Ocular Syndromes and Systemic Diseases


Fat Adherence Syndrome 448



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Fat Adherence Syndrome 448

General: Presence of a scar or adhesion that originates in extraconal fat and extends through Tenon capsule to attach to the muscle insertion or sclera; seen following retinal surgery.

Ocular: Persistent acquired restrictive strabismus after retinal surgery; diplopia.

Clinical: None.

Mets MB, et al. Ocular deviation after retinal detachment surgery. Am J Ophthalmol 1985; 99:667-672.

Parks MM. Causes of the adhesive syndrome. Symposium on Strabismus; Transactions of the New Orleans Academy of Ophthalmology. St. Louis: CV Mosby, 1978:269-279.

Wright KW. The fat adherence syndrome and strabismus after retina surgery. Ophthalmology 1986; 93:411-415.



Favre-Racouchot Syndrome (Nodular Elastoidosis) 449

General: Reaction to sun; permanent, slowly progressing condition; occurs in people chronically exposed to sun; usually apparent in fourth or fifth decade.

Ocular: Yellowish thickening of skin, with comedones and follicular cysts in and around orbit.

Clinical: Elastotic degeneration of skin; raised yellow patches and numerous comedones; orifices enlarged; citrine skin; cutis rhomboidalis nuchae; elastoma Dubreuilh.

Favre M, Racouchot JL. Elasteidose Cutanee Nodulaire a Kystes et a Comedons. Ann Dermatol Symp 1951; 78: 681-702.

Ritchie EB, Williams HN. Degenerative collagenous plaques of the hands. Arch Dermatol 1966; 93:202-203.

Feer Syndrome (Swift-Feer Syndrome; Infantile Acrodynia; Acrodynia; Pink Disease) 450

General: Etiology unknown, possibly allergic reaction to mercury or infection; onset in early childhood; both sexes equally affected.

Ocular: Proptosis; lacrimation; pronounced photophobia; severe conjunctival itching; conjunctival injection with occasional marked signs of inflammation; severe keratitis; mild optic neuritis; mydriasis.

Clinical: Restlessness; irritability; continuous profuse sweating; muscle hypotony; tachycardia; exanthema of palms and soles with exfoliation of large skin flaps; stomatitis; sleeplessness; cyanosis of fingers, toes, and nose; loss of teeth; rectal prolapse; muscle hypotonia; hypertension; hypertrichosis; gangrene of fingers.

Duke-Elder S, ed. Systems of Ophthalmology, vol. VIII, part 2. St. Louis: CV Mosby, 1976.

Feer E. Eine Eigenartige Neurose des Vegatativen Systems Beim Kleinkinde, Ergebn Inn Med Kinderh 1923; 24:100.

Felty Syndrome (Chauffard-Still Syndrome; Primary Splenic Neutropenia with Arthritis; Rheumatoid Arthritis with Hypersplenism; Still-Chauffard Syndrome; Uveitis-Rheumatoid Arthritis Syndrome) 451

General: Etiology not fully understood, possibly infection or allergy; onset in middle-aged patients or children; prognosis poor; collagen disorder; occasionally can occur without articular disease.

Ocular: Decreased tear formation; scleromalacia perforans; keratoconjunctivitis; chronic anterior uveitis; scleritis; vitreous opacities; macular edema; choroidal inflammation; papillitis; keratitic precipitates; band-shaped keratopathy.

Clinical: Rheumatoid arthritis; splenomegaly; leukopenia; anemia (mild); oral lesion with ulcers and atrophy.

Crosby WH. What to treat in Felty's syndrome. JAMA 1973; 225:1114.

Davis M. Endogenous uveitis in children: associated band-shaped keratopathy and rheumatoid arthritis. Arch Ophthalmol 1953; 50:443.

Felty AR. Chronic arthritis in the adult associated with splenomegaly and leukopenia. Bull Johns Hopkins Hosp 1924; 35:16.

Pau H. Differential diagnosis of eye disease. New York: Thieme, 1987.

Reddy CV, Foster CS. Adult rheumatoid arthritis. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology, vol. V. Philadelphia: WB Saunders, 1994:2889.



Fetal Alcohol Syndrome 452

General: Dysgenesis in children born to alcoholic mothers; both sexes affected; onset from birth.

Ocular: Antimongoloid slant of lid fissures; lateral displacement of inner canthi; ptosis; epicanthi; strabismus; myopia; optic nerve hypoplasia; diffuse corneal clouding; iridocorneal abnormalities with central corneal edema; lens opacification; motility disorders.

Clinical: Growth retardation; delayed development (physical and intellectual); maxillary hypoplasia; micrognathia; large, low-set ears; abnormal motor function; irritability; microcephaly; cerebral nervous system dysfunctions; abnormal philtrum; flattened nasal bridge; cardiovascular defects; thin upper lip.

Chan T, et al. Ocular manifestations in fetal alcohol syndrome. Br J Ophthalmol 1991; 75:524-526.

Cook CS, et al. Fetal alcohol syndrome. Arch Ophthalmol 1987; 105:1576.

Edward DP, et al. Diffuse corneal clouding in siblings with fetal alcohol syndrome. Am J Ophthalmol 1993; 115: 484-493.

Tenbrinck MS, Buchin SY. Fetal alcohol syndrome. JAMA 1975; 232:1144.

Ulleland CN. The offspring of alcoholic mothers. Ann N Y Acad Sci 1972; 197:167.



Fetal Anticonvulsant Syndrome 453

General: Congenital after maternal ingestion fo sodium valproate, carbamazepine and phenytoin.

Ocular: Myopia; astigmatism; strabismus; anisometropia; optic nerve hypoplasia

Clinical: Craniofacial dysmorphisms vary with type of abnormality

Glover SJ, Quinn AG, Barter P, et al. Ophthalmic findings in fetal anticonvulsant syndrome. Ophthalmology 2002; 109:942-947

Hoyt CS, Billson FA: Maternal anticonvulsants and optic nerve hypoplasia. Br J Ophthalmol 1978; 62: 3-6.

Waller PH, Genstler DE, George CC, et al. Multiple systemic and periocular malformations associated with the fetal hydantoin syndrome. Ann Ophthalmol 1978; 10: 1568-72



Fetal Hydantoin Syndrome 454

General: Syndrome due to an epoxide hydrolase 1, microsomal, arene oxide detoxification defect.

Ocular: Nystagmus.

Clinical: In vitro testing for the defect correlates most highly with congenital heart disease, cleft lip/palate, microcephaly, and major genitourinary, eye, and limb defects; hypersensitivity to phenytoin has occurred.

Buehler BA, et al. Prenatal prediction of risk of the fetal hydantoin syndrome. N Engl J Med 1990; 322:1567-1571.

Gennis MA, et al. Familial occurrence of hypersensitivity to phenytoin. Am J Med 1991; 91:631-634.

McKusick VA. Mendelian Inheritance in Man; A Catalog of Human Genes and Genetic Disorders, 12th ed. Baltimore: The Johns Hopkins University Press, 1998.



Online Mendelian Inheritance in Man, OMIM. McKusick-Nathans Institute for Genetic Medicine, Johns Hopkins University and National Center for Biotechnology Information, National Library of Medicine, February 12, 2007. World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim/.

Fibrinoid Syndrome 455

General: From 2 to 14 days postvitrectomy, white-gray criss-cross layers of fibrin appear on the surface of the retina and immediately behind the plane of the iris; occurs only in patients with diabetes mellitus and usually in those requiring insulin; seen more frequently in people who have been diabetic for 15 years or more.

Ocular: Fibrin material interlaced on the surface of the retina and behind the iris; retinal detachment; neovascular glaucoma; rubeosis irides.

Clinical: Diabetes mellitus.

Ho T, et al. Vitrectomy in the management of diabetic eye disease. Surv Ophthalmol 1992; 37:190-202.

Schepens CL. Clinical and research aspects of subtotal open-sky vitrectomy: 439VII Edward Jackson Memorial Lecture. Am J Ophthalmol 1981; 92:143-171.

Sebestzen JG. Fibrinoid syndrome: a severe complication of vitrectomy surgery in diabetes. Ann Ophthalmol 1982; 14:853-856.



Fibrosarcoma 456

General: Malignant tumor of fibrous connective tissue; most commonly seen in persons 30 to 70 years old; frequent metastases to lung; true fibrosarcoma has a tendency to occur in children (better prognosis); most fibrosarcomas now would be classified as malignant fibrous histiocytomas.

Ocular: Paralysis of extraocular muscles; proptosis; orbital edema; erosion of orbital bony walls; increased intraorbital pressure; metastases to choroid/orbit.

Clinical: Tumors of mesenchymal soft tissues of the extremities, especially in the knee region; progressive pain; edema; tumors of the sinuses; tumors of the lungs.

Henderson JW. Orbital tumors, 2nd ed. New York: Brian C. Decker, 1980:183-188.

Jakobiec FA, Tannenbaum M. The ultrastructure of orbital fibrosarcoma. Am J Ophthalmol 1974; 77:899-9l7.

Roy FH. Fibrosarcoma. In: Fraunfelder FT, Roy FH, eds. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000:337.

Winer JM, et al. Juvenile fibrosarcoma of the orbit and eyelid: a study of 5 cases. Arch Ophthalmol 1983; 101:253.

Fish Odor Syndrome (Trimethylaminuria) 457

General: Metabolic syndrome characterized by a strong body odor of rotting fish; trimethylamine levels elevated; it appears that the enzyme flavin-containing monooxygenase is defective in this disorder; possibly autosomal recessive pattern of inheritance.

Ocular: Hypertelorism, cortical blindness.

Clinical: Hydrocephalus; mental retardation; unusual facies; short stature; skeletal abnormalities; cryptorchidism; hyperextensible skin.

Chen H, Aiello F. Trimethylaminuria in a girl with Prader-Willi syndrome and del(15)(q11q13). Am J Med Genet 1993; 45:335-339.

Humbert JR, et al. The fish odor syndrome. Lancet 1970; 2:770-771.

Shelley ED, Shelley WB. The fish odor syndrome. JAMA 1984; 251:253-256.



Fisher Syndrome (Ophthalmoplegia Ataxia Areflexia Syndrome; Miller-Fisher Syndrome) 458

General: Acute idiopathic polyneuritis; prognosis good; complete recovery over several weeks (variant of Guillain-Barrée Syndrome; see Guillain-Barré Syndrome).

Ocular: Moderate ptosis; complete external and almost complete internal ophthalmoplegia; diplopia; sluggish pupil reaction to light; may present without total ophthalmoplegia.

Clinical: Dizziness; severe ataxia; loss of tendon reflexes; chest pains; difficulties in chewing; diminished or absent sense of vibration; upper respiratory tract infection preceding this syndrome.

Fisher M. An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J Med 1956; 255:57.

Igarashi Y, et al. Fisher's syndrome without total ophthalmoplegia. Ophthalmologica 1992; 205:163-167.

Swick HM. Pseudo internuclear ophthalmoplegia in acute idiopathic polyneuritis (Fisher syndrome). Am J Ophthalmol 1974:77:725.



Fish-Eye Disease (Corneal Opacities-Dyslipoproteinemia) 459

General: Etiology unknown; rare; described in Swedish family; currently considered a unique dyslipoproteinemia.

Ocular: Visual impairment; marked corneal opacities.

Clinical: Very-low-density triglycerides and cholesterol raised.

Carlson LA, Philipson B. Fish-eye disease. A new familial condition with massive corneal opacities and dyslipoproteinemia. Lancet 1979; 2:922-924.

Koster H, et al. A fish-eye disease-like familial condition with massive corneal clouding and dyslipoproteinemia. Report of Clinical, histologic, electron microscopic, and biochemical features. Cornea 1992; 11:452-464.

Magalini SI, Scrascia E. Dictionary of Medical Syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981.



Fleck Retina of Kandori Syndrome (Kandori Syndrome) 460

General: Possibly hereditary; onset young age; focal disturbance of the retinal pigment epithelium (RPE); affects both sexes; toxic causes also considered.

Ocular: Relatively large, irregular, yellowish flecks, sharply border-lined without pigmentation underneath retinal vessels and usually in the midperiphery; poor dark adaptation; normal photopic electroretinographic response; delay in generation of the scotopic response.

Clinical: None.

Bullock JD, Albert DM. Flecked retina. Arch Ophthalmol 1975; 93:26.

Carr RE. Abnormalities of cone and rod function. In: Ryan SJ, ed. Retina, vol. II, 2nd ed. St. Louis: Mosby, 1994: 512-513.

Kandori F. Very rare cases of congenital non-progressive nightblindness with fleck retina. Jpn J Ophthalmol 1959; 13:394.



Floppy Eyelid Syndrome 461

General: Origin unknown; more common in males; overweight; X-chromosome-linked inheritance pattern or possible hormonal influence; has been postulated that the degenerative changes in the tarsus may result from the combination of local pressure-induced lid ischemia and systemic hypoventilation.

Ocular: Easily everted, floppy upper eyelid and papillary conjunctivitis of the upper palpebral conjunctiva; upper eyelid everts during sleep, resulting in irritation, papillary conjunctivitis, and conjunctival keratinization; most distinct feature is rubbery, malleable upper tarsus; keratoconus; punctate keratopathy; blepharoptosis; lash ptosis.

Clinical: Obesity; sleep apnea.

Culbertson WW, Ostler HB. The floppy eyelid syndrome. Am J Ophthalmol 1981; 92:568-575.

Culbertson WW, Tsenc SC. Corneal disorders in floppy eyelid syndrome. Cornea 1994; 13:33-42.

Donnenfeld ED, et al. Keratoconus associated with floppy eyelid syndrome. Ophthalmology 1991; 98:1674-1678.

Goldberg R, et al. Floppy eyelid syndrome. Am J Ophthalmol 1986; 102:376-381.

Katz J, Kaufman HE. Corneal exposure during sleep (nocturnal lagophthalmos). Arch Ophthalmol 1977; 95:449.

Moore MB, et al. Floppy eyelid syndrome. Ophthalmology 1986; 93:184-188.

Netland PA, et al. Histopathologic features of the floppy eyelid syndrome. Involvement of tarsal elastin. Ophthalmology 1994; 101:174-181.



Floppy Iris Syndrome 462

General: Flomax (tamsulosin) is causative agent during cataract. Flomax is commonly prescribed for benign prostate hypertrophy.

Ocular: Floppy iris

Clinical: Benign prostate hypertrophy; noted during cataract surgery

Chang DF, Campbell JR: Intraoperative floppy iris syndrome associated with tamsulosin. J Cataract Refract Surg 2005; 31: 664-73

Gurbaxani A, Packard R: Intracameral phenylephrine to prevent floppy iris syndrome during cataract surgery in patients on tamsulosin

Schrehardt US, Stojkovic M, Hofmann-Rummelt C, et al. the pathogenesis of floppy eyelid syndrome. Ophthalmology 2005; 112: 694-704



Flynn-Aird Syndrome 463

General: May be basic hereditary enzyme deficiency, probably autosomal dominant; no sex predilection apparent.

Ocular: Severe myopia; bilateral cataracts; retinitis pigmentosa; total blindness; onset of visual difficulties in first or second decade of life.

Clinical: Hearing loss; joint stiffness; muscular wasting; kyphoscoliosis.

Flynn P, Aird RB. A neuroectodermal syndrome of dominant inheritance. J Neurol Sci 1965; 2:161-182.

McKusick VA. Mendelian Inheritance in Man; A Catalog of Human Genes and Genetic Disorders, 12th ed. Baltimore: The Johns Hopkins University Press, 1998.

Online Mendelian Inheritance in Man, OMIM. McKusick-Nathans Institute for Genetic Medicine, Johns Hopkins University and National Center for Biotechnology Information, National Library of Medicine, February 12, 2007. World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim/.

Regenbogen LS, Coscas GJ. Oculo-auditory syndromes. New York: Masson, 1985:99-103.



Foix Syndrome (Cavernous Sinus Syndrome; Hypophyseal-Sphenoidal Syndrome; Cavernous Sinus Neuralgia Syndrome; Godtfredsen Syndrome; Cavernous Sinus-Nasopharyngeal Tumor Syndrome; Cavernous Sinus Thrombosis) 464

General: Causes include tumor of lateral sinus wall or sphenoid bone, intracranial aneurysm, cavernous and lateral sinus thrombosis, or lesions; multiple myeloma; may result from infarctions or cancer or be idiopathic.

Ocular: Proptosis; severe ocular and periorbital pain; lid edema; paresis or paralysis of cranial nerves III, IV,V, and VI ; corneal anesthesia; optic atrophy.

Clinical: Postauricular edema; trigeminal neuralgia; deviation of the tongue toward paralyzed side; patients usually have prominent manifestations of sepsis and paranasal sinus; local skin infections are the most common cause.

Foix C. Syndrome de la Paroi Externe du Sinus Caverneux. Rev Neurol 1922; 37/38:827.

Fraunfelder FT, Roy FH. Current Ocular Therapy. 5th ed. Philadelphia: WB Saunders, 2000.

Newman NJ. Third-, fourth, and sixth-nerve lesions and the cavernous sinus. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology, vol. IV. Philadelphia: WB Saunders, 1994:2444.

Rucker CW. The causes of paralysis of the third, fourth and sixth cranial nerves. Am J Ophthalmol 1966; 61:1293.

Tveteras K, et al. Septic cavernous and lateral sinus thrombosis. Modem diagnostic and therapeutic principles. J Laryngol Otol 1988; 102:877.



Folling Syndrome (Phenylketonuria; Phenylpyruvic Oligophrenia; Ikiotia Phenylketonuria Syndrome) 465

General: Rare; autosomal recessive; phenylalanine cannot be converted to tyrosine; poor prognosis without early diet therapy; both sexes affected.

Ocular: Blue sclera; severe photophobia; corneal opacities; cataracts (controversial); partial ocular albinism; macular atrophy.

Clinical: Phenylketonuria; oligophrenia; partial albinism; muscle hypertonicity; hyper-reflexia of tendons; epilepsy; microcephaly; mousy odor of habitus; fair skin.

Dornguth S, et al. Golgi-Kopsch silver study of the brain of a patient with untreated phenylketonuria, seizures, and cortical blindness. Am J Med Genet 1992; 44:443-448.

Foiling A. Uber Ausscheidung von Phenylbrenztraubensaure in den Ham als Stoffwechselanomalie in Verbindung mit Imbezillitat. Ztschr Physiol Chem 1934; 227:169.

Pitt DB, O'Day J. Phenylketonuria does not cause cataracts. Eur J Pediatr 1991; 150:661-664.

Zeligman I, et al. Macular anetoderma, secondary to lichen sclerosus et atrophicus, acanthosis nigricans and apocrine hydrocystoma in a man with phenylketonuria. Birth Defects 1971; 7:256-258.

Foot-in-the-Wound Syndrome 466

General: Occurs when the anterior chamber intraocular lens haptic is within the wound.

Ocular: Haptic in the wound.

Clinical: None.

Hessburg PC. Complications noted in PMMA IOL haptics: fracture, pupillary capture named problems. Ophthal Times August 21, 1983.



Foramen Lacerum Syndrome (Aneurysm of Internal Carotid Artery Syndrome) 467

General: Most commonly caused by congenital aneurysm involving the intradural portion of the carotid artery.

Ocular: Periorbital pain; ptosis; oculomotor paralysis with ptosis, diplopia, and internal ophthalmoplegia; cranial nerves IV and VI may be involved; homonymous hemianopia (occasionally); loss of pupillary reflexes for light and accommodation; papilledema; optic atrophy.

Clinical: Meningism; mental disturbances; unilateral frontal or orbital headache; migraine attacks.

Dailey EJ, et al. Evaluation of ocular signs and symptoms in cerebral aneurysms. Arch Ophthalmol 1964; 71:463.

Geeraets WJ. Ocular Syndromes. 3rd ed. Philadelphia: Lea & Febiger, 1976.

Misra M, et al. Giant aneurysm of internal carotid artery presenting features of retrobulbar neuritis. Ind J Ophthalmol 1992; 39:28-29.



Forsius-Eriksson Syndrome (Aland Disease) 468

General: Associated with the natives of the Aland Islands; sex-linked inheritance; consanguinity versus mutant gene; affects males only; it has been considered a variety of incomplete congenital stationary night blindness.

Ocular: Microphthalmos; irregular latent nystagmus; myopia; astigmatism; dyschromatopsia; tapetoretinal degeneration; primary foveal hypoplasia or dysplasia; nystagmus.

Clinical: Prematurity; impaired hearing; mental retardation; epilepsy.

Forsius H, Eriksson AW, Ein Neues Augensyndrom mit X-chromosomaler Transmission. Eine Sippe mit Fundusal-binismus, Foveahypoplasie, Nystagmus, Myopic, Astigmatismus und Dyschromatopsie. Klin Monatsbl Augenheilkd 1964; 144:447.

McKusick VA. Mendelian Inheritance in Man; A Catalog of Human Genes and Genetic Disorders. 12th ed. Baltimore: The Johns Hopkins University Press, 1998.

Online Mendelian Inheritance in Man, OMIM. McKusick-Nathans Institute for Genetic Medicine, Johns Hopkins University and National Center for Biotechnology Information, National Library of Medicine, February 12, 2007. World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim/.

Vliet AGM, et al. Nystagmographical studies in Aland eye disease. Acta Ophthalmol 1973; 51:782.

Weleber RG, et al. Aland Island disease (Forsius-Eriksson syndrome) associated with contiguous gene syndrome at Xp21: similarity to incomplete congenital stationary night blindness. Arch Ophthalmol 1989; 107:1170.

Foster Kennedy Syndrome (Basal-Frontal Syndrome; Gowers-Paton-Kennedy Syndrome) 469

General: Caused by tumor in base of frontal lobe or sphenoidal meningioma.

Ocular: Central scotoma may be present on side of optic atrophy; enlarged blind spot and peripheral contraction of field (opposite eye); homolateral descending optic atrophy due to compression of the ipsilateral optic nerve at the optic foramen; contralateral papilledema due to increased intracranial pressure; ipsilateral proptosis.

Clinical: Anosmia; headache; dizziness; vomiting; memory loss; psychic changes; also may be caused by an olfactory groove tumor (usually a meningioma) or pituitary adenoma.

Banerjee T, Meagher JN. Foster Kennedy syndrome, aqueductal stenosis and empty sella. Am Surg 1974; 40:552.

Kennedy F. Retrobulbar neuritis as an exact diagnostic sign of certain tumors and abscesses in frontal lobes. Am J Med Sci 1911; 142:355.

Ruben S, et al. Pituitary adenoma presenting as the Foster-Kennedy syndrome. Br J Ophthalmol 1992; 76:117-119.

Yildizhan A. A case of Foster Kennedy syndrome without frontal lobe or anterior cranial fossa involvement. Neurosurg Rev 1992; 15:139-142.

4 a Syndrome (Adrenocortical insufficiency associated with achalasia, alacrima, autonomic and other neurological abnormalities) 470

General: Associated with autonomic and other neurologic abnormalities

Ocular: Alacrima

Clinical: Adreno-coritcal infufficiency; achalsia

Davidoff E, Friedman AH. Congenital alacrima. Surv Ophthalmol 1997; 22: 113-119

Gazarian M, Cowell CT, Bonney M, et al. The 4a syndrome: adrenocortal insufficiency associated with achalasia, alacrima, autonomic and other neurological abnormalities. Eur J Pediat 1995; 154: 18-23.




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