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Cleft Palate

Cleft Palate. CASE: Cleft Palate. BW 3140 g BL 51 cm HC 34 cm CC 34 cm AC 30 cm. 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. Maternal History: UTI- 1 st trimester, treated with cefuroxime

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Cleft Palate

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  1. Cleft Palate

  2. CASE: Cleft Palate • BW 3140 g • BL 51 cm • HC 34 cm • CC 34 cm • AC 30 cm • 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

  3. 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 pediadentist • OGT feedings • Feeding plate • Breast feed as tolerated

  7. Course in the NICU

  8. Course in the NICU

  9. 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 . Pediatricsin Review 2009;30;230

  10. 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 . Pediatricsin Review 2009;30;230

  11. 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. 19thEdition

  12. 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 . Pediatricsin Review 2009;30;230

  13. 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

  14. 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

  15. Velocardiofacial Syndrome • structural or functional palatal abnormalities, cardiac defects, unique facial characteristics, hypernasal speech, hypotonia, and defective thymicdevelopment • DiGeorge Syndrome (10%) • at least 2 of the following features: • Conotruncal cardiac anomaly • Hypoparathyroidism, hypocalcemia • Thymic aplasia, immune deficiency

  16. 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

  17. 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) • Furlowdouble-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. 19thEdition Samanich, J. Cleft Palate . Pediatricsin Review 2009;30;230

  18. 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. 19thEdition

  19. Cleft Palate: Sequelae • Recurentotitis 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. 19thEdition Samanich, J. Cleft Palate . Pediatricsin Review 2009;30;230

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