Referring Physicians >> Newsletters >> March 2012 Plastic & Reconstructive Newsletter >> Four Challenging Congenital Conditions
Review the presentation, referral recommendations, and treatment options for craniosynostosis, hand anomalies, brachial plexus injury, and hemangiomas/vascular malformations.
Craniosynostosis most often occurs by itself, but can also occur in conjunction with genetic disorders such as Apert, Crouzon, Pfeiffer, or Saethre-Chotzen syndrome.
The most common type is scaphocephaly (sagittal synostosis), which forces the head to grow long and narrow. It occurs frequently in premature infants, and more often in boys than in girls. Other types include plagiocephaly, brachycephaly, and trigonocephaly.
Craniosynostosis usually presents in the first months of life. Diagnosis is through physical examination of the infant’s head shape, sutures, and fontanelles, with confirmation from plain radiographs, CT, and/or MRI. Craniosynostosis differs from positional posterior plagiocephaly, which presents with skull flattening only, and no suture fusion.
Infants with craniosynostosis should be referred to a pediatric plastic surgeon as soon as possible after diagnosis. Traditional open surgery involves removing strips of skull bone to create artificial sutures; newer endoscopic techniques require the baby to wear a custom-made molding helmet up until 1 year of age.
For more information on the treatment of craniosynostosis or other craniofacial conditions, or to contact us, click here.
Syndactyly occurs in approximately 1 of every 2000-3000 births. Radiographs may be ordered to confirm whether the syndactyly is simple (the fingers are joined by a skin bridge only) or complex (two or more bones are fused).
Separation surgery is almost always indicated to improve appearance and function. For simple syndactyly, this usually takes place between 12 and 18 months of age. The digits are separated, and web space is created using skin grafted from the abdomen.
Children with simple syndactyly that involves the thumb or little finger, however, should undergo separation surgery at around 6 months to prevent permanent deformity caused by finger-length differences. Children with very complex syndactyly may also require earlier surgery, often conducted in stages, to correct the underlying skeletal malformation.
Polydactyly occurs in approximately 1 of every 500-1000 births. Ulnar polydactyly is the most common type, followed by radial and central polydactyly.
Surgical treatment usually takes place between 1 and 2 years of age. In cases of ulnar polydactyly, the extra digit is removed in a minor operation; a well-formed digit may require a more involved procedure. Treatment for radial polydactyly is more complex, requiring reconstruction of the skin, soft tissues, tendons, ligaments, and joints to create a single functional thumb from the two duplicated thumbs. Treatment for central polydactyly is similarly complex, and may require multiple operations.
Hypoplastic thumb can present by itself or with other radial deficiencies. In 60 percent of cases, both thumbs are involved.
Surgical treatment options depend on the type and severity of hypoplasia, but always aim to provide the child with the best possible grip and pinch function. In most cases, reconstruction is performed between 1 and 2 years of age. If required, pollicization (creation of a thumb from an existing index finger) should be performed at or after 1 year of age, when oppositional pinch develops, but before 3 years of age.
For more information on the treatment of congenital hand anomalies, or to contact us, click here.
Brachial Plexus Injury
Congenital brachial plexus injuries are found in approximately two of every 1,000 births—usually in larger infants who had shoulder dystocia during birth. They are usually obvious in the first days of life. Some infants will appear to have no muscle control or movement in the entire upper extremity. Others may be able to move their upper arm but not their wrist or hand, or vice versa.
An infant with a suspected brachial plexus injury should be evaluated by a pediatric plastic surgeon and pediatric neurosurgeon as soon as possible, ideally in a multidisciplinary Pediatric Brachial Plexus Clinic such as the one at UW’s American Family Children’s Hospital.
Approximately 90% of children are able to regain full motor function through physical therapy alone; the remaining 10% may be candidates for surgery beginning at 4 to 6 months of age.
For some children, surgical neurolysis may be the only intervention needed. More severe cases may require nerve transfers or grafts. Older children with incomplete recoveries may benefit from tendon transfers to restore extremity function.
For more information on the treatment of brachial plexus injury, or to contact us, click here.
Hemangiomas usually proliferate in the first year after birth, and begin to involute after the first year or two. By age 5, 50% of hemangiomas are completely involuted; by age 7, 70% are completely involuted. Most only require observation, but for more severe hemangiomas, pharmacologic therapy includes topical, intralesional, or systemic corticosteroids; interferon-alpha; or vincristine.
More recently, researchers have found the beta-blocker propanolol to be an effective treatment. Propanolol therapy should be preceded by a full cardiovascular workup, and be managed carefully by a pediatrician and plastic surgeon due to an increased risk of hypoglycemia, hyperkalemia, and bradycardic hypotension. Topical timolol gel can also be used to treat facial hemangiomas around the orbit—with less risk of cardiac complications.
Hemangiomas that block the child’s visual field, airway, oral or digestive tract, or auditory canals—or have severe ulceration—usually require surgical excision. Children who undergo surgical treatment, or have hemangiomas that do not involute by 6 years of age, often require laser therapy to restore normal skin appearance.
Vascular malformations are present at birth, can increase in size as the child grows, and do not involute without treatment. They are classified as follows:
For more information on the treatment of hemangiomas and vascular malformations, or to contact us, click here.