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We describe four members spanning three generations of a Caucasian family affected with distal arthrogryposis (DA). Based on Hall's original classification, we have placed our family under type IIB and present previously unreported ophthalmic features. All the members had different degrees of ophthalmoplegia, ptosis, astigmatism, and strabismus. Other findings in affected family members included keratoconus in the index patient, which was associated with abnormal electron microscopy of the affected cornea and increased thickness of the central cornea, small axial length of the globe and choroidal folds in the others.
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PMID:Distal arthrogryposis type IIB: unreported ophthalmic findings. 1627 94

Corneal disease is common in equine ophthalmology and requires vigilant monitoring and appropriate therapy to optimize the outcome. Many equine corneal diseases, particularly those that progress rapidly, may benefit from surgical intervention. These include descemetoceles, deep corneal lacerations and ulcers, corneal perforation/iris prolapse, ulcerative keratitis, corneal stromal abscesses, and corneoscleral neoplasia. Indications for corneal transplantation include optical, tectonic, therapeutic, and cosmetic purposes. Corneal transplantation is most often implemented in equine patients for tectonic and therapeutic reasons when a cornea is compromised by corneal stromal abscess, iris prolapse, or neoplasia. This article provides an outline of when to consider surgical intervention for corneal disease, the procedures available and expected outcomes, and how appropriate early surgical intervention can dramatically improve the end result.
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PMID:Equine corneal surgery and transplantation. 1527 28

Abnormalities in eyelid position and function can lead to chronic irritation of cornea and conjunctiva. Corneal ulceration, vascularization and pigmentation are common consequences. Loss of vision, or loss of the globe may be the end result. The combination of ptosis and entropion of the upper eyelid is commonly seen in breeds with abundant forehead skin folds. Treatment options include surgical reduction of skin folds, suspending the brows, enforced secondary granulation of the upper eyelid, or a combination of procedures. Lid-shortening procedures reduce corneal exposure, improve functional blinking and reduce likelihood of traumatic proptosis. Although a lateral canthoplasty is technically easier to perform, a medial canthoplasty is the preferred method of eyelid shortening. An involuted lateral canthus with entropion is seen in certain dog breeds with a broad skull base and redundant facial skin. Surgical correction is by either applying traction to the lateral canthus, or by resection of the lateral canthal ligament. A combination of entropion, ectropion and macroblepharon may result in the presence of diamond shaped eyelids. Surgical correction involves the combination of several procedures. Upper eyelid agenesis is a congenital absence of part of the upper eyelid in cats. Treatment options include a skin-orbicularis flap from the lower eyelid with a conjunctival pedical graft, the transposition of the lower eyelid to the upper eyelid, cross lid flap and subdermal plexus flap, and subdermal collagen injection. Several techniques for reconstruction of upper and lower eyelid defects are described.
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PMID:Adnexal surgery in dogs and cats. 1531 Feb 87

Long-term results achieved by our own operative technique in children with congenital dystrophic ptosis, with frontal muscle lobe shaping with or without shaping of corrugator muscle lobe attached to the tarsal plate, are presented. Data on 146 patients with congenital dystrophic ptosis operated on during the 1984-1998 period at Zagreb University Hospital Center were retrospectively analyzed. Postoperative success was defined as a situation with eyes open in which 1) upper eyelid covers the cornea at 12 o'clock position by 1-2 mm; 2) there is a good contour of the eyelid margin; 3) there is no lagophthalmos; and 4) there is symmetry with the other eye. Immediate re-operation due to undercorrection was required in 26 of 146 (18%) patients. Upon re-operation, 133 (91%) patients met the criteria for successful outcome at 6 months, 124 (85%) at one year, and 121 (83%) at 5 years. Correction of congenital dystrophic ptosis using a shaped frontal/corrugator lobe is an efficient and safe procedure ensuring long-lasting success.
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PMID:Long-term results of frontal lobe suspension in children with congenital dystrophic ptosis. 1563 94

Aniridia consists in a congenital absence of the iris, with incidence varying from 1/64,000 to 1/96,000. This complex embryologic malformation involves the iris, trabecula, and cornea with limbal stem cell deficiency. Aniridia is a genetic haplo-insufficiency expression of the PAX6 gene located on chromosome 11p13. The associated clinical ocular signs could be congenital cataract, congenital glaucoma (the most common complication), keratopathy, ptosis, nystagmus, foveal aplasia, or microphthalmia. More than half of aniridic patients will develop glaucoma, so a regular complete tensional check-up is recommended. Central pachymetry is thicker than in the general population and overestimates ocular pressure if a corrective coefficient is not used. When glaucoma is diagnosed, medical or surgical treatment should be adapted to the child's age. In younger children, trabeculotomy is preferable to trabeculectomy in the first step if the cornea is clear enough. In older children or teenagers, a classical medical treatment must be initiated first, but surgical treatment will often be needed later. Trabeculectomy is the surgical procedure to choose for these older patients or can be combined with cataract surgery in adults. In severe glaucoma, after failure of trabeculectomy with or without mitomycin C, glaucoma surgery with drainage placement could be necessary to control ocular pressure and preserve vision. The outcome of glaucoma in aniridia is always severe and requires medical and surgical treatment adapted from infancy to adulthood.
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PMID:[Glaucoma in aniridia]. 1731 8

Chlamydia conjuctivitis results from infection by chlamydia trachomatis, the commonest treatable sexually transmitted infection in Europe. Its clinical manifestations involve the conjunctiva and the cornea. The inflammation under the upper eyelid may be sufficient to present as ptosis, however previously it has not been documented to cause a preseptal cellulitis. We present such a case. A 15-year-old girl was diagnosed with a left viral conjunctivitis. Five days later, she returned with marked oedema of the left upper and lower lids accompanied by erythema. The tarsal conjunctiva revealed follicles and large papillae and extra ocular movements revealed discomfort on elevation. A secondary diagnosis of bacterial pre septal cellulitis was made and the treatment was changed a broad spectrum oral antibiotic. On review at two days, the patient now complained of a large amount of purulent discharge in association with the marked pre septal swelling. As previous bacteriology and virology had been negative, the patient was re swabbed for chlamydia. This proved positive and her symptoms completely resolved following administration of Azithromycin. In this particular case recognition of the pathogen is important to alert the patient to the likelihood of unknown genital infestation. In all cases of positive culture, the patient should be counselled to attend a genitourinary clinic and to alert any sexual partners to the need to do likewise.
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PMID:Chlamydial conjunctivitis presenting as pre septal cellulitis. 1735 43

Patients with upper lid paralysis suffer from a loss of the blink reflex/response in the affected eye, leaving the eye vulnerable to a host of predatory insults. Partial or total impairment of the orbicularis oculi muscle, lagophthalmos, disruption of the lacrimal apparatus, upper lid retraction, and the unopposed pull of gravity on the surrounding paralyzed tissues all contribute to increased corneal exposure and an increased risk of exposure keratitis. Management of the upper lid in these patients must therefore focus on restoration of the effects of the blink reflex/response and prevention of corneal exposure. Relevant anatomy and pathophysiology are discussed. The initial treatment is supportive, with surgery reserved for those patients that fall into two categories: those who have failed nonsurgical treatment to protect the cornea and those who have been treated effectively with conservative measures but are faced with the prospect of long-term or permanent paralysis. A variety of surgical procedures that may be classified as either static or dynamic are discussed. Standard static procedures include lid loading and tarsorrhaphy, whereas the palpebral spring implant and the temporalis muscle transfer are classified as dynamic. The goal of the corrective procedures is to allow complete eye closure, thereby providing corneal protection, with minimal (1 mm or less) ptosis in the open position.
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PMID:The evaluation and treatment of upper eyelid paralysis. 1847 Aug 35

A 70-year-old man had undergone a complicated cataract extraction followed by repair of iris prolapse ending up with secondary glaucoma. Refusing routine filtering surgery, patient underwent contact transconjunctival diode laser cyclophotocoagulation. This report highlights the occurrence of corneal edema restricted to the inferior half of cornea following a diode laser cyclophotocoagulation performed in the inferior 180 degrees. The necessity to perform a routine specular microscopy in all such cases is highlighted, especially in eyes with good visual acuity.
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PMID:Corneal edema following diode laser cyclophotocoagulation in an eye with secondary glaucoma. 1857 92

The author considered the following important points:(1) Anterior capsular synechia to a corneal incision (made by a keratome) after the evacuation of a traumatic cataract. This might be detached early by the use of a blunt-ended knife following a perforation of the cornea with a sharp-pointed knife, much like a tenotome.(2) The involuntary prolapse of capsule with a cataract incision.(a) The danger of this was demonstrated as the cause of glaucoma, especially if it be found necessary to divide opaque capsular membrane after the extraction.(b) The danger of sympathetic ophthalmia.Prolapse might be prevented:(a) By intracapsular extraction.(b) By extracting the lens through an intact pupil, after the use of capsule forceps, followed either by a partial or total iridectomy.(3) The treatment of opaque after-cataract.Various types of opaque capsule membrane were described.(a) Opaque lens fibres imprisoned between anterior and posterior remains of capsule.(b) Grey membrane made of new lens fibres from proliferating subcapsular cells.(c) Elschnig's cells.(d) Much thickened capsular membrane following an extensive haemorrhage into the anterior chamber occurring about the fifth day after extraction.(e) A thick membrane formed of fibrous tissue following the invasion of the coloboma of the iris after infection at the time of operation. The fibrous tissue comes from the undersurface of the conjunctival flap and causes an updrawn coloboma which is also made narrower by its contraction.When performing a capsulotomy thickened bands should be avoided and an incision made in thin capsule, parallel to thick bands.If the membrane is very thick and shows signs of being torn from its peripheral attachment when a single needle is used, then(1) Two needles may be used after the method of Bowman;(2) A Wheeler operation may be performed (Wheeler, 1939, Collected Papers, New York, 197);(3) Thick capsule may be divided by means of a Ziegler knife, as described by the author, but not in the manner described by Ziegler.The danger of performing a capsulotomy in the presence of soft lens matter was pointed out.The occasional occurrence of localized vitreous opacification at the site of a capsulotomy, even in the absence of iridocyclitis, was mentioned.
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PMID:The capsular complications of cataract extraction. 1886 98

Ectropion is the most frequent eyelid malposition, characterized by the eversion of the margin and exposure of the conjunctiva and the cornea. It is classified as congenital (primary and secondary) and acquired (involutional, paralytic, mechanical and cicatricial). Congenital ectropion is rare and it is usually associated with other malformations such as euryblepharon, ptosis, epicanthus inversus, and blepharophimosis syndrome. Involutional ectropion is the most frequent form of acquired eyelid eversion and a result of multiple factors. The mechanisms involved in its pathogenesis are discussed in this review. Cicatricial ectropion is caused by the shortening of the anterior lamella of the lid, secondary to congenital malformations, trauma, burns, skin conditions, scarring skin tumors, medications, allergies, blepharoplasty complications, and involutional changes that result in loss of skin elasticity. Mechanical ectropion is caused by eyelid tumors or inflammatory disorders that cause orbicularis spasm. The evaluation of the patient, selection of treatment and surgical techniques are described in detail.
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PMID:Ectropion. 2059 Apr 14


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