Crouzon Syndrome Treatment & Management

Updated: Jul 23, 2019
  • Author: Amarateedha Prak LeCourt, MD; Chief Editor: Dirk M Elston, MD  more...
  • Print
Treatment

Approach Considerations

Patient care necessitates multifaceted specialization and management. High among the approaches is the implementation of plastic reconstructive surgery, which has been found to have immediate benefits. Despite the reparative effects of surgeries, continued follow up is still generally required for patients (dependent upon the severity of the malformations). [23]

On the horizon for drug therapies, an US Food and Drug Administration–approved trial for compounds (leflunomide, cyclosporin A, and their functional analogs) that influence craniosynostosis is at animal testing level and has shown some effect. [41]

Next:

Medical Care

Nonsurgical treatment of Crouzon syndrome has been performed using orthopedic and orthodontic devices, as well as prosthetics. [42, 43]

Patients with sleep-disordered breathing as a consequence of syndromic craniosynostosis have been treated with Tübingen palatal plate appliances as well as continuous positive airway pressure machines. [34]

Speech and language therapy is common, and hearing aids may benefit patients affected by hearing impairment. [22]

Previous
Next:

Surgical Care

Neurosurgical procedures such as decompressive craniectomy are recommended in cases of intracranial hypertension, which can cause papilledema and optic atrophy. This surgery is difficult, and the procedure must be considered and undertaken in stages. Papilledema may persist despite craniectomy. [5] For intracranial hypertension derived from hydrocephalus, ventriculoperitoneal shunts have also been found to be effective. [19, 31]

Facial plastic surgery can be of great benefit. [44] It is usually based on various craniofacial osteogenesis distraction methods and systems; patients usually wear a custom-fitted helmet (or cranial band) for several months after surgery. [6, 45, 46, 47, 48, 49, 50, 51, 52] Additionally, ocular surgeries (eg,. lateral canthoplasty) have been used to repair the antimongoloid slants found in patients afflicted by craniosynostosis. [53] It is one of the few syndromes for which the cosmetic results of the surgery can be strikingly effective. The surgery minimizes the risk linked to the neurological complications, but it also has an impact on stigmatization feelings and the psychological and social implications. [23] Respiratory difficulties may be alleviated by tracheostomy or nasal stents. [19, 34]

Minimally invasive endoscopic surgery can be used to repair cerebrospinal fluid leaks. [31]

Asymptomatic patients could have malocclusions repaired with orthodontic treatment using orthognathic surgery (but may also forgo it). [19]

Previous
Next:

Consultations

Consultations with audiologists, ophthalmologists, otorhinolaryngologists, neurologists, neurosurgeons, pediatricians, psychiatrists, reconstructive surgeons, speech pathologists, and stomatologists are usually required.

Previous
Next:

Complications

Complications that may occur from surgical procedures are described below. [23, 31, 52, 54, 55]

Osteotomies (namely Le Fort II and III) can lead to infraorbital nerve damage, orbital/globe injury, strabismus, excess blood loss, and infection. In one case study, cerebrospinal fluid rhinorrhea and sinus pain persisted despite no initial complications post surgery.

Distraction osteogenesis can have complications such as surgical site infections (superficial and deep), abscess formation, correction device failures (eg, distractors), and asymmetric advancement.

For reconstructive surgeries of craniofacial malformations in general, psychological issues may arise from the consequences of multiple surgeries and recuperation periods. For younger patients, postoperative hospitalizations can affect school attendance, which can produce stigmatization by peers (eg, intrusive questions about physical changes or absences) or being held back from progressing in their education.

Owing to the respiratory issues associated with Crouzon syndrome, anesthesiologists may encounter difficulties with airway management and intubation when the patient is placed under general anesthesia.

Previous
Next:

Long-Term Monitoring

Children with syndromic craniosynostosis have been found to be at increased risk of sleep-disordered breathing such as obstructive sleep apnea syndrome. In one study, children with Crouzon were subsequently also affected by a higher likelihood of daytime sleepiness. [34, 56]

Previous