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Cleft lip and cleft palate

Last updated: March 26, 2021

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Cleft lip and cleft palate (CLP) are the most common congenital orofacial deformities. A combination of genetic predisposition and in-utero exposure to teratogens (nicotine, alcohol, antiepileptic drugs) can arrest the fusion of the facial processes required for normal facial development. Failure of fusion of the maxillary prominence with the medial nasal prominence causes cleft lip (CL). Failure of fusion of the palatine prominences causes cleft palate (CP). Cleft lip and/or palate may be unilateral or bilateral and complete or incomplete. In addition to facial deformity, infants with CLP have feeding, hearing, speech difficulties, and defects in the dentition. Infants with cleft lips and/or palate require special feeding techniques (feeding in upright position, special feeding bottles) since they cannot effectively create negative sucking pressure. Treatment should be initiated as early as possible and may involve an interdisciplinary team of plastic surgeons, oral maxillofacial surgeons, otolaryngologists, pediatricians, and speech therapists. Nasoalveolar molding and lip taping are non-surgical techniques used to decrease the size of the lip/palatal defects and should be initiated early (at 2 weeks of age). Repair of the cleft lip is done at 3 months of age and of the cleft palate at 6 months of age with the aim of optimizing feeding and speech development without interrupting normal maxillofacial growth.

  • Incidence
  • Unilateral CLP is more common than bilateral CLP
  • Majority of cases are non-syndromic.

References:[1][2][3][4]

Epidemiological data refers to the US, unless otherwise specified.

References:[5][6][7]

The development of CLP is dependent on the interaction of environmental factors and genetic predisposition

References:[8]

  • Facial deformities: See “Subtypes and variants” below.
  • Feeding difficulties: depends on the type and severity of the cleft
    • CL: minimal feeding difficulties; may have suckling difficulties
    • CP: difficulty in suckling; nasal regurgitation of milk; choking/coughing during feeds; excessive aerophagia
  • Speech difficulties (hypernasality, unintelligible speech): due to velopharyngeal insufficiency
  • Dentition defects (in CP): due to gaps in the upper jaw at the sites of the clefts
  • Hearing loss: due to recurrent/persistent otitis media with effusion (OME)

References:[9][10]

Types of cleft lip

  • Microform cleft lip: notch on vermillion border of the upper lip; fibrous tissue band from the notch to the nostril; drooping of the ipsilateral nasal ala
  • Incomplete cleft lip: cleft on the upper lip which does not extend into the nostril (unilateral/bilateral)
  • Complete cleft lip: cleft on the upper lip which extends into the nose (unilateral/bilateral); more often associated with cleft palate

Types of cleft palate

References:[2][11]

References:[12]

Approach

  • Interdisciplinary management is required (oral maxillofacial surgeons. plastic surgeons otolaryngologists, pediatricians, and speech therapists)
  • All cleft lips and palates should be surgically repaired.

Conservative measures before surgery

  • Proper feeding techniques: feeding in upright position to prevent nasal regurgitation; use of specialized feeding bottles ; frequent burping
  • Nasoalveolar molding: Use of a custom made orthodontic prosthesis to bridge and reduce the palatal gap and passively mold the maxillary, palatal, and nasal structures into a normal shape
  • Nasal stent: to lift the drooping nostril and shape the nose
  • Lip taping: use of adhesive tape to reduce the defect; makes definitive surgery easier
  • Lip adhesion: suturing the edges of the cleft lip, in conjunction with NAM can be done in infants with complete CL/CP who are not responding well to lip taping

Surgical repair

  • Cleft lip repair (chelioplasty)
    • Goals: achieving normal lip contour and nose, improve oral competence
    • Timing: 3 months, surgery is usually staged with cleft lip being the first surgery and cleft palate done at a later time due to risk of surgery to infant
    • Tympanostomy tubes are placed at the same sitting: in children with OME causing hearing loss/recurrent infections
  • Cleft palate repair (palatoplasty)
    • Goals include closure of the defect, optimal speech development, and normal maxillofacial growth
    • Timing: 6–9 months: palatoplasty
  • Further surgeries are often required as the child grows older.
  • Speech assessment and therapy

References:[13][14][15][16][17]

  1. Kosowski TR, Weathers WM, Wolfswinkel EM, Ridgway EB. Cleft palate. Semin Plast Surg. 2012; 26 (4): p.164-169. doi: 10.1055/s-0033-1333883 . | Open in Read by QxMD
  2. Shkoukani MA, Chen M, Vong A. Cleft lip – A comprehensive review. Front Pediatr. 2013; 1 : p.53. doi: 10.3389/fped.2013.00053 . | Open in Read by QxMD
  3. Facts about Cleft Lip and Cleft Palate. https://www.cdc.gov/ncbddd/birthdefects/CleftLip.html. Updated: November 12, 2015. Accessed: April 9, 2017.
  4. Craniofacial Development. https://web.duke.edu/anatomy/embryology/craniofacial/craniofacial.html. Updated: December 12, 2016. Accessed: April 26, 2018.
  5. Facial and palatal development.
  6. Development of the Palate. https://discovery.lifemapsc.com/library/review-of-medical-embryology/chapter-55-development-of-the-palate. . Accessed: April 26, 2018.
  7. Prevalence (Number of Cases) of Cleft Lip and Cleft Palate. https://www.nidcr.nih.gov/DataStatistics/FindDataByTopic/CraniofacialBirthDefects/PrevalenceCleft%20LipCleftPalate.htm. Updated: April 8, 2017. Accessed: April 8, 2017.
  8. Murthy J, Bhaskar L. Current concepts in genetics of nonsyndromic clefts. Indian J Plast Surg. 2009; 42 (1): p.68-81. doi: 10.4103/0970-0358.53004 . | Open in Read by QxMD
  9. Burg ML, Chai Y, Yao CA, Magee W, Figueiredo JC. Epidemiology, etiology, and treatment of isolated cleft palate. Front Physiol. 2016; 7 : p.67. doi: 10.3389/fphys.2016.00067 . | Open in Read by QxMD
  10. Sivertsen A, Wilcox AJ, Skjaerven R, et al. Familial risk of oral clefts by morphological type and severity: population based cohort study of first degree relatives. BMJ. 2008; 336 (7641): p.432-434. doi: 10.1136/bmj.39458.563611.AE . | Open in Read by QxMD
  11. Walls R, Hockberger R, Gausche-Hill M. Rosen's Emergency Medicine - Concepts and Clinical Practice. Elsevier Health Sciences ; 2013
  12. Sharma R, Nanda V. Problems of middle ear and hearing in cleft children. Indian J Plast Surg. 2009; 42 (3): p.144. doi: 10.4103/0970-0358.57198 . | Open in Read by QxMD
  13. Nahai FR, Williams JK, Burstein FD, Martin J, Thomas J. The management of cleft lip and palate: pathways for treatment and longitudinal assessment. Semin Plast Surg. 2005; 19 (4): p.275–285. doi: 10.1055/s-2005-925900 . | Open in Read by QxMD
  14. Grayson BH, Maull D. Nasoalveolar molding for infants born with clefts of the lip, alveolus, and palate. Semin Plast Surg. 2005; 19 (4): p.294–301. doi: 10.1055/s-2005-925902 . | Open in Read by QxMD
  15. Salyer KE, Rozen ShM, Genecov ER, Genecov DG. Unilateral cleft lip: Approach and technique. Semin Plast Surg. 2005; 19 (4): p.313–328. doi: 10.1055/s-2005-925904 . | Open in Read by QxMD
  16. Cleft Lip and Palate.
  17. Leow AM, Lo LJ. Palatoplasty: evolution and controversies. Chang Gung Med J. 2008; 31 (4): p.335-345.
  18. Swearingen B. Transsphenoidal surgery for pituitary adenomas and other sellar masses. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/transsphenoidal-surgery-for-pituitary-adenomas-and-other-sellar-masses?source=search_result&search=pituitary%20adenoma&selectedTitle=3~150#H21307890.Last updated: June 29, 2015. Accessed: December 19, 2016.
  19. Core Curriculum for Cleft Palate and Other Craniofacial Anomalies: Plastic Surgery. http://acpa-cpf.org/core_curriculum/plastic_surgery.html. Updated: April 9, 2017. Accessed: April 1, 2017.
  20. Tips For Feeding an Infant with Cleft Lip. http://www.stlouischildrens.org/our-services/cleft-palate-and-craniofacial-institute/tips-feeding-infant-cleft-lip. Updated: April 9, 2017. Accessed: April 9, 2017.
  21. Bottles for Cleft Affected Children. http://www.cleftopedia.com/special-feeders/cleftbottles/. Updated: April 9, 2017. Accessed: April 9, 2017.
  22. Chapman KL, Hardin-Jones, MA Goldstein JA, Halter KA, Havlik RJ, Schulte J. Timing of palatal surgery and speech outcome. Cleft Palate Craniofac J. 2008; 45 (3): p.297-308. doi: 10.1597/06-244.1 . | Open in Read by QxMD