CLEFT LIP & CLEFT PALATE / paediatric ..

Congenital disorders

1. CLEFT LIP & CLEFT PALATE

2. INTRODUCTION Cleft lip and cleft palate are congenital malformations resulting from the failure of fusion of maxillary processes during intrauterine development. The defect may occur either alone or together.

3. CLEFT LIP A cleft lip result from failure of maxillary process with nose elevation on frontal prominence. The extent of defect varies from a notch in the lip (partial or incomplete cleft) to a large cleft reaching the floor of nose (complete cleft). Cleft lip can occur on one side (unilateral)or may be on both sides (bilateral).

4. It results from failure of fusion of the hard palate with each other and with the soft palate. Cleft lip also usually occurs with cleft palate. Cleft palate may be complete (involving hard and soft palate, possibly including a gap in the palate) or incomplete (a ‘hole’ in the roof of the mouth, usually in soft palate). CLEFT PALATE

5. TYPES

6. INCIDENCE Cleft lip - 1 in 750 births Cleft palate – 1 in 2500 births Cleft lip is predominantly seen in males and cleft palate in females.

7. ETIOLOGY Cause is unknown. Most physicians believe that clefts occur due to a combination of genetic and environmental factors. Potential causes may be: Medications taken by the mother during pregnancy like anticonvulsants, acne medications containing Accutane and drug used for treating cancer, arthritis and psoriasis i.e.methotrexate . Exposure to viruses or chemicals while fetus is developing in the womb Exposure to X-ray Maternal conditions like anemia, Hypoprotinemia etc. Maternal intake of alcohol Maternal smoking during pregnancy

8. PATHOPHYSIOLOGY Failure or incomplete union of embryonic structures of the face results in cleft lip and palate. Fusion of maxillary and premaxillary processes normally occurs between fifth and eight week of gestation. The palatal processes fuse with in a month Failure of fusion results in cleft lip and cleft palate. This complete or partial non-union may affect the palatal bone and upper lip along with maxilla, premaxilla and tissues of the soft palate and uvula.

9. DIAGNOSTIC EVALUATION Antenatal ultrasonography Physical examination of the mouth – A gloved finger placed in the mouth to feel the defect or visual examination with a flash light will confirm the diagnosis.

10. COMPLICATIONS FEEDING PROBLEM – Due to a separation in the lip or opening in the palate, sucking is ineffective and the food and liquids can pass from the mouth back through the nose. There may be aspiration of feeds. RESPIRATORY INFECTIONS – Aspiration of feeds may result in infections like aspiration pneumonia. EAR INFECTIONS/HEARING LOSS – Children with cleft palate are at an increased risk of ear infections. Usually middle ear infections occur. If left untreated, may result in hearing loss. SPEECH PROBLEMS – The voice of these children may take a nasal sound and speech may be difficult to understand. DENTAL PROBLEMS – More prone to dental cavities and often have missing, extra, malformed or displaced teeth

11. SURGICAL MANAGEMENT - CLEFT LIP It may require one or two surgeries depending on the severity of defect. The initial surgery is usually performed at the age of 3 months. Common procedure for repair are Tennison Randall Triangular Flap (Z- plasty ) and Millard’s Rotational Advancement technique. Surgeons may also combine these two techniques, if needed.

12. SURGICAL MANAGEMENT - CLEFT PALATE Requires multiple surgeries First repair usually occurs when the baby is between 6-12 months. The initial surgery creates a functional palate, reduces the chance of fluid entering the middle ears and helps in proper development of teeth and facial bones. Children may need a bone graft when they are about 18 years old fill in the upper gum line so that it can support permanent teeth and stabilize upper jaw. About 20% of children with cleft palate require further surgeries to improve speech. Once the permanent teeth grow, braces may be put to straighten the teeth.

13. NURSING MANAGEMENT CARE OF THE BABY AT BIRTH Expressed breast milk or artificial feeding can also be given with long handled spoon or dropper or soft nipple with a large hole. Breast feeding is possible with the use of palatal prosthesis Explain to the parents about the risk of aspiration due to cleft palate. So they must be instructed to feed the baby in upright position. Small bolus should be given from the corner of the mouth. Give the baby sufficient time to swallow. Burp the baby in between the feeds and after feeding

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15. CARE OF THE BABY BEFORE SURGERY Consent must be taken prior to surgery All the investigation reports must be entered in patient’s file The baby must be kept NPO, at least 6 hours prior to surgery CARE OF THE BABY AFTER SURGERY Monitor the vital signs Observe for any bleeding from the site of surgery Turn the baby’s face to one side, for drainage of secretions and preventing aspiration Administer analgesic to minimize pain

16. Cont……. Surgical site is to be protected from any injury, by taking the following measures: Position the baby on back or side and arm or elbow restraints are applied to prevent him/her from touching the suture site An arched metallic device known as ‘Logan’s bow’ must be placed over the upper lip and taped on the infant’s cheeks to prevent tension at the suture line. Prevent infection at the site, by cleaning the operated area gently using aseptic techniques, after each feeding Do not allow the baby to put any object in the mouth, as this may injure the surgical repair