CLEFT LIP & CLEFT PALATE

1. CLEFT LIP & CLEFT PALATE Dr. Ryan Fernandes Asst. professor Department of surgery

2. Development of Face Face develops from the • Median nasal process • Lateral nasal process • Maxillary process • Mandibular arch • Globular arch • Olfactory pit & eye Any change in the development or fusion of these arches leads to formation of different types of cleft lip or cleft palate

4. INCIDENCE Common in caucasians 75% cases- unilateral Commonly occurs on the left side 50% cases – combined cleft lip & palate Common in boys 15- 25% of cases cleft lip alone 25-40% of cases cleft palate alone

5. Aetiology Familial – more common in cleft lip or combined cleft lip & palate Protein & vitamin deficiency Rubella infection Radiation Chromosomal abnormalties Maternal epilepsy & drug intake during pregnancy (steroid/ eptoin/ diazepam)

6. CLEFT LIP Central – rare Seen in upper lip between 2 medial nasal processes (Hare Lip) Lateral -maxillary & median nasal processes, commonest, can be unilateral or bilateral Incomplete cleft lip does not extend into the nose Complete cleft lip extends into the nasal floor Simple cleft lip is only the cleft in the lip Compound cleft lip is cleft lip with cleft of the alveolus

7. Anatomy Of Cleft Lip Occurs due to disruption of muscles of the upper lip & nasolabialregion. The facial muscles are divided into 3 muscular rings of Delaire – Nasolabial muscle ring Bilabial muscle ring Labiomental muscle ring

8. Unilateral Cleft Lip Nasolabial & bilabial muscle rings are disrupted on one side. Results in asymmetrical deformity involving the external nasal cartilages, nasal septum & anterior maxilla. These deformities influence the mucocutaneous tissues causing a displacement of nasal skin onto the lip as well as changes to the vermillion & lip mucosa

9. Unilateral Cleft Lip

10. Bilateral Cleft Lip Here the deformity is more profound but symmetrical. The two superior muscular rings are disrupted on both sides producing a flaring of the nose, a protrusive pre maxilla & an area of skin in front of the pre maxilla devoid of muscle known as prolabium.

11. Bilateral Cleft Lip

12. CLEFT PALATE It is due to failure of fusion of two palatine processes. Defect in fusion of lines between pre-maxilla (developed from median nasal processes) & palatine processes of maxilla one on each side. When premaxilla & both palatine processes do not fuse, it leads into complete cleft palate(Type I cleft palate)

13. Incomplete fusion of these 3 components can cause incomplete cleft palate beginning from uvula towards posteriorly at various lenghts So it could be – Type II a – bifid uvula Type II b – bifid soft palate Type II c – bifid soft palate & posterior part of hard palate.

15. Small maxilla with crowded teeth, absent/poorly developed upper lateral incisors Bacterial contamination of upper respiratory tract with recurrent infection is common. Chronic oitits media with deafness may occur Swallowing difficulties to certain extent & speech problems may occur. Cosmetic problems may occur.

16. Cleft Palate Embryologically, the primary palate consists of all anatomical structures anterior to the incisive foramen, namely the alveolus and upper lip. The secondary palate is defined as the remainder of the palate behind the incisive foramen, divided into the hard palate and, more posteriorly, the soft palate.

17. Cleft palate results in failure of fusion of the two palatine shelves. This failure may be confined to the soft palate alone or involve both hard and soft palate. When the cleft of the hard palate remains attached to the nasal septum and vomer - incomplete. When the nasal septum and vomer are completely separated from the palatine processes - complete

19. Soft palate The muscle fibres of the soft palate are orientated transversely with no significant attachment to the hard palate. In a cleft of the soft palate the muscle fibres are orientated in an anteroposterior direction, inserting into the posterior edge of the hard palate

20. Hard palate The normal hard palate can be divided into three anatomical and physiological zones. The central palatal fibromucosais very thin and lies directly below the floor of nose. The maxillary fibromucosa is thick and contains the greater palatine neurovascular bundle. The gingival fibromucosa lies more lateral and adjacent to the teeth.

21. Classification Cleft lip alone Cleft of primary palate Cleft of secondary palate Cleft of both primary & secondary palates Cleft lip & cleft palate together

22. Cleft lip alone Unilateral Bilateral Median

23. II. Cleft of primary palate only This lies in front of the incisive foramen. Complete – absence of pre maxilla Incomplete – rudimentary pre maxilla Unilateral Bilateral Median

24. III. Cleft of secondary palate only This is the cleft which lies behind the incisive foramen Complete – nasal septum & vomer are separated from palatine process Incomplete Submucus It can be - cleft with soft palate involvement cleft without soft palate involvement

25. Defect is often associated with other congenital anomalies – • Pierre-Robin syndrome – isolated cleft palate, retrognathia, posteriorly displaced tongue. • KlippelFeilsyndrome,Stickler’s syndrome(eye, skeletal,muscular,cleft disorder)

26. Problems in cleft disorders Difficulty in sucking & swallowing Speech is defective especially in cleft palate mainly to phonate B,D,K,P,T & G. Recurrent upper respiratory tract infection Chronic otitis media, middle ear problems Altered dentition or supernumerary teeth Respiratory obstruction Cosmetic problems

27. Primary Management Antenatal diagnosis An antenatal diagnosis of cleft lip, whether unilateral or bilateral, is possible by ultrasound scan after 18 weeks of gestation. Isolated cleft palate cannot be diagnosed by antenatal scan. When an antenatal diagnosis is confirmed, referral to a cleft surgeon is appropriate for counselling to allay fears.

28. Antenatal (Contd) Photographs of cleft lip shown to parents ‘before and after’. Introduction to a parent support group and meeting parents of a child with a similar cleft who has undergone surgery

29. Feeding Most babies born with cleft lip and palate feed well and thrive, provided that appropriate advice is given and support is available. Some mothers are successful in breastfeeding, particularly when the cleft is incomplete and confined to the lip. Good feeding patterns can be established with soft bottles and modified teats.

30. Feeding(Contd) Simple measures, such as enlarging the hole in the teat, often suffice. Feeding plates, constructed from a dental impression of the upper jaw, are rarely necessary to improve feeding.

31. Airway Hypoxic episodes during sleep and feeding. Intermittent airway obstruction is more frequent and is managed by nursing the baby prone. Persistent airway compromise - ‘Retained nasopharyngeal intubation’ to maintain the airway. Surgical adhesion of the tongue to the lower lip (labioglossopexy) in the first few days after birth is an alternative

32. Delaire timing of cleft surgery Unilateral/bilateral cleft lip alone,in one stage operation done in 4 to 6 months. Cleft palate alone involving only soft palate, in one stage surgery is done in 6 months. For cleft palate alone involving both soft & hard palates - soft palate in 6 months, hard palates in 18 months. In combined cleft lip & palate, unilateral or bilateral in 2 stages – cleft lip & soft palate in 6 months, hard palate in 18 months

33. Treatment for Cleft Lip Millard criteria used to undertake surgery for cleft lip (Rule of 10) 10 kg in weight 10 weeks old 10gm% haemoglobin

34. Millard cleft repair by rotating the nasolabial flaps Management of associated primary or secondary cleft palate deformity Proper post operative management like control of infection, training for sucking, swallowing & speech Tenninson’s Z plasty (Tenninson Randall triangular flap)

35. Cleft lip surgery Skin incisions are developed to restore displaced tissues, including skin and cartilage, to their normal position, while gaining access to the facial, nasal and lip musculature. Muscular continuity is achieved by subperiosteal undermining over the anterior maxilla. Nasolabial muscles are anchored to the premaxilla with non-resorbable sutures.

36. Oblique muscles of orbicularisoris are sutured to the base of the anterior nasal spine and cartilaginous nasal septum. Closure of the cleft lip is completed by suturing the horizontal fibres of orbicularisoris to achieve a functioning oral sphincter.

37. Cleft palate surgery Cleft palate closure can be achieved by one- or two-stage palatoplasty. The surgical principle is mobilisation and reconstruction of the aberrant soft palate musculature together with closure of the residual hard palate cleft by minimal dissection and subsequent scar formation

38. Principles Of Palatoplasty Timing is between 10 -18 months. Mucoperiosteum flap is raised. Palatal defect is closed using 3 layers – nasal, muscle or oral layers.

39. Hook of pterygoidhamulus is fractured to relax tensor palate muscle to relieve tension on suture line. Excess scar formation in the palate adversely affects growth and development of the maxilla.

40. Cleft palate is usually repaired in 12 to 18 months. Early repair causes retarted maxillary growth- due to trauma to the growth centre & periosteum of the maxilla during surgery if done early. Late repair causes speech defect.

41. Both soft & hard palates are repaired. Abnormal insertions of tensor palati is released. Mucoperiosteal flaps are raised in the palate which is sewed together. If maxillary hypoplasia is present, then osteotomy of the maxilla is done. With orthodontic help teeth extraction & alignment of dentition is done.

42. Regular examination of the ear, nose & throat during follow up period Post operative speech therapy Whenever complicated problems are present, staged surgical procedure is done. WardillKilner push back operation by raising muco-periosteal flaps based on greater palatine vessels

43. Secondary Management Hearing support is given using hearing aids. Speech problems occur due to velopharyngeal incompetence, articulation problems also can occur- speech therapy is given. It is corrected by pharyngoplasty, veloplasty, speech devices.