Related Experiment Video

Updated: Mar 17, 2026

Iris Fixation via External Pentagram Suturing
05:22

Iris Fixation via External Pentagram Suturing

Published on: May 5, 2022

2.0K

The ophthalmic sequelae of Pfeiffer syndrome and the long-term visual outcomes after craniofacial surgery

Neharika Sharma1, Timothy Greenwell1, Michael Hammerton2

  • 1South Australian Institute of Ophthalmology, Royal Adelaide Hospital, Adelaide, South Australia.

Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
|July 16, 2016
PubMed

Insights

Pfeiffer syndrome patients frequently experience ophthalmic issues like proptosis and keratitis. Fronto-orbital advancement surgery effectively corrects these, but long-term vision monitoring is crucial for optimal outcomes in craniosynostosis patients.

Area of Science:

  • Ophthalmology
  • Genetics
  • Craniofacial Surgery

Background:

  • Pfeiffer syndrome is a rare genetic disorder causing craniosynostosis and midface hypoplasia, leading to ophthalmic complications.
  • Ophthalmic sequelae are common in Pfeiffer syndrome, necessitating effective surgical and long-term management strategies.

Purpose of the Study:

  • To analyze ophthalmic sequelae rates in Pfeiffer syndrome patients.
  • To evaluate long-term visual outcomes following fronto-orbital advancement surgery.
  • To compare ophthalmic findings in Pfeiffer syndrome to other craniosynostosis syndromes.

Main Methods:

  • Retrospective analysis of medical records from 1988-2010 for Pfeiffer syndrome patients.
  • Diagnosis confirmed via clinical and genetic testing.
  • Long-term data presented as incidence rates per person-year.

Main Results:

  • Proptosis, refractive error, and strabismus were primary features; exposure keratitis and amblyopia were common secondary issues.
  • Fronto-orbital advancement significantly reduced proptosis rates and eliminated active exposure disease post-operatively.
  • Long-term follow-up revealed vision impairment from corneal scarring/amblyopia and blindness from optic atrophy.

Conclusions:

  • Pfeiffer syndrome presents high rates of proptosis and exposure keratitis, exceeding those in Apert and Crouzon syndromes.
  • Fronto-orbital advancement is effective in correcting orbital abnormalities associated with Pfeiffer syndrome.
  • Essential long-term ophthalmic follow-up is required to ensure the best possible visual outcomes for patients.
Abstract

More Related Videos

Full-Circle Cauterization of Limbal Vascular Plexus for Surgically Induced Glaucoma in Rodents10:10

Full-Circle Cauterization of Limbal Vascular Plexus for Surgically Induced Glaucoma in Rodents

Published on: February 15, 2022

2.0K
A Standardized Surgical Technique for Tessier Medial Transnasal Canthopexy06:08

A Standardized Surgical Technique for Tessier Medial Transnasal Canthopexy

Published on: January 23, 2026

230

Related Concept Videos

Glaucoma: Overview01:25

Glaucoma: Overview

Glaucoma is an eye condition characterized by increased intraocular pressure that damages the retina and optic nerve, leading to irreversible blindness if left untreated. The human eye has various components, including the cornea, iris, pupil, lens, and optic nerve. Aqueous humor is secreted by the epithelium of the ciliary body in the posterior chamber and flows through the trabecular meshwork and canal of Schlemm, maintaining normal intraocular pressure. The trabecular meshwork and the canal...
1.6K
Photoreceptors and Visual Pathways01:22

Photoreceptors and Visual Pathways

At the molecular level, visual signals trigger transformations in photopigment molecules, resulting in changes in the photoreceptor cell's membrane potential. The photon's energy level is denoted by its wavelength, with each specific wavelength of visible light associated with a distinct color. The spectral range of visible light, classified as electromagnetic radiation, spans from 380 to 720 nm. Electromagnetic radiation wavelengths exceeding 720 nm fall under the infrared category,...
10.6K
Open Angle Glaucoma: Treatment01:27

Open Angle Glaucoma: Treatment

In open-angle glaucoma, the iridocorneal angle remains open, but the trabecular meshwork becomes stiff, slowing down the outflow of aqueous humor. This causes a buildup of aqueous humor in the anterior chamber, leading to a sudden increase in intraocular pressure. The treatment for open-angle glaucoma focuses on reducing the elevated intraocular pressure by either decreasing the secretion of aqueous humor or increasing its outflow.
Drugs such as carbonic anhydrase inhibitors, α2- and...
1.1K
Angle Closure Glaucoma: Treatment01:28

Angle Closure Glaucoma: Treatment

Angle-closure glaucoma, or closed-angle glaucoma, is an eye condition where the iris bulges out and blocks the iridocorneal angle, resulting in a buildup of aqueous humor and increased intraocular pressure. Immediate medical attention is necessary due to the sudden onset of symptoms. The treatment for angle-closure glaucoma includes short-term and long-term approaches. Short-term treatment involves using eye drops like pilocarpine to lower intraocular pressure by increasing aqueous humor...
1.5K
Accessory Structures of the Eye01:17

Accessory Structures of the Eye

Optical perception, or vision, is an extraordinary sense dependent on converting light signals received via the ocular organs. These organs, known as eyes, are securely positioned within the bony cavities of the skull, called orbits. The orbits serve a dual purpose: a protective shield for the ocular globes and a stable attachment point for the soft ocular tissues. The eye's external protective mechanisms include the eyelids, which are edged with lashes that act as a barrier against foreign...
4.3K