

Oronasal fistula - 8.7% to 23%

Sites of fistulization are typically the anterior

hard palate and the junction of the hard and soft palate.

Velopharyngeal insufficiency

Pharyngoplasty
Timing motivation,caries, orthognathic surgery Unpredictable insufficient improvement, sleep apnea Risks bleeding, dehiscence, infection,obstruction

Orthodontia and Alveolar Bone Grafting

Terimakasih