Severe chemosis and treatment following fronto-orbital advancement surgery for Crouzon syndrome: A case report

Abstract

Rationale: Crouzon syndrome is a craniofacial malformation caused by premature fusion of fibrous sutures in infants. It is one of the most common craniosynostosis syndromes, and surgery is the only effective treatment for correcting it. Postoperative complications such as encephalocele, infections, hematoma have been reported. We herein report a case of a 62-month-old boy with Crouzon syndrome who underwent fronto-orbital advancing osteotomy, cranial vault remolding, and extensive osteotomy and subsequently developed left proptosis and severe chemosis, these complications are rare and we believe it will be of use to clinicians, physicians, and researchers alike.

Patient concerns: The patient's skull had been malformed since birth, and he had been experiencing paroxysmal headaches coupled with vomiting for 4 months. Having never received prior treatment, he underwent fronto-orbital advancement at our clinic; afterward, left proptosis and severe chemosis occurred.

Diagnosis: The patient was diagnosed with Crouzon syndrome, and the complications included left proptosis and severe chemosis, confirmed by the clinical manifestations, physical examination, and computed tomography (CT).

Intervention: We carried out cranial vault remodeling and fronto-orbital advancement. We applied ophthalmic chlortetracycline ointment on the conjunctivae, elevated the patient's head, evacuated the hematoma, and carried out a left blepharorrhaphy.

Outcomes: The proptosis and chemosis resolved with no recurrence. No other complications occurred during the follow-up period (12 months), and CT scans revealed that the hematoma had disappeared. The calvarial vault reshaping was satisfactorily performed, and the patient's vision was not impaired.

Lessons: Severe proptosis and chemosis are rare complications that can occur after fronto-orbital advancement for Crouzon syndrome. A detailed preoperative examination (including magnetic resonance imaging and CT) is essential for diagnosis. Complete hemostasis, evacuation of hematoma, and placement of a periorbital drainage tube during surgery all contribute to an effective treatment plan. An ophthalmic ointment should be administered, and the patient's head should be elevated during the procedure. Evacuation of retrobulbar epidural hematoma and blepharorrhaphy could also help relieve proptosis and chemosis. Our report describes 2 rare complications associated with the treatment for Crouzon syndrome, and we believe it will be of use to clinicians, physicians, and researchers alike.

Figure 1

Proptosis and chemosis. Unexpected left proptosis and severe chemosis that occurred 3 days postsurgery. (A) Frontal view. (B) Lateral view.

Figure 2

Cranial computed tomography scan after fronto-orbital advancement. (A) Retrobulbar epidural hematoma. (B) Left proptosis.

Figure 3

Evacuation of the hematoma. Incision of the left upper eyelids and orbital periosteum to evacuate the hematoma, performed on the 4th day after fronto-orbital advancement.

Figure 4

Six days after hematoma removal and left blepharorrhaphy. Chemosis and proptosis resolved gradually after evacuating the hematoma and performing a left blepharorrhaphy.

Figure 5

Postoperative computed tomography scan. (A) and (B) Complete resolution of the hematoma.

Figure 6

One month after hematoma removal and left blepharorrhaphy. The patient's proptosis and chemosis were completely resolved.

Figure 7

Postoperative computed tomography scans 1 year after hematoma removal and left blepharorrhaphy. (A) and (B) No hematoma recurrence.