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Congenital Ectropion Uveae

Isolated Congenital Ectropion


Presentation

  • Abstract Congenital ectropion uveae is a rare condition which may be present in one or both eyes. If the patient is followed glaucoma will always be found to be present.[ncbi.nlm.nih.gov]
  • Glaucoma is present in most of the cases reported.[nature.com]
Amyloidosis
  • Archives of Ophthalmology 121(10): 1511-1511, 2003 A case of primary congenital glaucoma with unilateral corneal amyloidosis . Nippon Ganka Gakkai Zasshi 107(11): 724-730, 2003[eurekamag.com]
Lesion of the Tongue
  • Relationship of familial prominent corneal nerves and lesions of the tongue resembling neuromas to multiple endocrine neoplasia Type IIB. Am J Ophthalmol 1995;120:456-461. 10. Spector B, Klintworth GK, Wells SA.[healio.com]
Cafe-Au-Lait Spots
  • au lait spots (skin), skeletal changes Opthalmic features: Anterior segment: eyelid neurofibromas, iris lisch nodules, congenital ectropion uvea, mammilations (nodules on iris surface), prominent corneal nerves Posterior segment: optic nerve glioma,[quizlet.com]
  • There were two cafe au lait spots on the trunk but there was no other systemic abnormality. None of the family members had similar ocular findings or any evidence of neurofibromatosis.[bjo.bmj.com]
Blister
  • (nonthermal) of right eyelid and periocular area, initial encounter S00.221D Blister (nonthermal) of right eyelid and periocular area, subsequent encounter S00.222A Blister (nonthermal) of left eyelid and periocular area, initial encounter S00.222D Blister[hmsa.com]
  • Zoster Ophthalmicus • Elderly patient in 60s and 70s • Immunocompromised • Skin rashes respecting the midline and dermatomal distribution • Eyelid scarring Ocular Cictricial Pemphigoid • Forniceal scarring • Symblepharon • Eyelid scarring • Oral mucosal blisters[reviewofophthalmology.com]
Facial Hemihypertrophy
  • Three patients had neurofibromatosis, two had facial hemihypertrophy, one had Rieger's anomaly, one had Prader-Willi syndrome, and one had no systemic anomalies.[ncbi.nlm.nih.gov]
  • […] glaucoma in this primary iris pigment epithelial hyperplasia, which may present at birth, infancy, or later stages in life has been well documented in the literature. 1 , 2 , 3 , 4 , 5 , 6 Systemic associations often reported are neurofibromatosis, facial[nature.com]
  • Neurofibromatosis, Prader-Willi syndrome, Rieger anomaly, and facial hemihypertrophy were found to occur concomitantly with CEU, suggesting the possibility of a neural crest cell disorder. Seven of 8 patients developed glaucoma.[jamanetwork.com]

Workup

  • Methods: : Patients with congenital eyelid anomalies presented during one year period (Dec 2006 - Nov 2007) with complete clinical workup were included. Karyotyping from blood was done in all the patients.[iovs.arvojournals.org]
  • References Laboratory Studies Aniridia: Chromosome analysis and genetic counseling are important parts of the workup. Axenfeld-Rieger syndrome: Patients may need workup for associated systemic abnormalities.[misc.medscape.com]
  • If a patient is found to have a cicatricial entropion, an infection or autoimmune disease workup needs to be performed before treatment of the entropion. The source of the entropion needs to be identified.[statpearls.com]
  • Further workup including neuroimaging, cocaine testing, and evaluation for neuroblastoma should be considered with any other neurologic or associated ocular findings.[doctorlib.info]

Treatment

  • We report an unusual case of unilateral congenital ectropion uveae in a 3-year-old child, with no evidence of neurofibromatosis, presenting as acute glaucoma, which was successfully managed by topical treatment only, avoiding surgical intervention.[ncbi.nlm.nih.gov]
  • Patients were evaluated for response to medical treatment, course of disease and dev[urbanedge.co.in]
  • Recent dramatic advances in diagnosis, as well as medical and surgical treatment, mean that you can offer your glaucoma patients more timely and effective interventions.[books.google.com]
  • The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]

Prognosis

  • They are usually interventions with good prognosis, and are usually performed under local anaesthesia, except in the case of children, which are operated under general anaesthesia.[barraquer.com]
  • Moer common in blue eyes than brown Rare in African Americans Males females Iris melanoma - prognosis May invade anterior chamber angle or ciliary body Overall prognosis: good Very small chance of metastases (5% within 10 years of treatment) Very slow[brainscape.com]
  • Expectations (prognosis) The outlook is usually good if the condition is treated before cornea damage occurs.[pittsburgheyeassociates.com]
  • Prognosis : good. Presenting signs Epiphora Eye: epiphora. Mucopurulent ocular discharge Eye: ocular discharge - overview when concurrent ocular infection is present. Periocular crusting associated with ocular discharge. Periocular irritation.[vetstream.com]
  • However she didn't have much if any information or a prognosis. Just that my baby needs to be seen monthly until something develops.[medhelp.org]

Etiology

  • Involutional changes are the most common etiology of entropion. As we age, the canthal tendons relax, and the eyelid retractors attenuate, causing misposition of the eyelid margin [1].[statpearls.com]
  • According to its etiology, ectropion is classified into 2 groups: acquired and congenital.[jamanetwork.com]
  • Definition tissue cleft or defect lined by pupillary ruff; no impact on VA/accomodation Concern: may lead to lens subluxation Tx: none, use CLs later in life Etiology: caused by incomplete fusion of the fetal fissure during gestation, inferior nasal,[flashcardmachine.com]
  • The exact etiology of developmental glaucoma with CEU is unknown.[healio.com]
  • Original Article Abstract Author Information Authors Based on the known neurocristopathic etiology of type 1 neurofibromatosis (NF1) and the neuroectodermal embryologic derivation of the iridocorneal angle , we examined a sample of young patients affected[journals.lww.com]

Epidemiology

  • Epidemiology Mortality/Morbidity The primary morbidity is ocular surface irritation. Corneal abrasions and scars can occur. Sex Entropion has no sexual predilection. Age Although all ages can be affected, entropion is seen primarily in older adults.[emedicine.medscape.com]
  • Congenital ectropion uvea is a rare disorder, and epidemiological data on its incidence are scarce. Although bilateral cases have been reported, the disease is usually unilateral and non-progressive.[panafrican-med-journal.com]
  • Congenital EU (CEU) is a rare disorder, and epidemiological data on incidence are scarce. Although bilateral cases have been reported, the disease is usually unilateral. In addition, it tends to be nonprogressive.[aao.org]
  • Epidemiology The older an individual is, the greater the chances of developing an entropion [1]. Bilateral disease is three times more common than unilateral.[statpearls.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology The pathophysiology depends on the type of entropion seen and is discussed below. Epidemiology Mortality/Morbidity The primary morbidity is ocular surface irritation. Corneal abrasions and scars can occur.[emedicine.medscape.com]
  • Offers an in-depth and thorough approach to the pathophysiology of oculoplastics and orbital disease, incorporating the perspectives of numerous specialties – all in one convenient volume.[books.google.ro]
  • Although it is not a permanent solution and does not address the pathophysiologic contribution of horizontal lid laxity, it is a great temporizing solution for a problem presenting significant irritation to a patient.[healio.com]
  • The progression of keratoglobus is unpredictable, mainly because of our limited understanding of the pathophysiology of the condition. Keratoglobus is associated with impaired vision, but not blindness.[lecturio.com]

Prevention

  • Prevent and plan for complications in advance by examining over 1,600 illustrations, photos and graphics (1,250 in color) capturing essential diagnostics techniques, imaging methods and surgical approaches.[books.google.com]
  • […] points to a neural crest cell theory of anterior segment dysgenesis. 8 Although CEU is a benign ocular abnormality, a high degree of caution should be exercised so as to detect associated glaucoma (which may be early or late) as early as possible to prevent[nature.com]
  • […] pupil block angle closure glaucoma -put hole to ensure proper aqueous flow -Rarely seen today (most surgeries extracapsular) Laser Peripheral Iridotomy Surgical treatment of glaucoma (YAG) -allows flow of aqueous to ant. chamber Used to prevent angle[quizlet.com]
  • Most cases are not preventable.[pittsburgheyeassociates.com]

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