Factors not associated with increased risk 

  • maternal 
    • alcohol consumption
    • topical corticosteroid use during first trimester 
    • of pregnancy 
    • antibiotic use during early pregnancy 
  • prematernal obesity 
  • water fluoridation 

Associated conditions 

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  • cleft palate may be a feature of more than 400 specific genetic or chromosomal syndromes, including
    • Robin sequence (previously called Pierre Robin sequence)
    • Loeys-Dietz syndrome 
    • X-linked cleft palate with ankyloglossia
    • Down syndrome
    • Prader-Willi syndrome
  • cleft lip with or without cleft palate is a feature of more than 200 specific genetic or chromosomal syndromes, including
    • Down syndrome
    • van der Woude syndrome
    • trisomy 13
    • Smith-Lemli-Opitz syndrome
  • sensorineural hearing loss 

Making the diagnosis 

  • clefts of lip and/or palate are generally recognizable upon newborn examination as visible or palpable disruption of normal lip and palate structure
  • routine prenatal ultrasound screening may help detect orofacial clefts in utero

Differential diagnosis 

  • determine whether patients with cleft lip and/or palate have either
    • nonsyndromic cleft
    • cleft in conjunction with a genetic or chromosomal syndrome

Testing overview 

  • ultrasound and magnetic resonance imaging may be used for prenatal screening for orofacial cleft
  • routine second-trimester prenatal ultrasound
    • may detect cleft lip with or without cleft palate
    • limited ability to detect cleft palate alone 
  • videonasopharyngoscopy and multiplanar videofluoroscopy may provide anatomic and dynamic data useful for correcting velopharyngeal insufficiency

Treatment overview 

  • clearance of airway (most immediate concern in new infant), followed by adequate nutrition due to feeding complications
  • multidisciplinary approach typically required for 
  • comprehensive care
  • preoperative dentofacial orthopedics may be used to approximate soft tissue prior to surgery (newer nasoalveolar molding technique more commonly used)
  • primary treatment is surgery, but high-quality evidence is lacking to determine best surgical techniques for cleft repair
    • goals of surgery include normalized appearance, language, speech, hearing and psychosocial development with intact palate, airway, masticatory function, and dental health
    • timing of surgical repair
      • cleft lip repair traditionally performed at age 10 to 12 weeks
      • most craniofacial surgeons suggest complete repair of palatal clefts between age 9 and 12 months (prevent negative effects of delayed repair on language and speech development)
  • choice of surgical techniques typically based on size and extent of cleft, as well as surgeon experience
  • consider rhinoplasty for cleft lip nasal deformity
    • primary (simultaneous rhinoplasty and lip reconstruction)
    • secondary (after lip reconstruction)
  • discharge when oral alimentation sufficient

Complications of surgery

  • oronasal fistulae (3.4% to 29% of cases) postoperatively
  • other surgical complications may include
    • bleeding
    • infection
    • irregular healing of scars and contractures