Cleft Palate

• S.M.P.• 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

CASE: Cleft Palate

• 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

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

Diagnosis

• Live Term Baby Girl• Cleft palate

• NPO• ENT Referral• Therapeutics:

– Obturator fitting c/o pedia dentist– OGT feedings– Feeding plate– Breast feed as tolerated

PLAN

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

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

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

Samanich, J. Cleft Palate . Pediatrics in Review 2009;30;230

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)

Samanich, J. Cleft Palate . Pediatrics in Review 2009;30;230

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. 2011. Nelson’s Textbook of Pediatrics. 19th Edition

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

Samanich, J. Cleft Palate . Pediatrics in Review 2009;30;230

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

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

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

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

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)

Kliegman et al. 2011. Nelson’s Textbook of Pediatrics. 19th EditionSamanich, J. Cleft Palate . Pediatrics in Review 2009;30;230

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. 2011. Nelson’s Textbook of Pediatrics. 19th Edition

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)

Kliegman et al. 2011. Nelson’s Textbook of Pediatrics. 19th EditionSamanich, J. Cleft Palate . Pediatrics in Review 2009;30;230