Posterior chamber intraocular lens implantation in a patient with oculocutaneous albinism.

A 56-year-old woman with tyrosinase-negative oculocutaneous albinism complained of gradually decreasing vision in the left eye. Her visual acuity was 20/300 in the right eye and hand motion in the left eye. She had pink skin and white hair and cilia. On examination, bilateral hypopigmented irides, an opaque lens in the left eye, a hypopigmented fundus in the right eye, and nystagmus were found. An extra-capsular cataract extraction with posterior chamber intraocular lens (IOL) implantation was performed. After retrobulbar anesthesia was administered, the nystagmus ceased temporarily. Postoperatively, visual acuity in the left eye improved to 20/200. We believe IOL implantation is useful in the treatment of cataracts in albino patients.     

Radiotherapy resolves leukemic involvement of the optic nerves.

A 40-year-old woman with acute lymphoblastic leukemia had a visual disturbance OD. The optic disc was slightly swollen in the right fundus, and blast cells in the cerebrospinal fluid were found. Radiotherapy to the brain and orbit resolved these findings. Five months later, visual acuity decreased OS. Radiotherapy also was effective in treating the leukemic involvement of the optic nerve.     

Body surface isopotential T map for assessment of right ventricular volume and pressure overloads in secundum atrial septal defect

The body surface isopotential T map was analyzed to detect right ventricular volume and pressure overloads in 25 patients with secundum atrial septal defect. Three patterns were distinguished: a T map resembling normal (type A,n=9); that with an isolated negative area in a positive area (type B,n=11); and that with rightward movement of maximum (type C,n=5). Right ventricular end-diastolic volumes in types B (161±19% of normal; %N) and C (175±40%N) were significantly (p<0.01) greater than those in controls (100±9%N) and type A (113±18%N). Right ventricular systolic pressure in type C (48±11 mmHg) was significantly (p<0.01) higher than those in the controls (30±5 mmHg), type A (31±4 mmHg), or type B (34±5 mmHg). These results suggest that the patients with type B have right ventricular volume overload and those with type C have both volume and pressure overloads.     

Hepatic angiomyolipoma: value of breath hold 2D FLASH GD-dynamic imaging with fat saturation

A case of hepatic angiomyolipoma in a 61-year-old woman is reported. Findings on ultrasound (US), computed tomography (CT), and magnetic resonance (MR) imaging are presented and the usefulness of breath hold 2D FLASH Gd-dynamic imaging with fat saturation is discussed.     

Costello syndrome showing moyamoya-like vasculopathy

This report describes a patient with Costello syndrome associated with moyamoya-like vasculopathy. His clinical findings were sparse, thin, and light-colored hair, bilateral ptosis, low-set ears, depressed nasal bridge, bulbous nose, short neck, loose pigmented skin with deep palmar and plantar creases, bilateral cryptorchidism, and delays in growth and development. Brain magnetic resonance imaging and cerebral angiography revealed moyamoya-like vasculopathy. A skin biopsy from the extensor surface of the right thigh revealed shortening and rupture of elastic fibers. Electron microscopy indicated reduced depositions of elastin. Formation of a stable elastic fiber system may be impaired in patients with Costello syndrome, and brain magnetic resonance imaging and magnetic resonance angiography would be recommended for these patients.     

MRI for preoperative staging of renal cell carcinoma using the 1997 TNM classification: comparison with surgical and pathologic staging

OBJECTIVE: The purpose of our study was to determine the accuracy of MRI for preoperative staging of renal cell carcinoma using the 1997 TNM classification. MATERIALS AND METHODS: We conducted a retrospective review of MRI performed in 40 consecutive patients with 42 renal cell carcinomas before radical (n = 35) or partial (n = 4) nephrectomy or exploratory laparotomy (n = 3). The interval between imaging and surgery ranged from 1 to 59 days (mean, 17.9 days). Imaging was performed with T1- and T2-weighted, dynamic gadolinium-enhanced, and time-of-flight sequences. MRI and surgical-pathologic staging was performed using the 1997 TNM staging system. MRI staging was compared with surgical-pathologic staging as the gold standard. Agreement between the two staging methods was assessed using the kappa statistic. RESULTS: Agreement between MRI and surgical-pathologic staging was good for T staging (kappa = 0.72 and 0.78 for reviewers 1 and 2 respectively), poor for N staging (kappa = 0.13, both reviewers), good for M staging (kappa = 0.66, both reviewers), and excellent for the assessment of venous involvement (kappa = 0.93, both reviewers). MRI overestimated the T stage in five patients and the N stage in five and underestimated the T stage in three, the N stage in four, the M stage in one, and the extent of venous thrombosis in two patients. CONCLUSION: MRI is a reliable method for preoperative staging of renal cell carcinoma using the 1997 TNM classification, in particular for assessing venous involvement.