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Hydrophthalmos

Congenital Glaucoma


Presentation

  • Buphthalmos: a Twelve-year Review at Ibadan Abstract Objective: To review the presentation, management, follow-up and outcome of management of children presenting with buphthalmos at the University College Hospital (UCH), Ibadan.[ajol.info]
  • It can be presented at birth or appear in the early childhood. In most cases the buphthalmos affects both eyes, but the effect can be different on both eyeballs.[humanitas.net]
  • Differential diagnosis The differential diagnosis depends on the major presenting symptom.[eyewiki.aao.org]
  • It should be considered a bilateral disease although about one-fourth of patients have only unilateral elevations of pressure even though trabecular abnormalities are present.[disorders.eyes.arizona.edu]
  • Present On Admission POA Help "Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery[icd10data.com]
Hemophilia A
  • It started to develop, albeit slowly, during the first half of the 20th century, mendelian inheritance was studied in a number of important disorders such as albinism, brachydactyly, and hemophilia.[wikivisually.com]
Fishing
  • Fish disease, fish pathology, fish gross pathology, salmon disease, salmon pathology, salmon gross pathology, Atlantic salmon, Salmo salar, eye pathology, gas buble disease, buphthalmos, exophthalmons, panophthalmitis, endophthalmitis.[flickr.com]
Weight Loss
  • Side affects include weight loss, lethargy, and metabolic acidosis. Topical carbonic anhydrase inhibitors include dorzolamide 2% (Trusopt) and brinzolamde 1% (Azopt) drops TID.[eyewiki.aao.org]
Respiratory Distress
  • Side affects include respiratory distress, caused by apnea or bronchospasm, and bradycardia.[eyewiki.aao.org]
Brachydactyly
  • It started to develop, albeit slowly, during the first half of the 20th century, mendelian inheritance was studied in a number of important disorders such as albinism, brachydactyly, and hemophilia.[wikivisually.com]
Cafe-Au-Lait Spots
  • Angle-closure is thought to be the cause of the glaucoma Neurofibromatosis type 1: Characterized by multiple neurofibromas, cafe au lait spots, iris Lisch nodules, and axillary/ inguinal freckling. Inheritance is autosomal dominant.[ncbi.nlm.nih.gov]
Freckles
  • Angle-closure is thought to be the cause of the glaucoma Neurofibromatosis type 1: Characterized by multiple neurofibromas, cafe au lait spots, iris Lisch nodules, and axillary/ inguinal freckling. Inheritance is autosomal dominant.[ncbi.nlm.nih.gov]
Lacrimation
  • Lacrimation c. Oedema of cornea d. Corneal opacity. 4. Buphthalmos is autosomal recessive in inheritance, and associated with neurofibromatosis, sturge weber syndrome. 5. Treatment: Trabeculectomy (Best),goniotomy[gradestack.com]
  • Patients with glaucoma often initially have no symptoms; later, they can exhibit excessive tearing (lacrimation) and extreme sensitivity to light ( photophobia ).[en.wikipedia.org]
  • He performed extensive studies of diseases involving the lacrimal apparatus, and in 1860 described the association of facial naevus flammeus with buphthalmos in a disease now known as encephalotrigeminal angiomatosis.[diki.pl]
  • Daily Visual Balance Check v • d • e Congenital malformations and deformations of eye, ear, face and neck ( Q10-Q18, 743-744 ) Eyes eyelid, lacrimal apparatus and orbit (Ptosis, Ectropion, Entropion, Distichia) Anophthalmia - Microphthalmia lens ( Ectopia[bionity.com]
  • Photophobia, blepharospasm, lacrimation and eye rubbing often occur together. These are thought to be caused by irritation of corneal nerves, which occurs as a result of the elevated IOP.[mdjunction.com]
Kayser-Fleischer Ring
  • Haabs striae KayserFleischer ring Pierre Demours Histology A text and atlas 4. Aqueous humour – The aqueous humour is a transparent, watery fluid similar to plasma, but containing low protein concentrations.[wikivisually.com]
Meningism
  • Sturge-Weber syndrome: Characterized by nevus flammeus of the face and meningeal angiomas. Glaucoma and associated angle anomalies are seen in 60% of cases.[ncbi.nlm.nih.gov]
Lethargy
  • Side affects include weight loss, lethargy, and metabolic acidosis. Topical carbonic anhydrase inhibitors include dorzolamide 2% (Trusopt) and brinzolamde 1% (Azopt) drops TID.[eyewiki.aao.org]
Perseveration
  • After that, mother and child returned to Greenville, and Aretha and he was deeply devoted to his mother and later recalled her perseverance, self-sufficiency, and pride as guiding lights in his life.[wikivisually.com]

Workup

  • Clinical Testing and Workup Various imaging techniques can be used to identify and assess neurological complications including x-rays of the skull (skull radiography) or magnetic resonance imaging (MRI) with gadolinium.[rarediseases.org]

Treatment

  • Treatment Treatment involves surgically opening a hole in the iris to allow flow of aqueous humor, thus relieving the pressure in the posterior chamber of the eye. Early treatment is critical for preserving vision.[wikidoc.org]
  • TREATMENT: Surgical treatment, at the earliest possible time, is essential if vision is to be saved. IMPLICATION: If visual fields are restricted, Orientation and Mobility instruction is indicated.[tsbvi.edu]
  • Treatment Treatment Options: No information regarding treatment is available but cataract and corneal surgery may be beneficial.[disorders.eyes.arizona.edu]
  • Treatment is both medical and surgical 743.2 Excludes Applies To Glaucoma: congenital newborn Hydrophthalmos ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions.[icd9data.com]
  • Treatment / Management The main aim of treatment is to reduce IOP to prevent progressive corneal opacification and glaucomatous optic atrophy and thereby preserve existing vision. The most definitive treatment of Buphthalmos is surgical.[ncbi.nlm.nih.gov]

Prognosis

  • The earlier the defect appears, the less favorable the prognosis. Long-term visual prognosis is fair. Depending on when treatment is instituted, there may be lowered acuity or restricted visual fields.[tsbvi.edu]
  • Prognosis The prognosis for primary congenital glaucoma depends on the age of onset. Glaucoma that presents at birth is more difficult to treat, with at least half of the affected eyes becoming legally blind.[eyewiki.aao.org]
  • Prognosis is usually poor in infants with corneal diameter of more than 16 mm. iii. Tears and breaks in Descemet's membrane (Haab's striae). These occur because Descemet's membrane is less elastic than the corneal stroma.[mdjunction.com]
  • Once considered a condition with a bleak prognosis, this condition can now be managed reasonably well with the modern surgical procedures. Questions To access free multiple choice questions on this topic, click here. References 1.[ncbi.nlm.nih.gov]
  • Prognosis: Goniotomies and trabeculotomies are not always successful.[alpfmedical.info]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • Etiology The etiology of primary congenital glaucoma is not known.[eyewiki.aao.org]
  • Etiology Buphthalmos occurs most frequently due to primary congenital glaucoma. Other conditions which can cause raised IOP in early childhood, for example, Sturge-Weber syndrome, neurofibromatosis, aniridia, e can also cause buphthalmos.[ncbi.nlm.nih.gov]
  • Etiology: (See also physiology and pathophysiology of aqueous humor circulation): The iris inserts anteriorly far in the trabecular meshwork (Fig. 10.2).[alpfmedical.info]
  • Using the existing STS and contig maps of this region ( 23 ), work is currently in progress to search for a candidate gene that may be involved in the etiology of this condition.[academic.oup.com]

Epidemiology

  • Epidemiology It typically manifests in infants and young children. Pathology It usually indicates the presence of congenital (infantile) glaucoma in a majority of cases.[radiopaedia.org]
  • Epidemiology Many studies have put the average prevalence of buphthalmos at 1 in 30,000 births.[ncbi.nlm.nih.gov]
  • EPIDEMIOLOGY Incidence: 1 in 30,000 live births Age -- birth to age 3; more severe with earlier onset Gender -- 65% male Genetics: Majority of cases sporadic Some autosomal recessive Chromosome 1p36 and 2p21 (CYP1B1) SIGNS 75% bilateral Immature angle[webeye.ophth.uiowa.edu]
  • There are no available data on the epidemiology of congenital glaucoma in the Brazilian population (6).[sboportal.org.br]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology Extensive growth of the human eye occurs in the first 5 years of life with the greatest increase in axial length seen in the first 4 years.[ncbi.nlm.nih.gov]
  • Pathophysiology Primary congenital glaucoma occurs from an increase in the intraocular pressure in the first year of life. The increased intraocular pressure is thought to occur from reduced aqueous outflow at the level of the trabecular meshwork.[eyewiki.aao.org]
  • Etiology: (See also physiology and pathophysiology of aqueous humor circulation): The iris inserts anteriorly far in the trabecular meshwork (Fig. 10.2).[alpfmedical.info]
  • Protein reabsorption in renal proximal tubule-function and dysfunction in kidney pathophysiology. Pediatr Nephrol 2004;19:714-21. [ PUBMED ] 4. Lee BH, Lee SH, Choi HJ, Kang HG, Oh SW, Lee DS, et al.[ijnm.in]

Prevention

  • The accumulation prevents the proper development of the eye. It can be presented at birth or appear in the early childhood. In most cases the buphthalmos affects both eyes, but the effect can be different on both eyeballs.[humanitas.net]
  • Treatment:- Management is essentially surgical Goniotomy Trabeculotomy Trabeculectomy Tube shunt implantation Ciliary body ablative procedures Long-term IOP monitoring with visual rehabilitation is a must to prevent Amblyopia. Post navigation[rushabheyehospital.com]
  • CONCLUSIONS: Although incidence of buphthalmos is 1 in 30,000 births2, early pediatric eye exams are critical to prevent further blindness due to the early age of onset1.[aaopt.org]
  • To prevent episcleral fibrosis and subconjunctival scarring thay may result in the surgical failure in filtration surgery, its sporous matrix modulates the migrations and proliferations of fibroblasts to create a vascular and long-lasting bleb without[clinicaltrials.gov]
  • Primary prevention There is no known way to prevent primary congenital glaucoma. Early detection and treatment are essential to maximize visual potential.[eyewiki.aao.org]

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