Development Disorders

1. DEVELOPMENTAL DISORDERS Cerebral Palsy Autism Cleft Palate/Lip

2. Presented by, Forum. Thakker Nisha . Salian

3. CEREBRAL PALSY Cerebral palsy (CP) is an umbrella term for a group of disorders affecting body movement, balance, and posture. Cerebral refers to the brain Palsy refers to a physical disorder Infants are usually slow to reach developmental milestones such as rolling over, sitting, crawling, and walking.

4. BRAIN STRUCTURE

5. Types of Cerebral Palsy Classification of CP is related to the type of movement disorder and/or by the number of limbs involved

6. The location of the brain injury will determine how movement is affected.

7. Causes of Cerebral Palsy D amage to certain parts of the developing brain. I njury during child birth, if the baby got stuck in the birth canal with no oxygen supply. In premature infants it can be caused by bleeding in the brain, brain infections such as encephalitis, meningitis, and herpes simplex. Head injuries. Sever jaundice. Medical negligence Many times it is unknown

8. Symptoms Delayed milestones Abnormal muscle tone Abnormal movements Skeletal deformities Joint contractures Mental retardation Seizures Speech problems Swallowing problems Hearing loss Vision problems Dental problems Bowel and/or bladder control problems

9. Terminologies Scoliosis— Scoliosis is an abnormal curving of the spine. The spine might look like the letter “C” or “S.” Malrotation— Intestinal malrotation is twisting of the intestines (or bowel) caused by abnormal development while a fetus is in utero, and can cause obstruction. Intestinal Volvulus-- Intestinal volvulus is defined as a complete twisting of a loop of intestine around its mesenteric attachment site. Sialorrhea— An excessive flow of saliva.

10. Other Complications GI issues The underlying neurologic impairment in cerebral palsy can affect the gastrointestinal system, most notably oral-motor function and motility (especially colonic, which typically results in constipation ). THE BRAIN-GUT AXIS

11. Dysphagia /Oral Motor Dysfunction Due to history of feeding difficulties, extended feeding times, malnutrition, and/or a history of aspiration pneumonia Medications used to reduce muscle tone can also increase dysphagia risk Dysmobility Present in several forms: Dysphagia , Gastroesophageal Reflux Disease (GERD), Delayed Gastric Emptying and Dumping Syndrome Children with severe gastroesophageal reflux that is unresponsive to medical therapy may require a surgical antireflux procedure (ARP)

12. Constipation Dysmotility , hypotonia, medications, and nonambulation contribute to constipation. Ensuring adequate fluid intake prior to increasing fiber intake can help prevent additional problems with constipation. Pulmonary aspiration The result of swallowing dysfunction with aspiration of saliva and/or aspiration of gastric contents. Spinal Abnormalities Scoliosis progression can result in diminished gastric capacity, GERD, difficulty in positioning for feedings, and changes in motility that can cause early satiety, nausea, and vomiting.

13. Bone Health Osteopenia and osteoporosis are frequently encountered due to Reduced ambulation and weight-bearing activity, Malnutrition, Limited sun exposure and The use of anticonvulsant medication, which alters vitamin D metabolism Osteoporosis should be prevented with an adequate intake of Dietary Calcium, Phosphorus and Vitamin D.

14. Nutritional Assessment of Children with Cerebral Palsy Medical issues, such as wounds, illness, or surgery, are typically the primary focus in the acute care setting. The ultimate goal in nutritional intervention should be to optimize health, fitness, growth, and function in individuals with cerebral palsy In clinical practice, the Subjective Global Assessment technique is not a good assessment tool for most individuals with disabilities, as the body habitués is not considered in this assessment. A physical assessment is important to identify signs and symptoms of dehydration and malnutrition.

15. Anthropometric Measurements Physical examination: The physical examination should detect signs of malnutrition Triceps skinfold thickness and mid-arm circumference Head circumference lower leg length or upper arm length Scoliosis, increased or decreased muscle tone should be assessed Auscultation of the lungs may reveal signs of chronic aspiration. Abdominal examination and, if needed, a rectal examination, may reveal constipation.

16. Stature Measurement: Changes due to scoliosis, spasticity, contractures, and/or limb differences may even make the individual appear to “shrink” Knee height, Upper arm length, Recumbent length, or Tibial lengths are techniques used to estimate stature

17. Weight: Weight does not reflect the typical distribution of body fat and muscle. Fat stores are typically depleted and muscle stores are low Alterations in body composition should be considered when estimating energy needs

18. Clinical Assessment Nutritional history: The type (purees, liquids and solid food) and the amount of food. If the child is able to self-feed, the amount of spilling should be assessed. Signs of oromotor dysfunction Medical history: An assessment of gastroesophageal reflux symptoms such as emesis, regurgitation, pain or food refusal Chronic respiratory problems, recurrent pneumonia and respiratory symptoms suggestive of chronic aspirations Recurrent infections, decubitus ulcers and constipation A review of the child’s medication

19. Growth history: Birth weight, length and head circumference and all previous weight, length and head circumference measurements It helps determine whether there is a decrease in growth velocity .

20. Investigation: Extensive blood work is usually not necessary. A complete blood count may help detect iron deficiency. Serum albumin may reflect nutritional status, but is not very reliable in this population. Electrolytes are usually normal. Phosphorus, calcium, alkaline phosphatase and vitamin D levels may be measured in patients with suspected osteoporosis and may be combined with a bone density scan. A gastric emptying scan is useful in diagnosing delayed gastric emptying and possibly pulmonary aspiration of gastric content.

21. Nutritional Management Energy Krick method Kcal/day = (BMR × muscle tone factor × activity factor) + growth factor Muscle tone factor: 0.9 if decreased, 1.0 if normal, 1.1 if increased Activity factor: 1.15 if bedridden, 1.2 if dependant, 1.25 if crawling, 1.3 if ambulatory Growth factor: 5 kcal/g of desired weight gain   Height-based method 14.7 cal/cm in children without motor dysfunction 13.9 cal/cm in ambulatory patients with motor dysfunction 11.1 cal/cm in nonambulatory patients Resting energy expenditure-based method 1.1× measured resting energy expenditure The effect of medications on energy expenditure is important to consider

22. Protein Protein needs are estimated using the RDA and actual weight or appropriate weight for height . There are no guidelines for estimating protein needs of individuals with disabilities under stress such as surgery. Protein intake has been increased up to 1.5–2 g/kg/day in clinical practice for presurgical/postsurgical planning and wound healing with normal renal status.

23. Fluid Requirements Fluid loss through Sialorrhea or sweating. Actual body weight is used to estimate fluid needs using the Holliday-Segar equation Weight Calculation 1-10 Kg 100 ml/kg 10-20 Kg 1000 ml + 50 ml/kg for each kg >20 Kg 1500 ml + 20 ml/kg for each kg

24. Vitamin D Shinchuk and Holick recommend supplementation if 25(OH)-D levels are less than 30 ng / mL The recent Institute of Medicine (IOM) report recommends supplementation only when 25(OH)-D levels are less than 20 ng /ml. 25(OH)-D levels should be checked every three months until levels are within normal range

25. Feeding and Feeding Regimen Improve oral intake Adequate positioning of the child during meals Food consistency may be adjusted Food caloric density may be increased Oral intake can be maintained as long as there is no risk of aspiration Enteral nutrition Before 12 months of age, an infant formula should be used Pediatric 1 kcal/ml formula is preferred. A 1.5 kcal/ml formula may be used with careful monitoring of hydration status. Feeding regimen: The choice of feeding regimen will be based on The child’s enteral access Activities, Caloric needs and Tolerance to feeds.

26. Conclusion Malnutrition should not be considered normal in CP children. Nutritional intervention should be provided by a multidisciplinary team of professionals to ensure adequate growth, improve quality of life and optimize functional status. Early nutritional intervention, appropriate support and continuing follow-up are necessary to ensure success.

27. Autism Spectrum Disorder (ASD) The term autism spectrum disorders (ASD) has been used to describe their variable presentation. Prevalence studies indicating that they are present in 6 per 1000 children. These disorders are characterized by three core deficits: Impaired communication, Impaired reciprocal social interaction and restricted, Repetitive and stereotyped patterns of behaviors or interests.

28. Pervasive Developmental Disorders (PDD) is a group of neuro-developmental disorders characterized by impairments in communication, reciprocal social interaction and restricted repetitive behaviors or interests. The term autism spectrum disorders (ASD) has been used to describe their variable presentation.

29. Listed in the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition ( DSM-IV ) and the International Classification of Diseases, Tenth Edition ( ICD-10 ); ASD is used to describe 3 of the following 5 PDD-- Autistic disorder, Asperger disorder, Pervasive Developmental Disorder-Not Otherwise Specified (PDD-NOS), Rett Syndrome and Childhood Disintegrative Disorder.

30. Autistic Disorder Qualitative abnormalities in social interactions, Markedly aberrant communication skills, restricted repetitive and stereotyped behaviors, and Moderate mental retardation with intelligence quotients (IQs) of approximately 35-50. Three fourths of autistic children function in the mentally retarded range.

31. Asperger’s Syndrome Persistent impairment in social interactions and by repetitive behavior patterns and restricted interests. No significant aberrations or delays occur in language development or in cognitive development. Generally evident in children older than age 3 years and occurs most often in males. Severe social impairment is associated with this condition.

32. Pervasive Developmental Disorder- Not Otherwise Specified (PDD-NOS) Describes a child aged 9 years with poor peer interactions, normal verbal abilities, and mild nonverbal disabilities The mild nonverbal disabilities make it difficult for the child to follow subtle social cues that most children easily interpret as anxiety, anger, or sadness.

33. Rett Syndrome Occurs almost exclusively in females. The specific mutation on the gene related to Rett syndrome ( methyl-CpG binding protein-2 [MECP2] ) was identified late in 1999. Initially have seemingly healthy development with low tone and subtle slowing of development. Early clinical feature is deceleration of head growth that begins when the individual is aged 2-4 months. Individuals who are less severely affected may tolerate or even prefer interpersonal contact, show affection to others, and suffer from learning disabilities and speech fragmentation related to breathing irregularity.

34. Childhood Disintegrative Disorder Also known as Heller syndrome. Characterized by a loss of previously acquired language and social skills and results in a persistent delay in these areas. Children develop normally through age 3 or 4. Social and emotional development also regresses, resulting in an impaired ability to relate with others. No single causative factor for childhood disintegrative disorder has been identified.

35. AUTISM Autism is a complex developmental disorder that has the following three defining core features: Problems with social interactions Impaired verbal and nonverbal communication A pattern of repetitive behavior with narrow, restricted interests The diagnosis of autism may not be made until a child reaches preschool or school age. The behavioral characteristics of autism are almost always evident by the time the child is aged 3 years

36. EPIDEMIOLOGY ASD occurs more often in boys than girls, with a 4:1 male-to-female ratio. The reported prevalence rates of autism and its related disorders have been increasing worldwide from approximately 4 per 10 000 to 6 per 1000 children. The possibility that the increase in the reported cases is a result of unidentified risk factor(s) cannot be ruled out

37. ETIOLOGY The exact cause of autism and the other ASDs is still not known. The etiologic theories have changed over the years. Obstetric complications Mothers who experienced diabetes, hypertension, or obesity during pregnancy are more likely to have children with autism spectrum disorders and other neuro-developmental disabilities.

38. An infectious basis The large number of children with autistic disorder born to women who were infected in the rubella epidemic. Also Cytomegalovirus infection is said to be associated with development of autism. Genetic factors Autism is both familial and heritable. The recurrence rate in siblings of an autistic child is 2% to 8% Monozygotic twins have a higher concordance rate than Dizygotic twins—90% and 10%, respectively. Fragile X syndrome is identified to be associated with Autism.

39. Environmental factors Prenatal infections with rubella and cytomegalovirus. The role of heavy metals in the etiology of autism is controversial. Exposures to toxins, chemicals, poisons, and other substances have been hypothesized to cause autism. Hypothesis The potential adverse effect of the measles, mumps, rubella (MMR) vaccine and Thimerosal , a mercury-based preservative used in some vaccines.

40. Diagnosis The severity of these impairments varies significantly among children with ASD. The impairments can be subtle and may not be detected before school age. Some of the widely used instruments for screening these high-risk children. The Checklist for Autism in Toddlers (CHAT), Modified-Checklist for Autism in Toddlers (M-CHAT), Childhood Autism Rating Scale (CARS )

41. Specific tools commonly used in the assessment include the Autism Diagnostic Interview-Revised (ADI-R) and The Autism Diagnostic Observational Schedule (ADOS). Currently, there are no laboratory or radiologic tests to diagnose ASD.

42. FEEDING ABNORMALITIES Restricted range of foods Unusual eating behaviors such as food cravings and pica. H igher instances of food selectivity by type Selectivity by type was defined as “eating a narrow range of food that was nutritionally inappropriate eating only a few different foods and often [refusing] to eat entire food groups

43. Two types of physiological issues that can directly or indirectly impact feeding skills and/or behavior: Sensory processing issues 2) Gastrointestinal (GI) issues Sensory modulation: Sensory modulation allows an individual to appropriately filter the multitude of sensory information that constantly bombards the nervous system. Dysfunction- hyperresponsivity , hyporesponsivity , and/or fluctuating responsivity resulting in atypical responses such as sensory seeking or sensory avoidance behaviors.

44. Sensory processing difficulties that both directly and indirectly impact eating processes :- abnormal responses to taste and smell heightened sensitivity to tactile input auditory filtering problems Gastrointestinal alterations : Ileal lymphoid nodular hyperplasia (LNH) , M ucosal abnormality L ow cho enzymes I ncreased exocrine secretions of pancreas I ntestinal mucosa is abnormally permeable I ncrease in immunoglobins levels

45. Pathways : Antigens in the diet Thimerosal -increased ionic conductance and permeability Elevated levels of VIP-impair gut motility, development and secretion Reduced secretin - the reduced blood levels of secretin could allow gastric HCl secretion to increase abnormally, elevate intestinal permeability

46. G.I. issues : GI disorders include gastroesophageal reflux disease (GERD) and constipation, diarrhea, or other symptoms resulting from food allergies. Difficulty expressing their discomfort and/or correctly identifying its source . Difficulty with self-monitoring during the meal may also affect the child’s ability to complete the meal This would be more likely if the child with ASD does not connect the internal feeling of hunger with the consumption of food . Children with ASD appear to eat based on external stimuli such as the time on a clock or the presence of food rather than on feelings of hunger

47. If a child’s appetite regulation is impaired or is unconnected to food consumption, it could force the child to use other methods to monitor food intake (e.g., visual appearance of the amount of food left on the plate; amount of time spent at the table, etc.); this, in turn, could lead not only to difficulty judging when mealtime is finished, but also to over- or undereating .

48. ALTERATION IN METABOLISM : Decreased sulphate levels( Sulfation is important for many reactions including detoxification, inactivation of catecholamines , synthesis of brain tissue, sulfation of mucin proteins which line the gastrointestinal tract . Increased oxidative stress-indicated by high levels ofGSSG Decreased NADPH, ATP(may be due to mitochondrial dysfunction) Decreased SAM (impaired methylation ) Primary amino acids: elevated glutamate- Overstimulation leads to excitotoxicity , creating oxidative stress, mitochondrial damage and may play a role in neurodegeneration

49. Decreased tryptophan may be due to decreased protein intake, and/or impaired digestion of protein into amino acid. Tryptophan is a precursor to the synthesis of serotonin, so decreased tryptophan is likely to impair serotonin synthesis ( neurogenesis and neurotransmission)

50. REMOVING CASEIN AND GLUTEN FROM DIET Implementation of a strict casein- and gluten-free (CFGF) diet leads to symptomatic improvement individual , improvement in social cognitive and communication skills. Mechanism: During digestion, pre- opioid type compounds from casein and gluten in the diet are activated because of an incomplete breakdown of proteins. These exorphins ( i.e.caseomorphins and gluteomorphins ) are then absorbed into the circulation where they exert an opioid - type action on the brain.

51. Transfer of peptides across the lumen of the gut is thought to occur due to the ‘leaky’ nature of the gastrointestinal tract . Caseomorphins and gliadomorphins are potent psychosis inducing factors. Elimination is a two step process: The first phase is removal of casein via removal of milk and other dairy products . Benefits seen in 2-3 days in children and 10-14 days in adults. Symptoms linked to casein intake include projectile vomiting; eczema, particularly behind the knees and in the crook of the elbow; white bumps under the skin; ear discharges and infections; constipation, cramps, and/or diarrhea; and respiratory disorders resembling asthma.

52. Gluten exclusion requires the removal of several common cereals from the diet, wheat, barley, rye, and oats. Elimination process usually takes a minimum of 3-4 weeks, and a trial period of three months is appropriate. Once the main sources of food intolerance – casein, gluten, and gliadin – are removed from the diet, other foods may emerge as sources of symptoms. Parents can keep food diaries, to associate the child’s consumption of a particular food with deterioration in behavior, sleep patterns, or performance.

53. VITAMINS: Vitamin B6 and Magnesium: Vitamin B6: Metabolic pathways of neurotransmitters, including serotonin, gammaamino - butyric acid (GABA), dopamine, epinephrine, and norepinephrine . Study: Each child received vitamin B6 at a dose of 2.5-25.1 mg/kg body weight/day (75-800 mg per day). Benefits seen from this trial, included better eye contact, less self-stimulatory behavior, more interest in surroundings, fewer tantrums, and better speech. Magnesium:Required for a wide range of enzyme-catalyzed metabolic pathways. An intake threshold for achieving benefit may be approximately 200 mg vitamin B6 (as pyridoxine) and 100 mg magnesium per day for the 70 kg individual.

54. Folic Acid: Folic acid is essential to numerous metabolic pathways. Favourable results on several non-fragile X autistic children by giving relatively large doses of folic acid (0.5- 0.7 mg/kg/day). Vitamin C : Vitamin C involved in a plethora of metabolic, antioxidant, and bio-synthetic pathways, and as a cofactor for certain enzymes necessary for neurotransmitter synthesis. In a double-blind trial for 30 weeks, vitamin C (8 g/70 kg body weight/day) improved total symptom severity and sensory motor scores .

55. Vitamin A: Vitamin A is especially important for cell growth and differentiation, especially in epithelial tissues of the gut, brain Core autism symptoms, such as language, eye contact, ability to socialize, and sleep patterns, were consistently improved . Zinc: Zinc is needed for the development and maintenance of the brain, adrenal glands, GI tract, and immune system. Serotonin synthesis relies on zinc-activated enzymes and for antioxidant enzyme activity and other proteins important for growth and homeostasis , cognition .

56. Lithium : low levels are seen,supplementation improved mood stabilization . ESSENTIAL FATTY ACIDS : Essential fatty acids (EFAs) function as homeostatic constituents of cell membranes, helping to relay signal information from outside the cell to the cell interior and are precursors to eicosanoids that influence other cells, similar to hormones. Essential fatty acids, particularly the omega-3s, are deficient in other neurodevelopmental disorders .

57. CARNITINE : is an amino acid indispensable for energy generation . Valproate , a drug prescribed for seizures, is known to deplete carnitine . Constipation and self-abuse decreased while mood improved. BIOPTERIN : Biopterin , in its reduced form (5,6,7,8- tetrahydrobiopterin , R-BH4), is a limiting factor for the biosyntheses of dopamine, epinephrine, and serotonin. Autistic children, particularly those six years or younger, can have relatively low R-BH4 in their cerebrospinal fluid (CSF) and abnormally high urine R-BH4, indicating increased loss from the body. Also , the enzyme ( dihydropteridine reductase ) that recycles biopterin into its biologically active reduced form, R-BH4, is lower in autism.

58. INOSITOL : is a precursor for the synthesis of phosphatidylinositol (PI), a phospholipid that is part of a complex cellular transmission pathway that facilitates serotonin receptor function .

59. CLEFT LIP /PALATE CLEFT LIP Craniofacial malformation An opening in the upper lip between the mouth and the nose... it can range from a slight notch in the coloured portion of the lip to complete separation in one or both sides of the lip extending up and into the nose.

60. CLEFT PALATE: “the roof of the mouth is not joined completely ranging from just an opening at the back of the soft palate to a nearly complete separation of the roof of the mouth (soft and hard palate). CLEFT LIP PALTE: Infants are born with a cleft lip and palate (i.e. cleft lip + palate). This type of cleft extends from the lip to the hard or soft palate

61. CLEFT LIP occurs when an epithelial bridge fails, Due to lack of mesodermal delivery and proliferation. CL usually occurs at the junction between the central and lateral parts of the upper lip on either side. The cleft may affect only the upper lip, or it may extend more deeply into the maxilla and the primary palate. (Cleft of the primary palate includes CL and cleft of the alveolus.) If the fusion of palatal shelves is impaired also, the CL is accompanied by CP, forming the CLP abnormality. In general, patients with clefts have a deficiency of tissue and not merely a displacement of normal tissue.

62. ETIOLOGY: Family History: Cleft lip more likely to be inherited than cleft palate Race: More common in Native American, Hispanic & Asian patients Sex: Males 2x as likely to have cleft lip; Females 2x as likely to have cleft palate Environmental factors: exposure of fetus to alcohol, cigarette smoke, or drugs Maternal Nutrition Deficiencies:especially lack of folate . DIAGNOSIS : USG, Genetic testing, physical examination

63. SUCKING PERFORMANCE AND FEEDING PROBLEMS Cleft of lip :Without adequate closure around the nipple, the infant may have problems producing a suck powerful enough to extract milk from the breast or bottle nipple Cleft of the lip and palate will usually result in an inability to form a complete seal, and negative air pressure cannot be generated .

64. EXTENSIVE FEEDING PROBLEMS Nasopharyngeal reflux, choking, prolonged feeding time, and slow or little weight gain, inadequate nutritional intake. Feeding is an immediate concern due to the delay in growth of children born with clefts. This can be a major concern for infants who will be undergoing surgery to correct their cleft A primary feeding concern associated with cleft palate is the formation of negative air pressure, necessary for adequate swallowing Without negative air pressure, a swallow cannot be properly triggered and aspiration or choking may occur.

65. Recurrent aspiration will lead to respiratory infections including pneumonia and even death. Signs and symptoms -inefficient or ineffective suck, and excessive air intake. This excessive air intake may result in choking and/or gagging. Infants witth clefting of the hard palate may limit the normal use of the tongue to compress the nipple. If the soft palate is not intact when it is elevated it does not create a barrier in which food and liquid cannot pass through the nasopharynx . Signs and symptoms associated with the inability to seal off the nasal cavity include: nasal reflux, and inefficient or ineffective suck

66. The impact of a cleft is not necessarily restricted to the oral cavity. There may be airway deficits due to a cleft palate Clinical signs or symptoms associated with an upper airway obstruction are: Inspiratory stridor Glossoptosis Micrognathia Clinical signs or symptoms related to neurological impairments coinciding with a cleft include: Incoordination of suck Swallow and respiratory sequence Hypotonicity and hypertonicity

67. DIFFERENT FEEDING TECHNIQUES ARTIFICIAL NIPPLES : Infants with isolated cleft palate may benefit most from being fed using crosscut nipples. Artificial nipples with large holes are recommended so that infants may passively receive a bolus of milk where a cleft palate prevents them from extracting it independently from the bottle

68. Advantage: Faster feeding times, less vomiting, satisfactory weight gain, and parental acceptance for infants. Disadvantage: The rapid flow may imperil the infant’s ability to synchronize sucking, swallowing, and breathing if milk is delivered directly to the pharynx.

69. COMPRESSIBLE BOTTLES: Compressible bottles allow the feeder to deliver milk to the infant who is unable to generate suction and extract fluid independently. Gentle squeezing of the plastic bottle forces formula from the tip of the nipple avoiding necessity of negative pressure created by sucking. Advantage: easier to use for the feeding of babies born with cleft condition the feeder is able to control the amount of milk expelled into the infant’s mouth. Disadvantage: No effect on child’s weight and growth

70. HABERMAN FEEDER : The Haberman feeder is specifically designed for cleft lip +/- palate use. Its elongated nipple can be compressed if the infant has difficulty in applying adequate negative pressure. Advantage: 1) it has flow lines on the nipple that assist in helping the infant achieve optimal flow from the nipple. 2) flow can be monitored without the necessity of squeezing the bottle. 3) Valve that prevents back flow, which reduces the excessive air buildup. The excessive air build up can cause uncomfortable gas and stomach problems as well as burping .

71. BREAST FEEDING AND PROSTHESES : Feeding obturators are passive devices designed to provide a normal contour to the cleft alveolus and hard palate. They separate the oral and nasal cavities and in doing so provide a surface to appose the nipple during suckling. Feeding device is inserted over the infant's hard palate, which allows him or her to compress the nipple easier because it provides a contact point and helps the infant to express milk .

72. Advantage: R educed choking, nasal discharge, and bottle feed duration and improved parental confidence in their sample of infants with cleft lip and palate who were prescribed feeding obturators . . It facilitates feeding, reduces nasal regurgitation and shortens the length of time required for feeding Disadvantage: A feeding appliance can be costly and needs to be replaced as the child grows to fit his or her mouth. Oral hygiene is also a concern because it is a plastic appliance, which can cause irritation to the palate.

73. However,Strong (Level I) evidence show that obturators do not facilitate feeding or weight gain in breastfed babies with a CLP, and that they do not improve the infant’s rate of bottle feeding. There was moderate evidence (Level II-2) obturators do not facilitate suction during bottle feeding.This is because obturators do not facilitate complete closure of the soft palate against the walls of the throat during feeding.

74. Position , pacing and other care Infants should be in an upright position with good head neck and trunk support . Feeding times should be limited so that infants do not experience hunger and unsatisfactory feeding. Burping the infants after feeding Oral care after feeding- one can use clean water, or water with hydrogen peroxide

75. TRANSITION TO SOLID FOODS Spoon feeding for infants with a cleft lip +/- palate should begin at approximately six months of age. Strained, thin pureed, foods should not be a problem for infants with clefts Avoid thickened foods to ensure that these consistencies do not get lodged in the cleft area .

76. PREOPERATIVE CARE : Assess fluid and calorie intake daily. Assess weight daily (same scale,same time, with infant completely undressed). Observe for any respiratory impairment. Provide 100–150 cal/kg/day and 100–130 mL /kg/day of feedings and fluid. Facilitate breastfeeding. Hold the infant in a semisitting position. Give the mother information on breastfeeding the infant with a cleft lip and/or palate such as plugging the cleft lip and eliciting a let-down reflex before nursing.

77. BENEFITS OF BREAST MILK IN INFANTS WITH CL/CLP Feeding with breastmilk protects against otitis media in infants with a CP Babies are more prone to otitis media than the general population due to the abnormal soft palate musculature. Moderate to weak evidence that breastmilk can promote intellectual development and school outcomes in babies with clefts. Antibacterial agents in breastmilk promote postsurgical healing and reduce irritation of mucosa. Breastfeeding facilitates the development of oral facial musculature speech,bonding , and pacifying infants postsurgery

78. POST-OPERATIVE CARE : CL repair ( cheiloplasty ) is generally carried out within a few months of birth and CP repair ( palatoplasty ) takes place between 6 and 12 months of age. Maintain intravenous infusion as ordered. Begin with clear liquids, then give half-strength formula or breast milk as ordered. Give high-calorie soft foods after cleft palate repair (Levels I and II-2) It is safe to commence/recommence breastfeeding immediately following CL repair and moderate evidence (Level II-2) for initiating breastfeeding 1 day after CP repair. There is strong evidence (Level I) that breastfeeding immediately following surgery is more effective for weight gain, with lower hospital costs, than spoon feeding.

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