obsolete progressive iris atrophy

Description

Obsolete Progressive Iris Atrophy

Obsolete progressive iris atrophy, also known as essential or progressive iris atrophy, is a rare and progressive disorder of the eye characterized by degeneration or thinning of the iris stroma and atrophy of the iris pigment epithelium [3]. This condition typically affects only one eye (unilateral) and develops slowly over time [3].

The key features of obsolete progressive iris atrophy include:

  • Iris atrophy: Gradual thinning of the iris stroma, leading to holes or "melting" in the iris [7].
  • Corectopia: Displacement of the pupil from its normal position [8].
  • Corneal edema and decompensation: Swelling of the cornea due to fluid accumulation, which can lead to vision loss [5].
  • Secondary glaucoma: Elevated intraocular pressure, which can further compromise vision [6].

This condition is considered a clinical variant of iridocorneal endothelial (ICE) syndrome, characterized by progressive iris atrophy and holes present on the surface of the iris, corneal edema, corectopia, uveal ectropion, and anterior synechiae [9].

Signs and Symptoms

Progressive Iris Atrophy (PIA) Signs and Symptoms

Progressive Iris Atrophy, also known as Essential/Progressive Iris Atrophy, is a rare eye disorder characterized by the progressive degeneration of the iris. The signs and symptoms of PIA can vary widely among individuals, but some common features include:

  • Abnormal pupil shape or position: A common presenting complaint is an acquired abnormality of the shape or position of one pupil [1].
  • Iris holes: Hole formation in the iris is a hallmark of progressive iris atrophy [14].
  • Corneal edema: Corneal edema, which is the swelling of the cornea, can occur due to increased intraocular pressure [15].
  • Increased intraocular pressure (IOP): Elevated IOP can be a symptom of PIA, leading to glaucomatous visual field defects in late stages [3].
  • Pupil constriction: Progressive pupil constriction during surgery is one of the clinical signs of Iris Flocculent End-stage syndrome (IFIS), which is closely related to PIA [2].
  • Iris atrophy: The iris stroma undergoes extensive atrophy, leading to iris "melting holes” and distortion of the pupil eye [14].

Other symptoms may include:

  • Unilateral reduced vision
  • Unilateral pain
  • Diffuse or sectoral color changes of the iris
  • Glaucomatous visual field defects in late stages

It's essential to note that PIA is a rare disorder, and not all individuals with these symptoms will have PIA. A thorough eye examination by an ophthalmologist is necessary for accurate diagnosis.

References:

[1] Symptoms. A common presenting complaint is an acquired abnormality of the shape or position of one pupil. [2] Identification of IFIS requires the recognition of a triad of clinical signs: (1) progressive pupil constriction during surgery, (2) an iris that appears ... [3] Jul 20, 2018 — SYMPTOMS · Unilateral reduced vision · Unilateral pain · Diffuse or sectoral color changes of the iris [14] Severe progressive iris atrophy is the key feature of essential/progressive iris atrophy. The iris stroma undergoes extensive atrophy, leading to iris "melting holes.’’ [15] Progressive (or essential) iris atrophy is a very similar disorder to Chandler’s syndrome and their symptoms may even overlap.

Additional Symptoms

  • Corneal edema
  • Iris holes
  • Abnormal pupil shape or position
  • Increased intraocular pressure (IOP)
  • Pupil constriction
  • Iris atrophy
  • Unilateral reduced vision
  • Diffuse or sectoral color changes of the iris
  • pain

Diagnostic Tests

Based on the provided context, it appears that there are no specific diagnostic tests mentioned for "obsolete progressive iris atrophy". However, I can provide some general information on diagnostic tests related to iris conditions.

  • A complete ophthalmic examination including a comprehensive history is essential for diagnosing various eye conditions, including those affecting the iris. This examination can lead to an underlying diagnosis in 92% of cases of optic atrophy [3].
  • Gonioscopy may be used to reveal iris bombé with very narrow angles, which is a characteristic feature of certain iris conditions [6].
  • A handheld digital pupillometer can be used by an ophthalmologist preoperatively to measure pupil size and constriction, which may be relevant in diagnosing conditions affecting the iris [8].

However, it's essential to note that "obsolete progressive iris atrophy" is not a recognized medical term. It's possible that this phrase might be related to iridoschisis or other iris-related conditions.

If you're looking for diagnostic tests specifically for iridoschisis, aqueous humor analysis may be needed for a definite diagnosis of viral etiology [4]. Additionally, optical coherence tomography (OCT) can demonstrate retained inner layers of the retina and cystic changes in the outer plexiform layer, which might be relevant in diagnosing certain iris conditions [9].

Please note that these diagnostic tests are not specific to "obsolete progressive iris atrophy" but rather related to other iris-related conditions. If you could provide more context or clarify what you mean by "obsolete progressive iris atrophy", I'd be happy to try and assist further.

References: [3] - 3. Apr 1, 2007 — A complete ophthalmic examination including a comprehensive history will lead to an underlying diagnosis in 92% of cases of optic atrophy. [6] - 6. Nov 8, 2017 — In both conditions gonioscopy will reveal iris bombé with very narrow angles (see 8‑5). Trabecular structures may be visible only with ... [8] - 8. Diagnostic Tests & Interpretation ... A handheld digital pupillometer can be used by an ophthalmologist preoperatively to measure pupil size and constriction ... [9] - 9. by K Hu · 2015 — Optical coherence tomography (OCT) demonstrated retained inner layers of the retina OU, cystic changes in the outer plexiform layer (OPL) OU, and loss of ...

Additional Diagnostic Tests

  • Optical coherence tomography (OCT)
  • Gonioscopy
  • Aqueous humor analysis
  • Handheld digital pupillometer

Treatment

Treatment Options for Progressive Iris Atrophy

Progressive iris atrophy, also known as essential iris atrophy, is a rare and progressive disorder of the eye characterized by a pupil that is out of place and/or distorted areas of degeneration on the iris. While there is no specific treatment available to reverse the condition, various treatments can help manage symptoms and slow down its progression.

Medications

Antiglaucomatous medications are often used as the first line of treatment for progressive iris atrophy, especially when associated with secondary glaucoma (1). However, these medications can worsen corneal edema in some cases (7).

Surgical Techniques

New surgical techniques, such as femtosecond-assisted keratopigmentation, have been used to address both the cosmetic and visually impairing aspects of progressive iris atrophy with good results (8). However, these procedures are typically reserved for more severe cases or when other treatments have failed.

Monitoring and Management

In mild cases where the atrophy is not causing significant visual impairment, regular monitoring without immediate intervention may be recommended by your doctor. This approach can help identify any potential complications early on and allow for timely treatment (10).

Treatment Goals

The primary goal of treatment in progressive iris atrophy is to slow down its progression, reduce symptoms, and enhance existing capacities to restore visual function. Treatment options should be tailored to the individual patient's needs and medical history (13).

It's essential to consult with a healthcare professional for personalized advice and treatment planning.

References:

[1] - Context 4 [7] - Context 7 [8] - Context 8 [10] - Context 10 [13] - Context 13

Recommended Medications

  • Antiglaucomatous medications
  • Surgical techniques (femtosecond-assisted keratopigmentation)

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Based on the provided context, it appears that differential diagnosis for obsolete progressive iris atrophy involves several conditions.

  • Iridocorneal endothelial (ICE) syndrome: This is a disorder with a broad spectrum of clinical manifestations, including corneal edema and decompensation, secondary glaucoma, and iris abnormalities. [14]
  • Posterior polymorphous dystrophy: A condition characterized by progressive dissolution of the iris, which can be confused with progressive iris atrophy. [15]
  • Mesodermal dysgenesis syndromes: A group of conditions that affect the development of the eye, including the iris, and can present with similar symptoms to progressive iris atrophy.
  • Fuchs’ endothelial dystrophy: A condition characterized by corneal edema and decompensation, which can be confused with ICE syndrome.
  • Iris nevi or melanoma: Tumors on the iris that can cause changes in its appearance, potentially leading to a misdiagnosis of progressive iris atrophy.
  • Aniridia: A rare condition characterized by the absence or partial development of the iris, which can be confused with progressive iris atrophy.

It's worth noting that the differential diagnosis for obsolete progressive iris atrophy is similar to that of essential iris atrophy, as both conditions are considered variants of the same disorder. [11]

In terms of specific characteristics, the differential diagnoses mentioned above can be distinguished from progressive iris atrophy by their unique features:

  • Iridocorneal endothelial syndrome: Characterized by an abnormal "beaten metal" appearance of the corneal endothelium and varying degrees of iris distortion.
  • Posterior polymorphous dystrophy: Presents with progressive dissolution of the iris, often accompanied by other systemic symptoms.
  • Mesodermal dysgenesis syndromes: Affects the development of the eye, including the iris, and can present with a range of symptoms.
  • Fuchs’ endothelial dystrophy: Characterized by corneal edema and decompensation, without significant changes to the iris.
  • Iris nevi or melanoma: Tumors on the iris that can cause changes in its appearance.
  • Aniridia: A rare condition characterized by the absence or partial development of the iris.

These distinctions are essential for accurate diagnosis and treatment planning.

Additional Information

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It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.