Makindo Medical Notes

one affected person in the family with a cleft, the likelihood of having a child with a cleft lip and/or palate is 2 to 5 per cent so a slight increased risk

About

• Seen in 16.86 cases per 10,000 live births
• Patients can have a cleft lip +/- cleft palate

Description

• Unilateral incomplete cleft lip
• Unilateral complete cleft lip/palate
• Bilateral complete cleft lip and palate

Aetiology

• It is important to appreciate that the upper lip and palate form separately and over different gestational age ranges.
• Although clefts in the lip and palate often occur together they arise from different embryological processes.
• Between the fourth and sixth weeks of pregnancy, a developing baby’s lip begins to form.

Clinical

• Difficulty feeding, usually obvious appearance
• Ensure look in mouth ans see if palate affected

Investigations

• A prenatal MRI can detect a cleft palate as early as the 15th or 16th week of pregnancy. The diagnosis will then be confirmed with an exam when the baby is born.

Pathology

Management

• May need specialist help on how to feed with a cleft. This can be assessed. A special feeding device may be needed. Weigh baby weekly to monitor any weight loss.
• Referral top the local specialist cleft lip service. Involve nurses, surgeons, speech and language therapists. Ensure baby can eat and drink. Surgical correction usually successful
• Most unilateral complete cleft lips will require a dentofacial maxillary appliance (DMA) Most bilateral complete cleft lips will require an elastic chain premaxillary retraction appliance (ECPR). The dentofacial appliance (referred to as a Latham-type device) will bring the segments of the palate closer together to optimize the outcome of the cleft lip repair.
• Cleft lip at 3-5 months: unilateral cleft lip is performed using a rotation-advancement, geometric, straight-line, or hybrid technique. For bilateral cleft lip repair, most surgeons use either the Millard or Mulliken technique and their variations.
• Cleft palate at 12 months: The 2-flap palatoplasty and Furlow double-opposing Z-plasty are most commonly used.
• There is sometimes associated ear related problems and ongoing monitoring and ear tests may be needed. Babies with middle ear fluid and related hearing loss need an operation to drain the fluid and insert ventilation tubes

References