cleft lip and cleft palate facial defceta.pptx

This is presentation of facial defects in children and their management especially the cleft anomalies of face

1. Cleft lip and cleft palate By Dr.Sai Likhitha final yr Post graduate Under guidance of PROFESSOR DR P.SATYA SRINIVAS;MS

2. TABLE OF CONTENTS Embryological development of face Introduction of cleft lip and cleft palate Epidemiology Aetiology Cleft anatomy Classification Management

3. Development of face Starts at 4 th week of embryo development .and gets completed at 10 th week. Face is derived from the structures that lie around the stomatodeum : Unpaired- frontonasal process Paired- first pharyngeal arch(mandibular arch) of each side Mesoderm covering developing forebrain----frontonasal process Ectoderm of frontonasal process---- nasal placodes--- nasal pits-----nasal cavities ,medial nasal processes and lateral nasal processes Lateral and caudal to nasal placodes are lens placodes.

4. Development of face ( continued ) Muscles of face are derived from 2 nd branchial arch , hence innervates by facial nerve Individual development of each part of face mouth nose and nasal cavities, paranasal sinuses ear eye cheeks palate

5. Time table of developmental events

6. Craniofacial anomalies Clefts Synostoses Atrophy-hypoplasia Hypertrophy –hyperplasia-neoplasia

7. Cleft lip and Cleft palate

8. Introduction Orofacial clefting is the most common birth defect in the world Normal anatomy of lips

9. Introduction ( continued ) Typical distribution of cleft types: cleft lip alone: 15% cleft lip and palate: 45% Isolated cleft palate: 40%

10. Epidemiology Most common in South East Asian and Native Americans. Incidence is 1 in 450 live births in these population Occurs spontaneously in Caucasian populations with incidence of 1 in 1000 live births 1 in 2000live births in African Americans Male predilection Left sided cleft lip is more common 29% have associated anomalies Family history is the strongest known predictive factor.

11. Epidemiology ( continued ) Isolated cleft palate occurs in 1 in 2000 live births More common in females 50% have a diagnosable syndrome and additional congenital anomalies

12. Aetiology and risk factors Family history Van der Woude syndrome- IRF6 mutation Stickler syndrome – procollagen gene DiGeorge , Down,Apert and Treacher Collins syndromes Maternal smoking and alcohol consumption Early exposure to anticonvulsant drug( phenytoin, valproate and diazepam) Folate deficiency Use of steroids Retinoid excess( vit A)

13. Clinical anatomy Muscle chains of the face Muscosal zones of hard palate

14. Cleft anatomy Continuity of orbicularis oris muscle is disrupted Aberrant muscle insertion into piriform aperture laterally and the anterior nasal spine medially contributes to the hall mark appearance of cleft lip and nasal deformity. Levator veli palatini muscle run parallel to cleft margin and insert aberrantly into posterior edge of the hard palate

15. Classifications: CLEFT LIP: Unilateral or bilateral Microform, incomplete or complete CLEFT PALATE: VEAU CLASSIFICATION . Veau 1 – midline and limited to soft palate . Veau 2 – extend anteriorly to involve midline of the posterior hard palate . Veau 3 – complete unilateral cleft of primary and secondary palate . Veau 4 – bilateral complete clefts Submucous cleft palate- traid of bifid uvula, zona pellucida , and a palpable notch of the posterior hard palate.

16. Classifications ( continued ) LAHSHAL code Complete or incomplete Nagpur classification:

17. Management The vast majority of management is delivered by multidisciplinary teams (MDTs) within specialist centres. The cleft team: Cleft coordinator/administrator Clinical nurse specialist Paediatrician Speech and language therapist ENT/Audiology Paediatric dentist Orthodontist Clinical psychologist Cleft surgeon

18. Management ( continued ) Prenatal management: prenatal surgery remain at present experimental in general the options are for termination or best supportive care in preparation for birth. Anomaly scan at 20 weeks – isolated cleft palate cannot be diagnosed. Neonatal management: aimed at addressing the urgent issues relating to the airway, breathing, eye protection and establishing feeding

19. Management ( continued ) Management in infancy at this age treatment fall into 2 categories: that directed at major functional issues and at skull surgery in cases of craniosynostosis . There is possibility of surgery in severely retrognathic patient at this level, most effective is distraction osteogenesis technique . Expressed breast milk is best . Soft bottles, feeding plates(constructed from dental impression of upper jaw), nasoalveolar moulding(NAM) – reduce width of the cleft and improve shape of the nose prior to surgery. .Management in Early childhood (1-7years) aimed at dealing with functional problems – airway obstruction, speech and feeding issues. Psychological and emotional support

20. Hearing- OME should be treatedies Speech- problems faced are velopharyngeal incompetence and articulation errors. Lateral videofluoroscopy and nasendoscopy are performed to assess Pharyngoplasty procedures are performed. Dental- correction of dental abnormalitues Dietary advice, fluoride supplements and fissure sealants. Wound healing/aesthetics-

21. Management ( continued ) Management in late childhood to maturity(7-12years) . Aimed at optimising the overall appearance
Usually skeletal and dental abnormalities are addressed first and then
soft tissues. Alveolar bone grafting . Adjunctive secondary surgeries can be performed

22. Management ( continued ) Orthodontc treatment – Performed in 2 phases . At age of mixed dentition (8-10years) At age of permanent dentition(12-18years) Secondary/revision surgery to improve aesthetics and/or function. Orthognathic surgery- usually performed at 16-17 yrs in females and 17-19 yrs in males

23. Time line of surgeries performed

24. Principles of cleft surgery Ultimate aim is to facilitate normal development and welll being. Essentially, oral and dental health should also be optimised in the management. Key outcomes measured include speech, facial growth, general wellbeing and dental health. Emphasis is placed on muscular reconstruction of the lip,Bose and face as well as muscles of the soft palate. Historically, studies have recommended initiating surgical repair by the “Rule of 10s.” This states that a baby should be at least 10 weeks of age or older, achieve a weight of 10 pounds, have a hemoglobin exceeding 10 g/dL, and have a white blood cell count <10,000/mm 3 before undergoing surgery.

25. Surgical techniques CLEFT LIP: Millards rotation and advancement technique Tennison Randal technique Triple wedge technique

26. Surgical techniques CLEFT PALATE: Double reversing Furlow z- plasty Waldill-kilner procedure Von- langenbeck operation Intravelar veloplasty