Various genetic disorders have been discovered in infants at the time of childbirth. Genetic disorders are caused by the mutation of genes and result in different abnormalities with varying severity. One such rare genetic disorder is Crouzon syndrome, which affects not just the appearance but also the functioning of the victims. However, thanks to medical advancements in recent years, corrective surgeries have brought about desirable outcomes to the appearance and functioning of patients.

Let us take a look at how plastic corrections have brought about tremendous changes in a boy who suffered from this syndrome as an infant.

How does Crouzon syndrome affect the appearance of a person?

Crouzon syndrome is the mildest form of a disorder called “craniosynostosis.” It was first described by the French physician Octave Crouzon in 1912 when he identified a mother and daughter with bulging offset eyes, skull deformities, and facial anomalies.

Crouzon syndrome is caused by the premature fusion of the fibrous joints between certain skull bones. These fibrous joints are called “sutures.” They allow an infant’s head to grow and expand before fusing to form the skull. Premature fusion affects the normal growth of the skull. This ultimately affects the shape of the face and the head.

Infants having this genetic disorder have wide-set, bulging eyes that are misaligned, shallow eye sockets, and narrow ear canals. Their appearance is further affected by a flattened forehead and a small beak-shaped nose. Furthermore, many infants have an underdeveloped jaw as well as a cleft lip and palate (an opening in the lip and the roof of the mouth).

Abnormalities in children suffering from this genetic disorder

Crouzon syndrome is estimated to affect one out of 60,000 newborn babies. The severity of this syndrome differs from one infant to another. However, Crouzon syndrome does not affect the intelligence of a person.

Children suffering from this genetic disorder have the following difficulties.

• vision problems
• hearing loss
• breathing difficulties
• inflammation in the front of the eyes
• drying of cornea
• fluid build-up in the brain
• sleep apnea

Plastics corrections for Crouzon syndrome

The deformities caused by Crouzon syndrome can be corrected optimally through plastic corrections that involve single or multiple surgeries, depending on the severity of the syndrome. Treating this requires the expertise of multiple specialists like pediatricians, oral surgeons, neurosurgeons, ENT specialists, ophthalmologists, and plastic surgeons. Look at the pictures above and observe the different stages of plastic corrections carried out for a child suffering from Crouzon syndrome.

The plastic corrections for Crouzon syndrome children are a long-drawn process. It requires cooperation and coordination between the doctors, the parents, and the child. The postoperative appearance corrects all anomalies to an optimum level and helps the child to function better.

Plastic corrections involve several procedures.

• Calvarial vault remodeling: This corrective surgery is usually performed on infants to reshape the skull when they are less than one year old.
• LeFort 3: This procedure is typically done between seven and ten years of age to correct the midface.
• Facial Bipartition (Monobloc): This procedure is usually carried out between seven and ten years of age to correct the alignment of the forehead with the midface.
• Box osteotomy: This procedure is done around 12 years of age once the child grows permanent canine teeth. It helps to narrow the distance between the eyes.
• Orthognathic surgery: This oral surgery is done around 16 to 18 years of age to improve the biting action.
• Rhinoplasty: This is the final facial surgery that is performed to improve the projection of the nose using a rib graft.

The treatment process for Crouzon Syndrome can indeed be quite complex, and the duration can vary significantly depending on the specific needs of the patient. In general, surgical interventions are the primary form of treatment and may begin in infancy and continue into late adolescence as the child grows.

For instance, surgeries to remodel the skull and alleviate increased intracranial pressure or to correct the skull and facial deformities may start early, often before the age of one year. These initial procedures are crucial and aim to lower the risk of cognitive disabilities, airway obstruction, and visual problems. As the child grows, further surgeries may be required to correct midface abnormalities and jaw alignment, typically during different stages of childhood and adolescence.

Orthodontic procedures may occur during the teenage years, and more comprehensive surgeries to correct and realign jaw bones may take place after age 17 or once growth is complete. Throughout this period, minimally invasive techniques, such as endoscopic repairs and the use of helmets for shaping the skull, may be utilized for better outcomes with less scarring and shorter recovery times. Moreover, due to the variability in the severity and presentation of the syndrome, each patient’s treatment plan is highly individualized, often involving a multidisciplinary team of specialists.

Given that each case is unique, the specific timing and need for repeat procedures will depend on the child’s developmental progress and the judgment of their medical team.

In general, the parents of children having Crouzon syndrome are highly stressed and guilty. They undergo counseling sessions to overcome these negative feelings. Furthermore, even before the plastic correction procedures begin, these parents are made aware of the prognosis (details of the treatment, expected outcome, chances of recovery, and recurrence).