Cleft lip and palate

it cover all the important headings

1. CLEFT LIP AND PALATE Presented by – Ms. JYOTI DWIVEDI M.SC FINAL YEAR STUDENT

2. Cleft lip and cleft palate refer to facial malformations that evolve during embryonic development resulting in nonunion of the bones and tissues of the lip and palate. • Cleft lip and cleft palate may occur independently or together. • There may be a combination of defects and degrees of involvement. • Facial defects are noticeable at birth. Cleft lip ( cheiloschisis ) and cleft palate ( palatoschisis ), which can also occur together as cleft lip and palate, are variations of a type of clefting congenital deformity  caused by abnormal facial development during  gestation . A  cleft  is a fissure or opening—a gap. It is the non-fusion of the body's natural structures that form before birth. Approximately 1 in 700 children born have a cleft lip or a cleft palate or both. In decades past, the condition was sometimes referred to as harelip, based on the similarity to the cleft in the lip of a  hare , but that term is now generally considered to be offensive. Introduction

3. Approximately 1 in 700 children born have a cleft lip or a cleft palate or both. In decades past, the condition was sometimes referred to as harelip, based on the similarity to the cleft in the lip of a  hare , but that term is now generally considered to be offensive. Cont..

4. Cleft lip is a birth defect that occurs when the tissues of the upper jaw and nose do not join as expected during fetal development, resulting in a split (cleft) in the lip. Cleft palate  is a condition in which the two plates of the  skull  that form the  hard palate  (roof of the mouth) are not completely joined. The  soft palate  is in these cases cleft as well. Definition

5. Cleft lip is classified according to its location and severity: Unilateral -  (on one side of the lip). The unilateral cleft may cause the nostril on that side to be tilted and lower than the other nostril. Bilateral -  (on two sides of the lip). The bilateral cleft may cause the nose to be broader and shorter than normal. Complete -  extends to the nose. Incomplete - does not extend to the nose. A cleft palate is first categorized according to whether it affects the hard palate, the soft palate, or both. The  hard palate  is the front part of the roof of the mouth. The  soft palate  is the back part of the roof of the mouth. In cleft palate , the roof of the mouth (Palate) has an opening (cleft)that may go through to the nasal cavity . Types of cleft lip AND CLEFT PALATE

6. Combination terms Complete classification of a cleft palate combines all of the appropriate terms. For example: A cleft of the hard palate with an incomplete unilateral cleft lip is a cleft in the front of the mouth and a cleft in one side of the upper lip that doesn't extend to the nose. A cleft of both the hard and soft palate with bilateral complete cleft lip describes a cleft that extends from the soft palate to the hard palate, and includes the alveolar ridge. Both sides of the lip have clefts extending to the nose. Cont..

7. There are many causes of cleft lip and palate. Problems with genes passed down from one or both parents, drugs, viruses, or other toxins can all cause these birth defects. Cleft lip and palate may occur along with other syndromes or birth defects. A cleft lip and palate can: Affect the appearance of the face Lead to problems with feeding and speech Lead to ear infections Causes

8. Combination of genetic and environmental factors; cleft palate is found mostly in girls and cleft lip alone or together with cleft palate is found mostly in boys family history of cleft lip or palate and other birth defects. Anti- convulsant drugs. Maternal tobacco use. Genetic predisposition. Maternal alcohol consumption Folic acid deficiency. Maternal smoking. Risk factors

9. • Incompletely formed lip • Opening in roof of the mouth can be palpated • Unilateral or bilateral cleft lip • With cleft palate, milk or formula escapes through the nose • Abdominal distention due to swallowing air • Slow weight gain CLINICAL MANIFESTATION

10. Traditionally , the diagnosis is made at the time of birth by physical examination. Recent advances in  prenatal diagnosis  have allowed  obstetricians  to diagnose facial clefts  in utero . Visual assessment of defects at birth and by palpation. ultrasound to identify cleft lip or cleft palate in utero. MRI to detect the extent of the abnormality; evaluation of the infant’s ability to suck, swallow and breathe. A genetic evaluation to determine reoccurrence. Diagnostic evaluation

11. CLEFT LIP - Within the first 2–3 months after birth,  surgery  is performed to close the cleft lip. While surgery to repair a cleft lip can be performed soon after birth, often the preferred age is at approximately 10 weeks of age, following the "rule of 10s" coined by surgeons Wilhelmmesen and Musgrave in 1969 (the child is at least 10 weeks of age; weighs at least 10 pounds, and has at least 10g hemoglobin). If the cleft is bilateral and extensive, two surgeries may be required to close the cleft, one side first, and the second side a few weeks later. The most common procedure to repair a cleft lip is the Millard procedure pioneered by  Ralph Millard . Millard performed the first procedure at a  Mobile Army Surgical Hospital  (MASH) unit in Korea. Management

12. Often an incomplete cleft lip requires the same surgery as complete cleft. This is done for two reasons. Firstly the group of  muscles  required to purse the lips run through the upper lip. In order to restore the complete group a full incision must be made. Secondly, to create a less obvious scar the surgeon tries to line up the scar with the natural lines in the upper lip (such as the edges of the  philtrum ) and tuck away stitches as far up the nose as possible. Incomplete cleft gives the surgeon more tissue to work with, creating a more supple and natural-looking upper lip.

13. Millard procedure

14. A blue line indicate incision. Movement of the flaps; flap A is moved between B and C. C is rotated slightly while B is pushed down.   Post-operation, the lip is swollen from surgery and will get a more natural look within a couple of weeks. Cont..

15. Often a cleft palate is temporarily covered by a  palatal obturator  (a prosthetic device made to fit the roof of the mouth covering the gap). Cleft palate can also be corrected by  surgery , usually performed between 6 and 12 months. Approximately 20–25% only require one palatal surgery to achieve a competent velopharyngeal valve capable of producing normal, non- hypernasal speech . However, combinations of surgical methods and repeated surgeries are often necessary as the child grows. One of the new innovations of cleft lip and cleft palate repair is the  Latham appliance . The Latham is surgically inserted by use of pins during the child's 4th or 5th month. After it is in place, the doctor, or parents, turn a screw daily to bring the cleft together to assist with future lip and/or palate repair. CLEFT PALATE

16. If the cleft extends into the maxillary alveolar ridge, the gap is usually corrected by filling the gap with bone tissue. The bone tissue can be acquired from the patients own chin, rib or hip. Lantham appliance when the infant is without any infections (respiratory, oral, or systemic), surgical treatments begin early and continue into adolescence; scheduling depends on the severity of the cleft.Chiloplasty used for correction of a cleft lip the involvement of a multidisciplinary team is needed: pediatrician, plastic surgeon, orthodontist, audiologist, speech therapist, prosthodontist, and psychiatrist. Speech and hearing A  tympanostomy tube  is often inserted into the  eardrum  to aerate the  middle ear . This is often beneficial for the hearing ability of the child. Cont..

17. Children with cleft palate typically have a variety of speech problems. Some speech problems result directly from anatomical differences such as velopharyngeal inadequacy . Velopharyngeal inadequacy refers to the inability of the soft palate to close the opening from the throat to the nasal cavity, which is necessary for many speech sounds, such as /p/, /b/, /t/, /d/, /s/, /z/, etc. This type of errors typically resolve after palate repair. Cont..

18. Speech-language pathology can be very beneficial to help resolve speech problems associated with cleft palate. In addition, research has indicated that children who receive early language intervention are less likely to develop compensatory error patterns later. Hearing loss Hearing impairment is particularly prevalent in children with cleft palate. The tensor muscle fibres that open the  eustachian tubes  lack an anchor to function effectively. In this situation, when the air in the middle ear is absorbed by the mucous membrane, the negative pressure is not compensated, which results in the secretion of fluid into the middle ear space from the mucous membrane. Children with this problem typically have a conductive hearing loss primarily caused by this middle ear effusion.

19. Hearing loss Hearing impairment is particularly prevalent in children with cleft palate. The tensor muscle fibres that open the  eustachian tubes  lack an anchor to function effectively. In this situation, when the air in the middle ear is absorbed by the mucous membrane, the negative pressure is not compensated, which results in the secretion of fluid into the middle ear space from the mucous membrane. Children with this problem typically have a conductive hearing loss primarily caused by this middle ear effusion.

20. Six-month-old girl before going into surgery to have her unilateral complete cleft lip repaired. The same girl, 1 month after the surgery. The same girl, age 8, the scar almost gone. Cont..

21. Assessment – Assess respiratory status. Monitor vital signs. Check weight periodically to evaluate adequate nutritional intake. Clean suture line to prevent infection. Use a gentle restraint to prevent the infant from placing hands on its mouth. Position infant so that rubbing of lip is prevented. Use wrist restraints after cleft-lip repair and elbow restraints following cleft-palate repair to prevent the child from putting anything into the mouth. NURSING CARE PLAN

22. intervention rationale Assess respiratory status and monitor vital signs atleast every 2 hours. Position on side after feedings. Feed slowly and use adaptive equipment as needed. Burp frequently. Position upright for feedings. Keep suction equipment and bulb syringe at bedside. Allows for early identification of problems.   Prevent aspiration of feedings. Facilitates intake while minimizing risk of inspiration. Helps to prevent regurgitation and aspiration. Minimizes passage of feedings through cleft. Suctioning may be necessary to remove milk or mucus. Pre-operative : NURSING DIAGNOSIS - High risk for aspiration(breast milk , formula ,or mucus) related to anatomic defect. GOAL- Infant has no episodes of gagging or aspiration. Expected outcome – Infants exhibits no signs of respiratory distress.

23. COMPLICATIONS Feeding problems. Ear disease. Speech problem Problem in socialization. Prognosis A child born with a cleft lip with or without cleft palate will require a multidisciplinary approach to management throughout his or her development. In the absence of an associated syndromic condition; however, children with cleft lip and/or palate can have excellent esthetic and functional outcomes. Cont..

24. HEALTH EDUCATION Parents can be directed to various internet resources for further information and advice Parents should be advised on appropriate feeding methods. Feeding the infant in an upright position avoids nasal regurgitation. Bottle feeding can be optimized using specialized fissured nipples and bottles controlling the flow rate of pumped breast milk or formula.

25. Soft-bodied bottles are squeezed in synchronization with infant sucking to reduce the effort of feeding and maximize the amount of feed entering the mouth. The infant should be burped during pauses in feeding. Feeding should not be continued for more than 30 minutes, to avoid the infant becoming tired. Parents should be warned that the neonate will lose weight (up to 10% of birth weight) after birth, but reassured that the birth weight will be regained within 2 postnatal weeks, and increase by at least 1 ounce per day thereafter. Infants should have their weight monitored at weekly intervals until it has stabilized. Parents should be provided with instruction on appropriate postoperative care to protect the wound and promote healing, with maintenance of good wound care and nutrition as well as careful bathing and feeding.

26. Prevention Although the etiologies of cleft lip and palate are multifactorial, several recommendations are suggested- Avoidance of teratogenic medications during pregnancy is mandatory. seizure medications should be adjusted by a neurologist before conception, to prevent teratogen exposure. Prenatal multivitamins (including folic acid supplementation), Antismoking counseling. Advice on the importance of minimizing perinatal alcohol consumption are strongly encouraged.   Cont..

27. Clefts of the lip and palate are generally divided into two groups, isolated cleft palate and cleft lip with or without cleft palate, representing a heterogeneous group of disorders affecting the lips and oral cavity. These defects arise in about 1.7 per 1000 liveborn babies, with ethnic and geographic variation Typically, children with these disorders need multidisciplinary care from birth to adulthood and have higher morbidity and mortality throughout life than do unaffected individuals. . Prevention is the ultimate objective for clefts of the lip and palate, and a prerequisite of this aim is to elucidate causes of the disorders.   CONCLUSION

28. BIBLIOGRAPHY http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002046/ http://www.nlm.nih.gov/medlineplus/ency/article/001051.htm https://online.epocrates.com/noFrame/showPage.do?method=diseases&MonographId=675&ActiveSectionId=21 http://www.healthlinkbc.ca/kb/content/special/aa112054.html Peadiatric nursing.caring for children.Jane ball and Ruth binder.Page no 486 -490. Peadiatric nursing care plan.Jaypee.Page no 465. Essential peadiatric nursing.Piyush gupta.2 nd edition.Page no 466 -477. Cont..