• A. Van der Woudes syndrome• 1. Autosomal dominant, with variable penetrance.• 2. Associated with CLP or CP (40%-50% penetrance).• 3. Associated with lip pits (accessory salivary glands, 70%-80% penetrance).• 4. May also have absent second molar, syndactyly, abnormal genitalia, and popliteal pterygia.• B. Waardenburgs syndrome• 1. A group of anomalies arising from abnormal development and migration of neural crest cells.• 2. Features may include cleft lip, cleft palate.• C. Down syndrome (trisomy 21)• D. Trisomy 13• E. Sticklers syndrome• 1. A group of anomalies caused by connective tissue dysplasia.• 2. Typical features: Cleft palate, progressive joint degeneration, and various ocular abnormalities that may lead to blindnes s.• 3. Autosomal dominant inheritance.• 4. Other anomalies: Cardiac, sensorineural, and learning disorders or mental retardation.
INCIDENCE• 1. The overall incidence is 1 in 1,000 live births.• 2. White ancestry: 1 in 750 live births.• 3. Asian ancestry: 1 in 500 live births.• 4. African ancestry: 1 in 2,000 live births.
DEMOGRAPHICS• The ratio of left (L) to right (R) to bilateral (B) clefts (L:R:B): 6:3:1.• The ratio of CLP to CL is 2:1.• Three percent are syndromic.
GENETICS• The risk of having a child with CLP .• a. If parents have one child with CLP: 4%.• b. If one parent has CLP: 2% to 4%.• c. If parents have two children with CLP: 9%.• d. If one child and one parent have CLP: 14% to 17%.• Most cases are sporadic (and multifactorial), but may be X-linked, autosomal dominant (Van der Woudes syndrome) or familial.
• First documented cheiloplasty occurred in china in 390 A.D.• Earliest report in western medical literature is by Yupman(1295-1350).• Ambrose Pare , a Renaissance surgeon, used hare lip needles for repair.• Rose in 1891 & later Thompson 1912 ,used a straight line repair.• Malgaigne in 1843 , introduced the concept of flaps.• Mirault used vermillion flap.• Blair & Brown modified the straight line repair with a triangular flap of vermillion &lower lip skin in 1930-1940.• Cardoso was the first to emphasize the importance of “Cupid’s bow”. Hagedorn used a quadrengularly shaped flap in 1884.• Tennison described the use of a triangular flap of the lower lip skin adjacent to the vermillion inserted into a back-cut in the medial lip.• Millard in 1955 introduced the rotation advancement flap concept.
HISTORY• Age of the patient• Problem with feeding , airway• Any other associated anomalies• Prenatal history of any drug use, alcohol, smoking etc• Family history• consanguinity• Previous surgeries• Drug history
EXAMINATION• Examination of the cleft: type, width, associated cleft alveolus or palate ,nasal deformaty,• Systemic examination
CLASSIFICATION• Comeplete• Incomplete• Microform• LAHSHAL otto kriens• Kernahan’s classification and its modifications.
PRENATAL DIAGNOSISDiagnosis is usually made in the second orthird trimester.With the advancement of 3-DUltrasonography ,visualization of the cleftlip has become more convenient accurate.Fetal surgery results in less scarring andcan be performed laparoscopically ,but it isdone rarely.
GOALS AND PRINCIPLES• Approximation of medial and lateral lip elements should be achieved without loss of natural landmarks• The scar of union should be placed along natural lines, with appreciation of the anatomical subunits of the lip-nose complex.• The muscle should be repaired.• The nostril margins should be of equal circumference and the alar bases should be symmetric from the anterior view.• The ideal repair should approximate the medial and lateral lip elements appropriately at all levels without interruption or loss of landmarks and achieve balance by providing length where tissue is short and excision where height is excessive.• “nostril sill roll” is often present and should be used as a tool to accurately approximate the nostril sill on both sides of the cleft and to give natural appearing nostril sill.• Restoration of bony platform.• Reconstruction of distorted nasal anatomy .
STAGING OF INTERVENTION• A. Initial evaluation• 1. Reassure the parents and family that they are not to blame.• 2. Explain the stages and operations that should be expected throughout the childs lifetime.• 3. Evaluate for associated anomalies.• 4. Consultations• a. Genetics, for evaluation and possible counseling• b. Social work• c. Feeding/nutrition• (1) The child may need special nipples or bottles (e.g., cross-cut nipple).• (2) Monitor for appropriate weight gain.• d. Otolaryngology: Children with cleft lip and palate have a high incidence of eustachian tube dysfunction, and therefore otitis media, requiring close follow-up.• (1) The child may need myringotomy tubes.• (2) If untreated, repeat otitis may affect hearing and speech development.
• B. Wide clefts (>1 cm)• 1. Goal: Bring the segments closer together to facilitate a tension-free repair.• a. Has not been shown to change skeletal development in the anteroposterior direction.• b. Does not seem to prevent future crossbite.• 2. Passive: Preoperative taping• a. Steri-Strip tapes applied across both segments of the lip.• b. Requires reliable parents who can reapply the tape and keep it on at all times.• 3. Passive: Lip adhesion operation• a. Suturing the edges of the cleft together is performed under anesthesia.• b. The definitive lip repair is performed once the segments have moved closer together.• c. Variable success.• 4. Active: Latham-type device• a. An orthodontic appliance that must be placed onto the palatal segments under anesthesia.• b. Parents turn a screw daily, which slowly brings the palatal segments into better alignment.• c. Removed at the time of definitive lip repair.
• C. Repair• 1. Timing (controversial)• a. Repair at 3 months is generally accepted.• b. Some argue for earlier repair in order to produce better scars.• 2. Rule of tens: For increased anesthetic safety, an infant should• a. Be 10 weeks old.• b. Weigh 10 pounds.• c. Have a hemoglobin level of at least 10 mg/dL.• 3. Cleft palate repair and secondary alveolar grafting .• 4. May also choose to address the cleft nasal deformity at time of lip repair.• VII. Intraoperative considerations• Peak of Cupids bow and midline on the medial segment. Measure the anticipated distance for the new Cupids bow (approximately 3-4 mm).• e. Peak of Cupids bow on the lateral segment.• 2. Account for distortion from the uncountered pull of the orbicularis on the medial segment. The philtral columns are usually slightly C-shaped.• B. Mark lines for expected repair type.• C. Only after marks are completed, infiltrate tissue with local anesthetic to avoid distortion of anatomy and measurements.
ADVANTAGES AND DISADVANTAGES OF DIFFERENT TECHNIQUES• MILLARDS REPAIR ADVANTAGES : * ADVANCEMENT CORRECTS THE FLARING OF ALA * C-FLAP…. UNILATERAL COLUMELLAR LENGTHENING * SCAR IN THE NATURAL PHILTRAL COLUMN POSITION * PRESERVATION OF PHILTRAL DIMPLE * PRESERVATION OF CUPIDS BOW * IT IS POSSIBLE TO ADJUST THE DEGREE OF LIP LENGTHENING DURING SURGERY, HENCE IT HAS BEEN LABELLED A ‘CUT-AS-YOU-GO TECHNIQUE’. DISADVANTAGES : *IT PLACES A SCAR ACROSS THE PHILTRUM AT THE NASAL BASE. INADEQUATE FLAP ROTATION LEADING TO NOTCHING AND INADEQUATE VERTICAL LIP LENGTH.
• TENNISON–RANDALL TECHNIQUE ADVANTAGES:EASY TO LEARNHEIGHT OF WHITE LIP AND SYMMETRY OF THE CUPID’S BOW WERE BETTER RESTOREDBY USING THE TENNISON TECHNIQUE.
POST OPERATIVEEVALUATION OF UNILATERALCLEFT LIP REPAIR
• Height of white lip and symmetry of the Cupid’s bow were better restored by using the Tennison technique.• The physiological confi guration of the white lip and less visible scars were achieved by using the Olekas technique. All techniques were equal in red lip and nose formations.