Тема: Index of Suspicion
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Старый 15.08.2005, 11:38
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Case2

Case2
Presentation

A 3-year-old boy is seen in the ED because of a "bloodshot eye." Several weeks ago he was hit in the eye with a ball. Two days ago his eye became red but has not bothered him. He has had no ocular discharge, fever, or respiratory symptoms. He comes from a disrupted family complicated by parental drug use and has siblings in foster care. He has not received routine care, although he was seen regularly in infancy and is fully immunized.

Physical examination reveals an active, well-nourished toddler who has conjunctival injection and a blood layer in the anterior chamber of his right eye (hyphema). There appears to be no vision in his affected eye, and a red reflex cannot be obtained. There are no signs of facial trauma, strabismus, or pigmented yellow lesions around the eye. Examination of the skin shows no petechiae or bruising except on the anterior shins. The child is admitted for management of hyphema.

Laboratory findings include a WBC count of 8.5x103/mcL (8.5x109/L), Hgb concentration of 12 g/dL (120 g/L), platelet count of 275x103/mcL (275x109/L), international normalized ratio of 1, and partial thromboplastin time of 41 seconds. A skeletal survey shows no fractures. Imaging confirms the cause of the hyphema.
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Case 2 Discussion

Further history revealed that the child’s right eye has glowed like a "cat’s eye" when viewed from across the room since he was 24 months old. CT scan revealed a calcified mass within the right globe and no abnormalities of the left globe. The findings of a calcified mass within the globe on CT scan were consistent with retinoblastoma.

The Condition
Hyphema in children most commonly occurs after blunt or lacerating trauma, but the differential diagnosis is broad. Spontaneous hyphema can arise from juvenile xanthogranuloma, leukemia, hemophilia, thrombocytopenia, aspirin or warfarin ingestion, and intraocular tumors such as retinoblastoma.

Although hyphema in children usually resolves without sequelae, complications can ensue and include increased intraocular pressure, optic atrophy, synechiae between the iris and the lens, and corneal blood staining. Because complications are more common with rebleeding, initial management is aimed at preventing further bleeding. Placement of a hard metal shield to protect the eye (Fox shield) is an important preventive step. Bed rest or quiet ambulation should be instituted, along with elevation of the head of the bed. Admission should be strongly considered for all children, especially if there is any question of compliance. Because patients who have sickle cell hemoglobinopathy are at higher risk for complications, Hgb electrophoresis should be obtained if the diagnosis is in question.

Ophthalmologic consultation is required for any child presenting with hyphema to prevent late sequelae. The ophthalmologist administers medicines that lower intraocular pressure, decrease inflammation, and prevent synechiae. Surgical intervention is considered if intraocular pressure cannot be managed with maximal medical therapy or if corneal blood staining occurs.

The Underlying Condition
Retinoblastoma, although rare, is the most common malignant intraocular tumor in children (incidence of 1 in 17,000) and must be considered in any case of spontaneous hyphema. The tumor typically is diagnosed in the first years after birth. The initial sign usually is leukocoria, and family members often describe a glow or cat’s eye appearance. Retinoblastoma also can present with strabismus, hyphema, and periocular inflammation resembling orbital cellulitis. The disease can be familial or sporadic and can present unilaterally or bilaterally. Bilateral disease almost always is familial. A mutation of the retinoblastoma gene (a tumor suppressor gene) on chromosome 13 is responsible for tumor development. Metastasis is uncommon. Spread can occur by direct extension along the optic nerve into the brain or the subarachnoid space or through the bloodstream to bone, bone marrow, lung, and lymph nodes. When retinoblastoma is diagnosed, both parents and all siblings should be examined for retinoblastoma.

The management of retinoblastoma involves a multidisciplinary team using multiple modalities that prioritize tumor eradication. Careful and judicious attempts should be made to preserve vision. Treatments include chemotherapy, external beam radiation, laser photocoagulation, enucleation, or combinations of these approaches. Although enucleation remains the most common treatment for unilateral disease, the trend in more recent years has been toward less frequent use of this approach, probably because of the more effective use of other approaches such as radiation, chemotherapy, and laser therapy. The prognosis for patients who have unilateral retinoblastoma without optic nerve invasion or metastasis is excellent, with more than 90% survival among those using current therapy. However, if tumor is disseminated, the mortality increases substantially. Surgical washout, which sometimes is performed for bleeding in the eye, carries the potential for dissemination, which could have proved a lethal choice for this child.

Lessons for the Clinician
The possibility of retinoblastoma should be entertained for any child who develops a spontaneous hyphema. In this case, the child’s complicated social situation might have led the clinician to suspect nonaccidental trauma as the cause of the child’s hyphema, delaying the diagnosis. When the retina cannot be visualized, it is imperative to obtain an imaging study to rule out disease in the posterior pole.


Carrie Phillipi, MD, PhD
Laurie Christensen, MD
John Samples, MD
Oregon Health and Science University, Portland, Or
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