1. Cleft Palate

2. CASE: Cleft Palate S.M.P. BW 3140 g
Full term via stat cesarean section due to NRFHRP
28 year old G1P1 (0101)
39 1/7 weeks AOG, MT 38 AGA
Apgar 9, 9
BW 3140 g
BL 51 cm
HC 34 cm
CC 34 cm
AC 30 cm
Aileen Elbo Dailo
November 19, 2013
Under Dra. M. Saludares
Normal CAS (August 28, 2013) and 2d echo
Homemaker, college graduate, no vices
HPI: asymptomatic

3. Personal/Social History OB History: Feeding history
Maternal History:
UTI- 1st trimester, treated with cefuroxime
Past Medical History:
(+) asymptomatic MVP
Family History:
Diabetes, Hypertension, Heart disease, Stroke
Personal/Social History
Unremarkable
OB History:
G1 – present pregnancy
Feeding history
Mixed feeding, expressed breastmilk+milk formula

4. Physical Findings Thinly meconium-stained amniotic fluid
Flat fontanels
No molding
Cleft palate
(-) alar flaring
Good air entry, no retractions
HR 150bpm, Good cardiac activity,
Soft abdomen
Grossly female genitalia
Full pulses

5. Diagnosis
Live Term Baby Girl
Cleft palate

6. PLAN NPO ENT Referral Therapeutics:
Obturator fitting c/o pedia dentist
OGT feedings
Feeding plate
Breast feed as tolerated
Strict aspiration precautions

7. Course in the NICU Subjective Day 1 of life
Tolerates milk formula (10-15 ml every 2 hours)
Active
No vomiting or regurgitation
5 urine output
3 meconium passages
Objective
T – 36.8 C HR 146 bpm RR 49 cpm
Weight 3020 g
(+) cleft palate
OGT at level 17
Good air entry
Good cardiac tone, no murmurs
Soft abdomen
Full pulses
Assessment
Term baby girl
Cleft Palate, Incomplete
Plan
Continue feedings
Referral to pedia dentist

8. Course in the NICU Subjective Day 2 of life
Tolerates milk formula (15-20 ml every 2 hours)
Active
No vomiting or regurgitation
5 urine output
6 meconium passages
Objective
T – 36.9 C HR 145 bpm RR 49 cpm
(+) cleft palate
Good air entry
Good cardiac tone, no murmurs
Soft abdomen
Full pulses
Assessment
Term baby girl
Cleft Palate, Incomplete
Plan
Pedia dentist: No need for obturator plate at this time
Referral to maxillofacial surgeon
For obturator fitting as outpatient pending surgical intervention
Adivsed proper feeding position with dropper or soft baby bottle with nipple. BF is encouraged
FF-up as soon as 1st tooth erupts

10. Cleft Palate Failure of the palatal shelves to fuse
Cleft palate: 1 in 2500 (Caucasians)
Cleft lip+/- cleft palate: 1 in 750
Cleft palate: Females > Males
Cleft lip: Males > Females
Syndromes associated w/ Cleft Lip +/- cleft palate : >200
Ethnic factors (Cleft lip +/- cleft palate)
Native Americans (1 in 230 to 1,000)
Asians (1 in 400 to 850)
African Americans (1 in 1,300 to 5,000)
Incidence of associated congenital malformations and of impairment in development is increased: Cleft palate alone > cleft lip
Most common in Native Americans (1 in 230 to 1,000) and Asians (1 in 400 to 850)
least common in African Americans (1 in 1,300 to 5,000)
Samanich, J. Cleft Palate . Pediatrics in Review 2009;30;230

11. Clefting Defects between the 6th and 9th weeks AOG
primary palate begins to form at about 35 days
complete lip development by the 6th week
palatal fusion follows
Cleft lip: interruption or hypoplasia of the mesenchymal layer  failure of fusion of the medial nasal process, maxillary process, and lateral nasal process (unilateral or bilateral)
Cleft palate: palatal shelves fail to fuse
Multifactorial traits:
Genetic: mutations in single genes (TBX22, IRF6, MSX1); Part of chromosomal aneuploidy or deletion syndromes (trisomy 13, velocardiofacial syndrome), and others result from.
environmental factors: teratogens (anticonvulsants)
Most common in Native Americans (1 in 230 to 1,000) and Asians (1 in 400 to 850)
least common in African Americans (1 in 1,300 to 5,000)
Samanich, J. Cleft Palate . Pediatrics in Review 2009;30;230

12. Cleft Palate
Occurs in the midline and might involve only the uvula or can extend into or through the soft and hard palates to the incisive foramen
When associated with cleft lip: involve midline of the soft palate and extend into the hard palate on one or both sides, exposing one or both of the nasal cavities as a unilateral or bilateral cleft palate
Can also have a submucosal cleft indicated by a bifid uvula, partial separation of muscle with intact mucosa, or palpable notch at the posterior of the palate
Kliegman et al Nelson’s Textbook of Pediatrics. 19th Edition

13. Pierre Robin sequence (PRS)
micrognathia (small mandible)
retropositioned tongue
U-shaped cleft palate
failure of the mandible to grow properly  positioning of the tongue in the back of the pharynx  blocks the ability of the palatal shelves to fuse properly
severe respiratory distress: mortality rate as high as 30%
careful monitoring: first 1 to 4 weeks
over time, the lower jaw generally “catches up” in growth vs. surgical intervention (jaw expansion)
isolated birth defect, but may be part of syndromes such as trisomy 18 or Stickler syndrome
An important type of clefting defect to recognize in the neonatal period is Pierre Robin sequence (PRS), which includes
micrognathia (small mandible), a retropositioned tongue, and a U-shaped
cleft palate.
The initial embryologic event is believed to be failure of the mandible to grow properly, leading to
positioning of the tongue in the back of the pharynx, which blocks the ability of the palatal shelves to fuse properly.
Affected children often experience severe respiratory distress, leading to a mortality rate as high as 30% and
requiring interventions ranging from prone positioning to allow the tongue to fall forward out of the airway to
intubation and tracheostomy. Children who have PRS require careful monitoring, particularly in the first 1 to 4 weeks
after birth. Over time, the lower jaw generally “catches up” in growth, but sometimes surgical intervention is required
for jaw expansion. PRS generally occurs as an isolated birth defect, but may be part of syndromes such as trisomy 18 or Stickler syndrome.
Samanich, J. Cleft Palate . Pediatrics in Review 2009;30;230

14. Trisomy 18 Edward’s Syndrome
second most common autosomal trisomy after trisomy 21
severe psychomotor and growth retardation, microcephaly, microphthalmia, malformed ears, micrognathia or retrognathia, microstomia, distinctively clenched fingers, and other congenital malformations

15. Stickler Syndrome
distinctive facial appearance, eye abnormalities, hearing loss, and joint problems
somewhat flattened facial appearance
underdeveloped bones in the middle of the face, including the cheekbones and the bridge of the nose
High myopia, glaucoma, cataracts, retinal detachment
Hearing loss
Loose or hypermobile joints, arthritis, scoliosis, khyphosis, platyspondyly

16. Velocardiofacial Syndrome
structural or functional palatal abnormalities, cardiac defects, unique facial characteristics, hypernasal speech, hypotonia, and defective thymic development
DiGeorge Syndrome (10%)
at least 2 of the following features:
Conotruncal cardiac anomaly
Hypoparathyroidism, hypocalcemia
Thymic aplasia, immune deficiency
Velo: cleft palate
Cardio: interrupted aortic arch type B (50%),truncus arteriosus (34.5%) and tetralogy of Fallot (16%). Other cardiac defects include pulmonary atresia with ventricular septal defect
Facial:

17. Pediatrics in Review 1988;9;331 Lorraine Suslak and Franklin Desposito
Infants with Cleft Lip/Cleft Palate

18. Cleft Palate: Treatment
Immediate problem: Feeding
Difficulty creating sufficient suction in the mouth to complete a feeding without tiring
Soft artificial (cross-cut) nipples with large openings, a squeezable bottle
Plastic obturator
Small, frequent feedings, not longer than 30mins
Burped 2-3x during a feeding: bottle positioned as upright as possible to avoid air in the nipple, or fed with an angled bottle
Timing of surgical correction is individualized
Width of the cleft
Adequacy of the existing palatal segment
Morphology of the surrounding areas
Neuromuscular function of the soft palate and pharyngeal walls
Complete rehabilitation can require years of special tx by a team consisting of a pedia, plastic surgeon, ent, oral and maxillofacial surgeon, pedia dentist, prosthodontist, orthodontist, speech therapist, geneticist, medical social worker, psychologist, and public health nurse
Parents have the added concern that the baby must gain weight before the cleft can be repaired
Infants who have a CP have a connection between the mouth and the nose and, therefore, have difficulty creating
sufficient suction in the mouth to complete a feeding without tiring. Often they are unable to obtain enough
nutrition by breastfeeding alone.
Mothers should be encouraged to try breastfeeding, but if weight gain is poor,supplementation with a bottle, ideally
using pumped human milk, will be needed.
SWALLOW MORE AIR: should be burped two to three times during a feeding,
with the bottle positioned as upright as possible to avoid air in the nipple, or fed with an
angled bottle.
be cautioned not to stop for too long in the middle of the feeding to burp the baby or the infant may lose interest in
completing the feeding
squeezable bottles, which allow them to provide the force for giving a mouthful of milk to the baby, rather
than having the baby suck to provide the force
3 months of age when the infant has shown satisfactory weight gain and is free of any oral, respiratory, or systemic infection.
Modified Millard rotation-advancement technique: a staggerred suture line minimizws notching of the lip and retraction of scar tissue

19. Cleft Palate: Treatment
Cleft lip: “rule of 10s”– 10lbs, 10 weeks old, and hgb of 10.0 g/dL
Goals of surgery:
Union of the cleft segments
Intelligible and pleasant speech
Reduction of nasal regurgitation
Avoidance of injury to the growing maxilla
Cleft palate: Usually by 1 year of age (speech development)
Furlow double-opposing Z-plasty (most common)
may need revisions as they grow older
When delayed beyond 3rd year: a contoured speech bulb can be attached to the posterior of the maxillary denture
Cleft palate: usually crosses the alveolar ridge and interferes with teeth formation in the anterior maxillary region
May be displaced, malformed, or missing (replaced by prosthetics)
When delayed beyond 3rd year: a contoured speech bulb can be attached to the posterior of the maxillary denture
So that contraction of the pharyngeal and velopharyngeal muscles can bring tissues into contact with the bulb to accomplish occlusion of the nasopharynx and help the child develop intelligible speech.
Kliegman et al Nelson’s Textbook of Pediatrics. 19th Edition
Samanich, J. Cleft Palate . Pediatrics in Review 2009;30;230

20. Cleft Palate: Treatment
Postoperative management: gentle aspiration of nasopharynx (minimizes atelectasis or pneumothorax which are common complications)
Maintenance of clean suture line and avoidance of tension on the sutures
Bottle-fed with arms restrained and with elbow cuffs
Fluid or semi-fluid diet for 3 wks
Hands, toys, and other foreign bodies are kept away from the surgical site
Kliegman et al Nelson’s Textbook of Pediatrics. 19th Edition

21. Cleft Palate: Sequelae
Recurent otitis media and subsequent hearing loss
Displacement of maxillary arches and teeth malposition
Misarticulations and velopharyngeal dysfunction (10-20% after repair)
Emission of air from the nose
Hypernasal quality
Compensatory misarticulations (glottal stops)
Middle ear infection: anomalies of the insertion of the tensor and levator veli palatini muscles into the eustachian tube, affected children are unable to open and ventilate the middle ear adequately
Speech defect: inadequacy of palatal and pharyngeal muscles
After CP repair, about 10% to 20% of patients experience velopharyngeal insufficiency, a phenomenon in which
the soft palate cannot form a tight closure with the pharynx, causing air to escape through the nose rather than
being directed through the mouth during speech.
This effect manifests as audible nasal air escape and as hypernasal resonance during speech, particularly
when making the s, z, sh, f, p, and b sounds. Affected children may develop maladaptive compensatory articulation
errors for these sounds, making their speech more difficult to understand.
Children who have CP also may have speech problems caused by dental
malocclusion from the cleft or from fistula formation after surgery.
Fistulas can develop between the oral and nasal cavities, leading to air escape during
speech and to regurgitation of food or liquids through the nose.
Surgical correction of velopharyngeal insufficiency, fistula, or other structural anomalies
generally is necessary before speech therapy, an integral part of treatment for the child who has CP, can be successful.
Kliegman et al Nelson’s Textbook of Pediatrics. 19th Edition
Samanich, J. Cleft Palate . Pediatrics in Review 2009;30;230