Unilateral cleft lip

Unilateral cleft lip diagnosis and management

1. Unilateral cleft lip

2. Epidemiology Cleft lip and palate (46%) Isolated cleft palate (33%) Isolated cleft lip (21 %) Associated with cleft palate: bilateral cleft lips (86%) and unilateral cleft lips (68%) Unilateral clefts are nine times as common as bilateral clefts and occur twice as frequently on left side than on the right

3. Males are predominant in cleft lip and palate population Isolated cleft palate occurs more commonly in females. In Caucasian population, cleft lip with or without cleft palate :1 in 1,000 live births. CLP twice as common in Asian population, and approximately half as common in African Americans. Isolated cleft palate: 0.5 per 1,000 live births .

4. lntrauterine exposure to phenytoin : 10-fold increase cleft lip. Maternal smoking during pregnancy doubles incidence of cleft lip. Other teratogens , alcohol, anticonvulsants, and retinoic acid, are associated with malformation patterns that include cleft lip and palate

5. Most common syndrome associated with cleft lip and palate is van der Woude syndrome Velocardiofacial , DiGeorge , or conotruncal anomaly syndromes are the most common diagnoses associated with isolated cleft

6. Anatomy

7. Subcutaneous cleft lip Partial cleft lip Complete cleft lip

8. Subcutaneous cleft lip

9. Cleft of muscle only Intact skin and mucosa ??Nasal deformity

10. Partial cleft lip Vermilion only  2/3 rd of lip height Divided muscle cause displacement of alar base laterally and inferiorly Vermilion tissue curves upwards Hypoplastic changes at edges of cleft

11. Complete cleft lip Displacement of non cleft element Ala displaced laterally, inferiorly, posteriorly Ala is flat, elongated S-shaped Lateral distortion of the nasal septum

12. Lip and Columella Superiorly turned vermillion border Merged columella

13. More muscle forces on non cleft side  upward and outward rotation of premaxillary segment Reduced functional input on cleft side  collapse and medial displacement of maxillary segment

14. History Hippocrates referenced presence of cleft lip but not its repair Chinese physicians first to repair cleft lip deformity around 390 A.D by direct closure Flemish physician Yperman (1295–1350), performed first modern cleft lip repair, or cheiloplasty

15. Straight-line repair as described by Rose and Thompson Triangular flap methods- Tennison , Skoog , and Randall 1957, Millard used rotation-advancement method 1995, Fisher

16. Timing of lip repair Rule of 10 10 weeks 10 pounds Hb - 10 gm% WBC< 10000/mm3

17. First 2 weeks Early surgery: To recreate functional balance of facial musculature Facilitate feeding Minimize traumatic experience of patient

18. Lip adhesion Use of tape or surgically Advantages: Approximation of maxillary segments Improved symmetry of skeletal base Disadvantage: Two surgeries, waste of tissue Lesser segment collapse

19. Pre surgical orthopedic treatment Primary goal: realign maxillary segments Two types: active and passive During first 2 to 3 months after birth, active soft tissue and cartilage molding can take place Matsuo and Hirose

20. Active appliances Use an acrylic plate and controlled forces, sometimes from extra-oral traction (bonnet with straps) Pin-retained variety (Latham) Passive appliances Alveolar molding plate made of a hard outer shell and a soft acrylic lining Nasoalveolar moulding (NAM)

21. Nasoalveolar moulding

22. Latham device

23. Surgical techniques of complete cleft repair Lateral quadrilateral flap: LeMesurier repair Lateral triangular flap: Introduce flap in lower half of medial lip Tennison repair Rose-Thompson repair Introduce lateral flap in upper half Trauner Millard Mohler rotation advancement

24. Principles Establish a symmetric, balanced Cupid's bow and whilte roll Construct an adequate dry vermillion height Construct a philtral column with same shape and height as philtral column on non-cleft lip side Construct a normal unscarred columella and establish a symmetric columellar -labial junction

25. Reorient and repair the orbicularis oris muscular sling Create an adequate labial sulcus Correct cleft nasal alar deformity Atraumatic , nonlinear skin closure.

26. Advantages: Minimal or no tissue is discarded Suture line camouflaged Technique flexible Normal looking cupids bow Good access to nose for primary reconstruction Supplies tissue for deficient areas of nose

27. Disadvantages: Contracture of vertical scar- notching of vermilion/ lip height shortening In case of severe tissue deficiency, lateral advancement flap under tension Extensive undermining of cheek Slightly difficult Difficult to overcome mismatch of vermilion

28. Surgical repair of microform cleft lip Vertical height of affected side=normal side Elliptical excision and straight repair Triangular flaps of white roll and vermilion Vertical height difference > 1-2 mm Modified Mohler rotation-advancement repair

29. Primary cleft nose repair Principles Symmetry of nasal floor, ala, dome on both sides Proper nasal tip projection Symmetric repositioning of lower lateral cartilage Closure of nasal floor Repositioning of alar base Reshaping of nasal ala

30. Postoperative instructions Arm restraints Careful bathing is allowed after 24 hours. Lip and nasal area washed with simple soap and water starting 3 days after surgery. Patients are allowed to return to bottle feeding immediately after surgery. The first follow-up visit is 1 week after surgery. At this time sutures removed. One month after repair gentle massaging of scar several times a day and continued for 6 months.

31. Complications Airway related complication (1.4%) Infection: antibiotics 5-7 days post op Wound dehiscence: suture removal after 5 days+ tape splintage Scar contracture/ hypertrophy: Taping 2-3 months Massage Vit E cream

32. Secondary lip deformities: notching/ asymmetric vermilion, short/ long/tight lip Secondary nasal deformities: short/ deviated columella , horizontal/ small/ large nostril, flat ala, depressed alar base, asymmetric nasal tip, caudal septal dislocation

33. References Bardach’s Surgical technique in cleft lip and palate. Grabb and Smith’s plastic surgery. 7 th edn . Unilateral Cleft Lip—Approach and Technique. Salyer . Semin Plast Surg. 2005 Unilateral Cleft Lip and Nasal Repair: Techniques and Principles. Meara . Iran J Pediatr . 2011