Long-term treatment of antiphospholipid syndrome-associated cerebral arterial thromboses with intravenous immunoglobulin: a case report.

We report a now 13-year-old male with trisomy 21, hypothyroidism, and insulin-dependent diabetes who developed acute hemiplegia due to the antiphospholipid antibody syndrome (APS) at age four. The risks of long-term anticoagulation were initially considered to be high; hence, he was treated with monthly infusions of intravenous immunoglobulin (IVIG) at 2 g/kg for 2 years and then every other month for 7 years. Antiphospholipid antibodies were no longer detectable within 6 months and have continued to be negative. There was no clinical deterioration or further changes on magnetic resonance arteriography over 7 years. IVIG may be an alternative therapeutic choice for children with APS who are not candidates for conventional anticoagulation.

Indium-111-labeled white blood cells: dosimetry in children.

The distribution of [111In]oxine-labeled leukocytes was studied by whole-body gamma camera imaging in nine pediatric patients. Images were obtained at approximately 24 hr after administration of the material. Organ distribution was estimated from stored data by manual region of interest assignment. Dosimetry estimates based on geometric mean and conjugate view absolute activity calculations showed close agreement in these patients. Leukocytes were distributed in liver, spleen and marrow. The mean percent uptakes +/- s.d. were: spleen, 31.2 +/- 18.3%; liver, 26.3 +/- 10.8%; and marrow, 14.2 +/- 5.7%. A significant portion (28.3 +/- 9.9%) of administered white cell activity was found outside these organs in the remainder of the body. Mean organ absorbed doses (rad/mCi) were: spleen, 115.0 +/- 84.8; liver, 13.9 +/- 7.8; marrow, 7.6 +/- 3.8; and total body 2.5 +/- 1.0. The mean organ absorbed doses (rad/dose administered) were: spleen, 13.7 +/- 10.6; liver, 1.48 +/- 0.62; marrow, 0.79 +/- 0.26; and total body, 0.28 +/- 0.09.

Intraorbital wooden foreign body: CT and MR appearance.

A case of an intraorbital wooden foreign body mimicking air on standard CT window setting and on MR is presented. Its higher attenuation with higher CT window setting as well as its elongated and well-delineated shape on both CT and MR helped to distinguish it from air.

CT and MR evaluation of a wooden foreign body in an in vitro model of the orbit.

We made an in vitro model of a wooden foreign body using both fresh and dry pine wood in both fat and soft-tissue background mediums. Air/wood/background medium interfaces were studied with MR and CT to determine which method provided the best image contrast for detecting a wooden foreign body. CT was demonstrated to be superior to MR in the evaluation of the orbit in the in vitro model.

Renal neoplasms of childhood.

Wilms' tumor is the most common childhood renal tumor. This article describes the epidemiology, histopathologic features, and clinical manifestations of Wilms' tumor along with the spectrum of imaging findings using different modalities. The distinguishing features of other renal tumors encountered in children, such as clear cell sarcoma, rhabdoid tumor, congenital mesoblastic nephroma, multilocular cystic renal tumor, renal cell carcinoma, and angiomyolipoma are also reviewed.

Tracheobronchography in children.

Tracheobronchography (TBG) can provide anatomic information supplementing endoscopy in evaluating complex congenital and acquired tracheal abnormalities. With the use of modern non-ionic contrast agents and flexible fiberoptic bronchoscopes, TBG can be performed safely and comfortably in children. As some tracheal lesions have a significant risk of mortality, obtaining maximum definition of the abnormality is imperative prior to attempting surgical management. Twenty-two patients underwent TBG at St. Christopher's Hospital for Children (Philadelphia) from July 1990 through June 1995. With the use of iohexol as a contrast agent, flexible bronchoscopes and adequate sedation and analgesia, without the need for intubation or general anesthesia, there were no significant complications or discomfort during or after the procedure. Techniques and illustrative radiographs are presented. TBG provides a useful adjunct to endoscopy.

Comparison of MRI and MRA findings in children with a variety of neurologic conditions.

The role of MRA in the evaluation of children is evolving. We compared MRA and MRI in children with a variety of neurologic conditions to determine when MRA provides positive, cost-beneficial information. A total of 114 patients were retrospectively studied. MRA and MRI were performed and compared. MRA was abnormal in 34 (30%) of 114 patients: five (83%) of six with Menkes' disease, four (33%) of 12 with sickle cell disease, 12 (38%) of 32 with vascular malformations, one (6%) of 17 with headaches, seven (24%) of 24 with new focal deficits, one (10%) of 10 with seizures, and four (31%) of 13 with miscellaneous diagnoses. MRA and MRI were concordant in 73 (64%) of 114. Maximum concordance was in patients with Menkes' disease (100%) and minimum in those with new focal deficits (50%). The best MRA cost/benefit ratios were obtained in patients with Menkes' disease, vascular malformations, and sickle cell disease. A normal MRI usually forecasted a normal MRA. However, abnormal MRI findings did not always predict MRA abnormalities. Positive, cost-beneficial information is provided by MRA mostly in conditions known to involve the cerebral vasculature. Indications to perform MRA should be based on the neurologic diagnosis and MRI findings.

Teratoid Wilms tumor arising as a botryoid growth within a supernumerary ectopic ureteropelvic structure.

We report a case of a teratoid Wilms tumor arising within a supernumerary ectopic ureteropelvic structure in a 7-year-old boy. The tumor was near the right kidney but was completely separate from it. On the contralateral side, the child had a duplication of the ureteral system. Pathologic examination revealed the mass to be completely enveloped by a fibromuscular sac lined by urothelium. The tumor had a botryoid, polypoid architecture and, in addition to areas of classic Wilms tumor, had extensive squamous, mucinous, and columnar elements. Teratoid Wilms tumors are rare and, to the best of our knowledge, have not previously been described as arising either in anomalous urinary structures or at 7 years of age.

Myocardial calcification in an extremely low birth weight infant with chronic renal failure and secondary hyperparathyroidism.

Myocardial calcification has been rarely described in premature infants after myocardial infarction and myocarditis with coxsackievirus B1. In adults and older children, metastatic myocardial calcification has been reported in chronic renal failure. We report a case of myocardial calcification in a 680-gm preterm infant after a prolonged course of renal failure complicated by secondary hyperparathyroidism. Subclinical myocardial injury was evidenced by a high serum creatine phosphokinase MB band concentration, which probably provided a susceptible substrate for the deposition of calcium crystals, because the multiplication product of serum calcium and inorganic phosphorus levels transiently exceeded 75 mg x mg/100 ml, indicating serum saturation during the course of secondary hyperparathyroidism. We report this case as an unusual complication of renal immaturity in extremely low birth weight infants and an indication of a relatively intact parathyroid glandular function in them. Hypoxia, myocardial dysfunction, and renal failure are common complications in such infants, and in the presence of renal failure, the serum levels of calcium and inorganic phosphorus should be maintained below the pathologic level to avoid ectopic calcification of the tissues, including the myocardium.

Chest masses in children: the plain film revisited.

Mass lesions in the chest can usually be best evaluated by conventional means, primarily plain chest film examination. Fluoroscopy is often useful, especially to differentiate normal thymus from abnormal anterior mediastinal masses and to evaluate possible vascular masses. It makes sense to do a barium swallow for most middle mediastinal masses before resorting to the more expensive CT or MRI. In addition to the added expense, the marked increase in radiation dose should also be considered when CT is contemplated.

Intravenous digital subtraction angiography: its use in evaluating vascular injuries in children.

Angiography is often essential in the evaluation of patients with suspected vascular injuries. However, arterial cannulation has a significant inherent complication rate, especially in small patients. Therefore, a less invasive method of evaluating children with such injuries is desirable. We report nine children (age range, 9 months to 16 years; median, 7 years) in whom intravenous digital subtraction angiography (IV-DSA) was used to evaluate suspected vascular injuries. IV-DSA showed a false aneurysm in two children, one managed by ligation alone and the other by resection with primary anastomosis. One child was found to have an arteriovenous fistula, which was treated by ligation. An iliac occlusion, following an arteriogram to evaluate a liver tumor, was documented by IV-DSA and successfully managed with aggressive anticoagulant therapy. Demonstration of a normal vascular tree avoided exploration in four children. A planned skin flap was abandoned in a child who suffered an electrical injury when IV-DSA showed lack of adequate vascular supply. In all nine cases, the IV-DSA obviated the need for intraarterial angiography. In addition to avoiding arterial cannulation, IV-DSA required significantly less radiation and less time to perform than conventional arteriography. Motion artifact, vessel overlap, and some loss of minute detail are some of its potential shortcomings. IV-DSA is an effective method of screening children with suspected vascular injuries. In addition, our experience has shown that IV-DSA can be the definitive diagnostic procedure, although intraarterial angiography may be necessary in selected cases.

Milk of calcium cholelithiasis in children.

Milk of calcium bile is uncommon and occurs mainly in the adult population. The authors report on 2 children, each having a distinct clinical history and presentation, and each with milk of calcium bile/calculi possessing differing chemical composition and highly notable gross morphology. J Pediatr Surg 36:644-647.

Phrygian cap simulating mass lesion in hepatobiliary scintigraphy.

Hepatobiliary imaging has been used to demonstrate that defects in the region of the gallbladder fossa in sulfur colloid studies correspond to normal gallbladder and not tumor. We report a case of delayed filling of a portion of the gallbladder due to a Phrygian cap anomaly. The case demonstrates that without delayed images incomplete filling of the gallbladder can be confused with a mass lesion in the liver.

Use of bone scintigraphy in the management of slipped capital femoral epiphysis.

Fourteen patients with slipped capital femoral epiphysis (SCFE) who had high-resolution and magnification bone scintigraphy during treatment were studied. By demonstrating the vascular status of the femoral head and physiologic condition of the growth plate, scintigraphy was found to assist the clinician in the management of patients with this disorder.

Bubble gum simulating abdominal calcifications.

CT examination of the abdomens of two children demonstrated sites of high attenuation in the stomach, which were revealed to be bubble gum. Investigation of the CT appearance of samples of chewing gum showed that it consistently has high attenuation (178-345 HU). The attenuation of gum base, which contains calcium carbonate, was 476 HU. In addition, examination of a volunteer who had swallowed bubble gum confirmed the CT appearance.

MRI appearances of metachromatic leukodystrophy.

BACKGROUND: The leukodystrophies constitute a wide spectrum of cerebral disorders of varying etiology. The imaging appearances on CT and MRI are recognizable as abnormalities of white matter; however, it may be impossible to arrive at the correct diagnosis based on imaging studies alone. PATIENTS AND METHODS: Three patients of varying age and clinical symptomatology diagnosed with metachromatic leukodystrophy (MLD) had remarkably similar MRI appearances. A "tigroid" or "leopard-skin" appearance was demonstrated within deep white matter in each case. RESULTS: All of the patients had biochemical confirmation of MLD. CONCLUSION: Although the "tigroid" pattern previously was considered to be pathognomonic of Pelizaeus-Merzbacher disease, the diagnosis of MLD must now be considered when these MRI appearances are encountered.

Osteolysis of the calcaneus in a child with localized scleroderma.

In a young girl with localized scleroderma a circumscribed area of lysis developed in the calcaneus beneath the involved skin and soft tissues of the foot. A biopsy revealed vascular changes characteristic of scleroderma associated with infarction and severe resorption of the bone. A geographic pattern of bone destruction in a child with localized scleroderma has not previously been reported.

CT urograms in pediatric patients with ureteral calculi: do adult criteria work?

BACKGROUND: Secondary signs of urinary obstruction associated with ureteral calculi are useful adjuncts to diagnosis in adults with renal colic evaluated by unenhanced helical CT. OBJECTIVE: Our purpose was to evaluate the frequency of secondary signs of obstruction in children with renal colic undergoing unenhanced helical CT. MATERIALS AND METHODS: Ureteral calculi were identified in 20 of 61 children with acute flank pain examined by unenhanced helical CT. Each imaging study was evaluated for the presence of secondary signs of urinary obstruction. The frequencies of individual signs were compared with each other by means of the McNemar test. RESULTS: Six children had no secondary sign identified. In the remaining 14 children, proximal ureteral dilatation was seen in 10, renal enlargement in 10, hydronephrosis in 9, tissue rim sign in 6, decreased kidney attenuation in 5, and perinephric stranding in 1. Comparison of the frequencies strongly suggested that perinephric stranding occurs less frequently than proximal ureteral dilatation (P = 0.004), hydronephrosis (P = 0.008), or renal enlargement (P = 0.012). CONCLUSION: Perinephric stranding, a common secondary sign in adults with ureteral calculi, occurs less frequently in children than other reported secondary signs.