Cleft Lip & Palate

Etiology:

During early pregnancy, separate areas of the face develop individually and then join together to create what we all consider to be a normal appearance.

In approximately 1 in 1000 births, some parts do not join properly resulting in facial clefts. For example if the medial and lateral growth centers that make up the upper lip fail to fuse, the result is a cleft lip and likewise for a cleft palate.

Cleft lip

A cleft lip is a condition that creates an opening in the upper lip between the mouth and nose. It can range from a slight notch in the colored portion of the lip to complete separation in one or both sides of the lip extending up and into the nose. A cleft on one side is called a unilateral cleft, likewise a cleft on both sides is considered bilateral.
Photos of cleft lips

Treatment & Timing of Surgery

Cleft lip surgery is usually performed when the child is about 3 months old. This timing is more of historic significance but it is used now as it allowed for the tissues to grow enough to enhance long term outcomes. Surgery at this age is safe and routinely performed by trained craniofacial surgeons all over the world.

The objective of cleft lip surgery is to close the separated lip tissue, restore oral competency while establishing normal shape to the mouth and nose. The ultimate goal is to restore function and eliminate the social stigmata associated with this condition so that, if done well, it should be hard to tell the cleft ever existed.

Preoperative Management

  1. Be patient. This condition is not of surgical urgency. Therefore, proper education tends to lead to a better overall experience with regards to meeting expectation.
  2. A trained craniofacial surgeon, who is associated with a state recognized craniofacial team, should follow a child with a cleft. A cleft is a multidisciplinary condition and requires the trained eye of a variety of health care providers.
    1. Your pediatrician is essential to ensuring that your child is healthy prior to surgery and assisting in the postoperative care.
    2. A lactation specialist is often helpful with regards to assisting in breast feeding as well as selection of bottle nipples (Haberman, Pigeon nipple, etc)

Day of Surgery

  1. Take a deep breath.
  2. The child should have not eat or drink 6 hours prior to surgery. You may give water or pedialyte 4 hours before surgery but nothing there after. The reason for this is to minimize the risks of anesthesia.
  3. Arrive 2 hours earlier than your scheduled surgery time to the hospital
  4. The operation typically takes between 3 to 5 hours
  5. Afterwards, your child will have a small dressing on the lip
  6. In my practice, I recommend one overnight stay. Staying in the hospital for one night reduces the stress of the parents in managing any acute post-operative issues, allows the child to be fed by a trained professional, enhances post-operative pain management and most importantly is safest.
  7. I see the child in my office weekly for the next 2-4 weeks and then monthly thereafter.
cleft-lip-palate
cleft-lip-palate
cleft-lip-palate
cleft-lip-palate

Cleft Palate

Cleft Palate

The palate is the roof of your mouth and as such separates the mouth from the nose. It is made of bone and muscle and is covered by a thin, wet skin that forms the red covering inside the mouth.

The palate plays two important roles early in life. First it has an extremely important role during speech. The intact palate moves back towards the throat when talking. By doing this it prevents the movement of air through the nose during certain sounds. Inability to do this result in a condition called velopharyngeal insufficiency or hypernasality. The palate is also very important when eating. It prevents food and liquids from going up and out of the nose.

cleft-lip-palate
cleft-lip-palate

Illustration

Treatment and Timing of Surgery

A cleft palate is initially treated with surgery safely when the child is between 8 to 18 months old. The major reason the palate is repaired at this age is that this is the typical time when early speech develops. Closure of the palate with, in part, enhance this important milestone.

The major goals of surgery are to close the gap or hole between the roof of the mouth and the nose and reorient the muscles in the soft portion of the palate while making the palate long enough so that the palate can perform its function properly.

There are many different techniques that surgeons will use to accomplish these goals. The choice of techniques may vary between surgeons and should be discussed between the parents and the surgeon prior to surgery.

Diagram of techniques

Preoperative management

  1. Again education and discussion with a trained craniofacial surgeon is essential to understanding the pathophysiology of this condition and managing surgical expectation.
  2. Relationship with a state recognized craniofacial team is vital with cleft palate disease
    1. Pediatrician to optimize pre and post-operative medical issues
    2. Lactation specialist to ensure proper nutrition and growth
    3. Otolaryngologist to monitor and treat hearing dysfunction
    4. Oral Surgery/Dentistry to monitor dental development and jaw growth
    5. Speech pathologist for maximize post-operative speech development
    6. Broad certified craniofacial surgeon

Day of surgery

  1. Again take a deep breath.
  2. The child should have nothing by mouth 6 hours prior to surgery. You may give water or pediolyte 4 hours up to surgery but nothing there after. The reason for this is to minimize the risks of anesthesia.
  3. Arrive 2 hours earlier than your scheduled surgery time to the hospital
  4. The operation typically takes between 2 to 3 hours
  5. Afterwards, your child will be brought to the recovery room where they will begin a liquid diet
  6. In my practice, I prefer one overnight stay. The reason is to reduce the stress of the parents in managing any acute post-operative respiratory issues, allows the child to be fed by a trained professional, enhances post-operative pain management and most importantly its safest.
  7. The child is maintained on a liquid diet for 1 week followed by a soft diet for 1 additional week after which an age appropriate diet is resumed.
  8. The child is then follow weekly for the next 2-4 weeks and then monthly thereafter.

References:

  1. Cleft Lip and Palate: Review Ciminello FS, Morin RJ, Nguyen TJ, and Wolfe SA Compr Ther. 35(1) pgs 37-43, 2009
  2. Pierre Robin Sequence: History and Pathophysiology Frank S. Ciminello MD, George Dreser MD, Nicole A. Anderson MD, and S. Anthony Wolfe MD, FACS Stryker Hyperguide, 2008
  3. Pierre Robin Sequence: Medical and Surgical Management Frank S. Ciminello MD, Nicole A. Anderson MD, George Dreser MD, Silvio Podda MD, and S. Anthony Wolfe MD, FACS Stryker Hyperguide, 2008
  4. Switch Cranioplasty and BMP-2 in a Hemicranial Reconstruction in an Infant. S. Podda, F. Ciminello, and S.A. Wolfe Craniofacial Surgery (12); pp 77-82, 2008
  5. Distraction Osteogenesis of the Cleft Maxilla. Granger B. Wong MD, DMD, FACS, Frank S. Ciminello MD, and Bonnie L. Padwa DMD, MD Facial Plastic Surgery, Vol 24(4); pp 467-471, 2008