What are Craniofacial Anomalies?

Craniofacial anomalies are a diverse group of deformities in the growth of the head and facial bones. Anomaly is a medical term meaning “irregularity” or “different from normal.”

These abnormalities are congenital (present at birth) and there are numerous variations–some are mild and some are severe and require surgery.

What are the most common types of craniofacial anomalies?

  • Cleft lip. An abnormality in which the lip does not completely form. The degree of the cleft lip can vary greatly, from mild (notching of the lip) to severe (large opening from the lip up through the nose).
  • Cleft palate. Occurs when the roof of the mouth does not completely closed leaving an opening that can extend into the nasal cavity. The cleft may also include the lip.
  • Craniosynostosis. A condition in which the sutures (soft spots) in the skull of an infant close too early, causing problems with normal brain and skull growth. Premature closure of the sutures may also cause the pressure inside of the head to increase and the skull or facial bones to change from a normal, symmetrical appearance.
  • Hemifacial microsomia. A condition in which the tissues on one side of the face are underdeveloped, affecting primarily the ear (aural), mouth (oral), and jaw (mandibular) areas.
  • Vascular malformation. A birthmark or growth, present at birth, that is composed of blood vessels and can cause functional or aesthetic problems.
  • Hemangioma. A hemangiomas is an abnormally growing blood vessel in the skin that may be present at birth (faint red mark) or appear in the first months after birth.
  • Deformational (or positional) plagiocephaly. A misshapen (asymmetrical) shape of the head (cranium) from repeated pressure to the same area of the head.

What Is Craniofacial Surgery?

  • Craniofacial surgery is a type of plastic surgery that deals with the correction and reconstruction of malformations or problems of the skull.
  • One of the most common conditions treated with craniofacial surgery is craniosynostosis, the early closing of the spaces between the bones of the skull, resulting in abnormal skull growth.
  • The particular surgery to correct craniosynostosis is called a cranial vault remodeling.
  • A plan for craniofacial surgery may be put in place as early as 8 or 9 months of age, or as soon as the problem is recognized.

Pre-op essentials

  • About 6 weeks before your child’s surgery, your child may begin blood enrichment therapy to build up the quality of his or her blood using medication.
  • Your child also will be given iron supplements to help his or her body produce red blood cells. This blood therapy will reduce the need for blood transfusions during surgery and will help your child heal and recover faster after surgery.
  • In the 6 weeks leading up to the surgery, your child will need to have a blood count done at 3 different points in the therapy process: at the start of the medication therapy, halfway through the therapy, and then at the end of therapy, prior to surgery.
  • The blood test will determine your child’s blood type, a cross-matching blood type and a red blood cell count. In case your child needs a blood transfusion during the surgery, matching blood from the blood bank will be made available, or you and/or other family members may choose to donate matching blood instead.
  • Several weeks before surgery you will meet again with your surgeon for a preoperative history and physical and to discuss the surgery.

What to expect during surgery

  • When general anesthesia is needed, there are important rules for eating and drinking that must be followed in the hours before the surgery.
  • Our office will give you specific eating and drinking instructions for your child based on your child’s age. No matter what age your child is, you should follow the specific instructions given to you on the phone by the nurse.
  • If your child is very scared or upset, the surgeon may give a special medication to help him or her relax.
  • Once your child is asleep, an IV line will be inserted into a vein in your child’s hand or arm so that medication can be given to keep him or her sleeping throughout the surgery. Your child will have no pain during the surgery and no memory of it afterward.
  • To complete the craniofacial surgery, the surgeon will make an incision or cut in your child’s scalp to get to the skull bones. This incision will run from ear to ear, and will be a zig-zag rather than a straight incision. The zig-zag incision will allow your child’s hair to grow over the scar and make it less noticeable as it heals.
  • Your child will have sutures or stitches in his or her scalp to close the zig-zag incision. All of the sutures will be dissolvable, meaning that they will not need to be removed.
  • As the skin heals, the parts of the sutures that are under the skin will dissolve on their own and will be absorbed into the skin. Any part of the suture that you can see on the top of the skin’s surface will dry up and fall off.

After Surgery

  • Your child will be moved to a recovery room to be watched until he or she is alert and vital signs are stable. The length of time your child will spend in the recovery room will vary because some children take longer than others to wake up after anesthesia.
  • Children coming out of anesthesia may react in different ways. Your child may cry, be fussy or confused, feel sick to his or her stomach, or vomit.
  • These reactions are normal and will go away as the anesthesia wears off.
  • Your child will have a gauze dressing wrapped around his or her entire head like a helmet.
  • Under the dressing, your child will have a small tube that will be pinned to his or her hospital gown. The tube will suction out the bloody drainage from the incision and collect it in a small container. The amount of drainage will decrease over the next few days, and the drainage will change from bloody to light pink. The dressing and the drainage tube will stay in place for at least 3 days.
  • Your child’s face will be swollen and his or her eyes might be swollen shut. Although it is hard for parents to see their child’s face so swollen, this swelling is completely normal and is not painful. Sometimes, the swelling gets worse on the second or third day after surgery.
  • By the time your child returns for his or her first post-surgical follow-up visit, usually 1 week after surgery, much of the swelling will have gone down.
  • Your child can be given pain medication as needed, when he or she wakes up.
  • When your child is alert, he or she will be moved to the Pediatric Intensive Care Unit (PICU) for the first night so the nursing staff can monitor his or her condition closely.
  • He or she will be moved to a regular hospital room when the doctor feels your child is ready, usually sometime the next day after surgery.
  • When your child is drinking well and urinating normally, the IV will be removed by a nurse.

At Home Care

  • Your child will stay in the hospital until the doctor feels he or she is ready to go home, which usually is about 3 to 4 days after surgery.
  • When your child is discharged from the hospital, the gauze dressing will be removed. A nurse will give you instructions on cleaning the scar, which usually is just light soap and water, followed by a coating of antibiotic ointment.

A complete list of instructions for taking care of your child at home will be given to you before you leave the hospital.

If you notice any of the following changes in your child, call the surgeon right away:

  • Fever higher than 38.5˚C
  • Trouble breathing or skin color changes (pale, blue or gray)
  • Bleeding or foul-smelling drainage from the scar
  • Unusual redness or swelling
  • Any unusual changes in the look of the skull

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