3 primary diagnoses
- Giant fornix syndrome (GFS)
- Silent sinus syndrome (SSS)
- Silent brain syndrome (SBS)
The common factor in these 3 conditions is an alteration of the volumetric relationship between the bony orbit and its soft tissue contents.
In SSS the orbital bony volume is increased due to contraction and atrophy of a chronically infected maxillary sinus, causing the orbital floor to descend.
In SBS the orbital bony volume is also increased, but in this case the orbital roof is pulled up, as a result of ventriculoperitoneal (VP) shunting early in life, the reduction of intracranial pressure pulling up the orbital roof (Arch Ophthalmol 1996; 114:1206).
GFS, most commonly seen in the elderly, is caused by a reduction in orbital soft-tissue volume, mostly from loss of fat, perhaps due to blepharoplasty surgery or age-related fat atrophy, causing a deep dead space to be created in the fornices, where colonization with bacteria and fungus occurs (Ophthalmology 2004; 111:1539).
- "Senile sunken upper lids" is another term for GFS.
- "Prostaglandin associated periorbitopathy" is a variant of this disease.
- In addition to fat atrophy, from direct suppression of adipogenesis by prostaglandins, Muller's muscle is also stimulated by prostaglandins, with lid retraction and lagophthalmos as additional causes of chronic ocular irritation (OPRS 2012; 28:e33).
- In the original description of GFS all 12 patients had Staphylococcus aureus colonization of their fornices as evidenced by culture (Ophthalmology 2004; 111:1539).
- There can be overlap between the enophthalmos syndromes; SBS can cause GFS (Cornea 2012; 31:1065).
- An aspergilloma developed in the deep fornix of an adult who had undergone VP shunting as a child and had developed SBS (Cornea 2012; 31:1065).
- This patient developed fungal keratitis, which led to enucleation; the aspergilloma was not discovered until the enucleation.
Too few cases have been described in the literature for accurate epidemiologic data on GFS, but data from case series can be cited.
- In a series of 6 cases there were 4 women and 2 men with an age range of 61–85 (OPRS 2013; 29:63).
- In another series of 5 cases the mean age was 75 years (range 70–95) and all patients were female (OPRS 2012; 28:4).
- In the original description of 12 cases the mean age was 85 (range 77–93) and 10 were female (Ophthalmology 2004; 111:1539).
Very few cases of SBS have been described in the literature.
- In the original description there were 2 patients aged 24 and 25 years who had undergone VP shunting in their teens (OPRS 2009; 25:434).
- The disease can present in the fourth decade and VP shunting can have taken place in infancy (Cornea 2012; 31:1065).
SSS can present throughout life.
- In the original description there were 19 patients with mean age 36 years (range 29–46), (Ophthalmology 1994;101:772).
With GFS, the complaints are primarily related to chronic unilateral relapsing conjunctivitis.
- In addition to the discharge there might be symptoms of chronic irritation and dryness.
- Patients might be aware of chronic discharge and injection.
- In the prostaglandin analogue there is glaucoma treated with prostaglandin analogues or Latisse (bimatoprost 0.03%) has been used for hypotrichosis.
With SBS there is a history of VP shunting at a young age.
With SSS the only complaint is the asymmetry, but displacement of the globe does not usually cause visual symptoms (Figure 1).
- There is a case report of vertical diplopia due to SSS, worse on upgaze. (OPRS 2013; 29:e130).
- However, the patient presented with a one day history of diplopia, there was periocular pain, hypertropia and elevation deficit were noted on examination, orbital imaging showed maxillary sinusitis, with a normal orbital floor and the SSS was evident on CT scan one year later.
- Therefore, visual symptoms including diplopia, and periocular pain might be from the causative process of infection and inflammation rather than the secondary enophthalmos and globe displacement.
- There might be a history of treatment for chronic sinusitis.
- Patients might be unaware of the asymmetry and the sinus disease.
- There might be other causes of acquired and congenital asymmetry and the SSS might be contributory.
- History of trauma, especially blunt trauma and orbital fracture repair
- Radiotherapy to the periocular region and/or orbit
Figure 1. Silent sinus syndrome.
In GFS the primary clinical feature is chronic discharge and conjunctival injection (Figure 2).
- Deep superior fornix with evidence of bacterial colonization
- The inferior fornix might be involved in GFS (OPRS 2007; 23:256).
- The sunken and elongated superior fornix can create the appearance of involutional ptosis.
Figure 2. Giant fornix syndrome.
SBS can cause severe keratopathy due to lagophthalmos and upper lid entropion (OPRS 2009; 25:434).
In SSS the primary clinical features are enophthalmos, hypoglobus and asymmetry of the maxillary sinus buttress.
- There might be other secondary causes of ocular surface abnormalities including poor eyelid apposition to the globe, resulting in exposure keratopathy.
- In rare cases, SSS can involve a sinus other than the maxillary sinus.
- Frontal sinus atelectasis can cause hyperglobus (Otolaryngol Head Neck Surg 2013; 148: 354).
- Ethmoid sinus atelectasis can cause medial displacement of the globe (J Laryngol Otol 2010;124:206).
In GFS there might be other involutional changes causing eye irritation, such as upper lid horizontal laxity with the rubbery changes of floppy eyelid syndrome.
- Hypertrichosis and increased pigmentation of periocular skin with prostaglandin analogue use
- Periocular change can occur within 3 months of regular use of prostaglandin analogue eye drops.
For GFS, culture of the fornix is helpful; important variants of bacterial colonization such as pseudomonas and methicillin-resistant Staph aureus (OPRS 2012; 28:4) might be recognized.
- Although not indicated to establish the diagnosis of GFS, CT scan might show air collecting between the conjunctiva and the lid (Eye 2006; 20:1481).
- This air collection has led to misdiagnosis, such as after trauma when it can be mistaken for a sign of ocular or orbital penetration (J Emerg Med 2013; 44:e311).
For SBS, imaging shows loss of volume above the orbital roof and superior bowing.
For SSS, imaging shows asymmetry of the orbital floor.
- Orbital imaging might show bilateral bowing of the orbital floors and bilateral shrunken maxillary sinuses in cases of bilateral disease (Ear Nose Throat J 2012; 91:e19).
- VP shunting
- Chronic sinusitis