Tumoral calcinosis of the foot.

Tumoral calcinosis is characterized by periarticular deposition of calcium phosphate, usually in the setting of normocalcemia and hyperphosphatemia. The term tumoral calcinosis can be used to describe lesions with periarticular deposition of calcium phosphate resulting either from a primary disorder or a secondary disorder, such as renal failure with associated secondary hyperparathyroidism. Treatment entails phosphate deprivation, control of any primary disease processes such as secondary hyperparathyroidism, and resection for recalcitrant symptoms. We present a case of tumoral calcinosis involving the foot which required resection and ultimately hyperparathyroidectomy.

Conformal proton radiation treatment for retroperitoneal neuroblastoma: introduction of a novel technique.

BACKGROUND: Postoperative irradiation for locoregionally advanced neuroblastoma requires coverage of the paraspinal retroperitoneum. The proximity of both kidneys and the liver, and a more complex target configuration, can pose a dosimetric challenge for conventional X-ray treatment and intraoperative irradiation. We utilized proton radiation therapy (PRT) to reduce dose to uninvolved kidneys, liver, intestine, and spinal cord. PROCEDURE: A 4-year-old male underwent PRT for neuroblastoma of the right adrenal gland, following chemotherapy and delayed surgical resection. Clinical target volume (CTV), boost volume, and normal structures were outlined on the 3D treatment planning CT scan. The patient received 25.2 CGE (cobalt Gray equivalent) to the CTV and 34.2 CGE to the boost region, using 1.8 CGE per fraction, five treatments per week. Dose-volume histograms (DVHs) were obtained for target and nontarget structures. RESULTS: The 95% isodose volume enclosed CTV and boost volumes. The dose to 50% of the ipsilateral kidney, with tumor involvement of the medial renal surface, was < or = 16 CGE (47% of prescribed total dose). Doses to 50% and 20% of the contralateral kidney in close proximity to deep left-side, paraspinal soft tissue involvement were restricted to 1 CGE and 10 CGE, respectively. Eighty percent of the liver received 27 CGE (80% of prescribed dose). Using a patch technique, unique to charged particle therapy, the spinal cord was almost completely spared during boost volume irradiation. CONCLUSIONS: PRT can achieve excellent dose conformity for advanced retroperitoneal, paraspinal lesions, while respecting normal tissue tolerance levels.

Conformal proton radiation therapy of the posterior fossa: a study comparing protons with three-dimensional planned photons in limiting dose to auditory structures.

PURPOSE: Conventional radiation therapy for pediatric posterior fossa tumors can cause sequelae such as hearing loss and impairments in language and learning. Modern three-dimensional (3D) treatment techniques have improved dose conformity to the posterior fossa. This report compares the normal tissue dose-sparing capabilities of proton radiation therapy (PRT) with 3D conformal photon plans. METHODS AND MATERIALS: Nine children underwent previous PRT for primary CNS malignancies. Using original planning CT scans, the posterior fossa, inner and middle ear, and temporal lobes were delineated. Three-dimensional treatment plans were generated for protons and photons. Normal tissue exposures were calculated by averaging mean doses received and by analysis of dose-volume histogram. RESULTS: The 95% isodose encompassed the posterior fossa in all plans. Normal structures received markedly less radiation from PRT plans than from 3D photon plans. The cochlea received an average mean of 25 +/- 4% of the prescribed dose from PRT, and 75 +/- 6% from photons. Forty percent of temporal lobe volume was completely excluded using protons; with photons 90% of the temporal lobe received 31% of the dose. CONCLUSION: PRT resulted in increased dose sparing of normal structures analyzed. Posterior fossa conformity of 3D photons came at the expense of increasing amounts of normal tissue receiving low to moderate doses.

Periosteal chondroma of the cuboid presenting in a 7-year-old-boy.

This case report discusses the finding of a periosteal (juxtacortical) chondroma of the cuboid in a 7-year-old male. While this lesion is well recognized in the tubular bones of adults, this case is unusual due to the child's age, the site of the lesion (cuboid), and the difficulty in establishing the diagnosis due to the cellular atypia. The child was treated with marginal resection and curettage with no evidence of local recurrence on follow-up. A review of the literature is also included in the discussion.

Proton radiation therapy (PRT) for pediatric optic pathway gliomas: comparison with 3D planned conventional photons and a standard photon technique.

PURPOSE: Following adequate therapy, excellent long-term survival rates can be achieved for patients with optic pathway gliomas. Therefore, avoidance of treatment-related functional long-term sequelae is of utmost importance. Optimized sparing of normal tissue is of primary concern in the development of new treatment modalities. The present study compares proton radiation therapy (PRT) with a three-dimensional (3D)-planned multiport photon and a lateral beam photon technique for localized and extensive optic pathway tumors. METHODS AND MATERIALS: Between February 1992 and November 1997, seven children with optic pathway gliomas underwent PRT. For this study, we computed proton, 3D photon, and lateral photon plans based on the same CT data sets, and using the same treatment planning software for all plans. Radiation exposure for normal tissue and discrete organs at risk was quantified based on dose-volume histograms. RESULTS: Gross tumor volume (GTV) ranged from 3.9 cm3 to 127.2 cm3. Conformity index (relation of encompassing isodose to GTV volume) was 2.3 for protons, 2.9 for 3D photons, and 7.3 for lateral photons. The relative increase of normal tissue (NT) encompassed at several isodose levels in relation to NT encompassed by the 95% proton isodose volume was computed. Relative NT volume of proton plan isodoses at the 95%, 90%, 80%, 50%, and 25% isodose level increased from 1 to 1.6, 2.8, 6.4, to a maximum of 13.3. Relative volumes for 3D photons were 1.6, 2.4, 3.8, 11.5, and 34.8. Lateral plan relative values were 6, 8.3, 11.5, 19.2, and 26.8. Analysis for small (<20 cm3) and larger (> 80 cm3) tumors showed that protons encompassed the smallest volumes of NT at all isodose levels. Comparable conformity and high-dose gradient were achieved for proton and 3D photon plans in small tumors. However, with increasing tumor volume and complexity, differences became larger. At the 50% isodose level, 3D photons were superior to lateral photons for small tumors; this advantage was equalized for larger tumors. At the lowest isodose level, 3D photons encompassed the highest amount of NT. Analysis of organs at risk showed that PRT reduced doses to the contralateral optic nerve by 47% and 77% compared to 3D photons and lateral photons, respectively. Reductions were also seen for the chiasm (11% and 16%) and pituitary gland (13% and 16%), with differences at clinically relevant tolerance levels. Furthermore, reduced dose exposure of both temporal lobes (sparing 39% and 54%) and frontal lobes was achieved with PRT. CONCLUSION: PRT offered a high degree of conformity to target volumes and steep dose gradients, thus leading to substantial normal tissue sparing in high- and low-dose areas. It is expected that this will result in decreased long-term toxicity in the maturing child. Advantages of proton versus 3D photon plans became increasingly apparent with increasing target size and tumor complexity. Even in small tumors, conformity of 3D photon irradiation came at the expense of a larger amount of NT receiving moderate to low radiation doses. Lateral photons resulted in inferior dose distribution with high radiation exposure of clinically relevant normal tissues.

Irreducible dislocation of the knee.

Irreducible knee dislocation is a rare injury. This case report describes a knee dislocation in a 39-year-old male U.S. Army noncommissioned officer who was injured while playing in a softball game. Arthroscopy showed the medial collateral ligament and capsule to be locked in the intercondylar notch, covering the medial femoral condyle. Arthrotomy and open reduction were required. Staged posterior cruciate ligament reconstruction using patellar tendon autograft was later performed. Review of the magnetic resonance imaging scan showed the irreducible lesion. The diagnostic clinical and radiographic features of this unusual injury are described.

Proton radiation therapy for chordomas and chondrosarcomas of the skull base.

OBJECT: Local tumor control, patient survival, and treatment failure outcomes were analyzed to assess treatment efficacy in 58 patients in whom fractionated proton radiation therapy (RT) was administered for skull base chordomas and chondrosarcomas. METHODS: Between March 1992 and January 1998, a total of 58 patients who could be evaluated were treated for skull base tumors, 33 for chordoma and 25 for chondrosarcoma. Following various surgical procedures, residual tumor was detected in 91% of patients; 59% demonstrated brainstem involvement. Target dosages ranged from 64.8 and 79.2 (mean 70.7) Co Gy equivalent. The range of follow up was 7 to 75 months (mean 33 months). In 10 patients (17%) the treatment failed locally, resulting in local control rates of 92% (23 of 25 patients) for chondrosarcomas and 76% (25 of 33 patients) for chordomas. Tumor volume and brainstem involvement influenced control rates. All tumors with volumes of 25 ml or less remained locally controlled, compared with 56% of tumors larger than 25 ml (p = 0.02); 94% of patients without brainstem involvement did not experience recurrence; in patients with brainstem involvement (and dose reduction because of brainstem tolerance constraints) the authors achieved a tumor control rate of 53% (p = 0.04). Three patients died of their disease, and one died of intercurrent disease. Actuarial 5-year survival rates were 100% for patients with chondrosarcoma and 79% for patients with chordoma. Grade 3 and 4 late toxicities were observed in four patients (7%) and were symptomatic in three (5%). CONCLUSIONS: High-dose proton RT offers excellent chances of lasting tumor control and survival, with acceptable risks. In this series all small- and medium-sized tumors with no demonstrable brainstem involvement have been controlled; all such patients are alive. Surgical debulking enhanced delivery of full tumoricidal doses, but even patients with large tumors and disease abutting crucial normal structures benefited.

Disruption of the anterior cruciate ligament in a 4-year-old child.

Anterior cruciate ligament (ACL) injuries in children are rare. Thus, the natural history of ACL injuries in skeletally immature patients is unknown. This case represents the longest follow-up (11 years) reported in the literature of an ACL injury in a child of less than 5 years old.

Acute gonococcal flexor tenosynovitis in an adolescent male with pharyngitis. A case report and literature review.

A 15-year-old boy had acute gonococcal flexor tenosynovitis of the middle finger associated with symptomatic gonococcal pharyngitis. The patient had a history of blunt trauma to the hand. The need for a careful history in any sexually active adolescent is emphasized. Acute flexor tenosynovitis may be the only manifestation of disseminated gonococcal infection.

Anterior cruciate ligament reconstruction: evaluation with MR imaging.

Fifty magnetic resonance (MR) imaging examinations were performed in 37 patients after arthroscopic anterior cruciate ligament (ACL) reconstruction with patellar bone-tendon-tibial bone autografts. T1-weighted sagittal and axial images were obtained. In 34 patients with clinically stable ACL autografts, 43 of 47 MR examinations demonstrated a well-defined, intact ACL autograft. All three patients with ACL laxity failed to demonstrate a well-defined autograft, for an overall correlation between MR imaging and clinical examination results of 92%. Of the 12 patients who underwent second-look arthroscopy, 100% correlation was present between MR imaging and arthroscopic results. As in the nonreconstructed knee, buckling of the posterior cruciate ligament was suggestive of ACL laxity. MR imaging also documented optimum placement of bone tunnels in the femur and tibia. MR imaging has proved to be an excellent noninvasive imaging modality for evaluating ACL reconstruction, while also providing ancillary information about the postoperative knee.

Additional chenodiol therapy after partial dissolution of gallstones with two years of treatment.

During the National Cooperative Gallstone Study, therapy with chenodiol, 750 or 375 mg/d, for 2 years resulted in confirmed, complete gallstone dissolution in 14% and 5% of patients, respectively, and partial dissolution (greater than 50%) in 27% and 18%. The present study was done to determine the frequency with which complete dissolution occurs in patients having partial dissolution of gallstones who receive additional therapy. Eighty-six of one hundred thirty-eight eligible patients continued to receive 750 mg/d (61 patients) or 375 mg/d (25 patients) of chenodiol for 1 year. Patients whose oral cholecystogram at the end of the year showed further (greater than 50%) dissolution continued to receive chenodiol, (28 patients at 750 mg/d and 11 patients at 375 mg/d) for a second year (total duration of therapy, 4 years). A final oral cholecystogram was taken at the end of the fourth year. Complete dissolution occurred in 23% and 16% of patients receiving chenodiol, 750 or 375 mg/d, respectively, for an additional 1 or 2 years.

Naloxone, a specific opioid antagonist, reverses chronic idiopathic constipation.

Two patients with long-standing idiopathic chronic constipation, which responded only to large daily doses of laxatives and additional suppositories and enemas, were treated with the specific opioid antagonist, naloxone, on a single-blind crossover basis. Both patients responded to naloxone treatment, with increased passage of faeces and increased wet and dry faecal weight. Although naloxone is poorly absorbed after oral administration, there was a positive response during oral as well as intravenous treatment, suggesting that the primary effect of naloxone is at specific opiate receptor sites in the myenteric plexus and other neural and endocrine cells of the intestinal wall.

Detection of e antigen and antibody: correlations with hapatitis B surface and hepatitis B core antigens, liver disease, and outcome in hepatitis B infections.

Testing for e antigen and antibody (anti-e) was performed by immunodiffusion and counterelectrophoresis in patients with polyarteritis nodosa fulminant hepatitis, and chronic active hepatitis (CAH), in 59 asymptomatic carriers of hepatitis B surface antigen (HBsAg) who underwent liver biopsy, and in 150 carriers followed with sequential SGPT determinations. Counterelectrophoresis was more sensitive that immunodiffusion. Neither e antigen nor anti-e was found in the absence of HBsAg. Among HCsAg-positive patients with polyarteritis nodosa and CAH, e antigen was found in 16 of 18 and 13 of 22, respectively. It was not found in any of 43 patients with fulminant hepatitis, of whom 24 were HBsAg-positive. The e antigen was detected in none of 13 biopsied carriers with normal histology, 4 of 28 with nonspecific changes of 11 of 18 with CAH or chronic persistent hepatitis. Conversely, anti-e was present in 9 of 13 with normal biopsy, 7 of 28 with nonspecific changes, and none of 18 with CAH or chronic persistent hepatitis. The e antigen was found more commonly in nonbiopsied carriers with elevated SGPT, and anti-e in those with normal SGPT. Six carriers whose antigenemia terminated spontaneously had anti-e. The presence of e antigen correlated with a high titer of HBsAg, and with immunofluorescent detection of hepatitis B core antigen in the nuclei of hepatocytes. Conversely, anti-e was associated with significantly lower titers of serum HBsAg (P less than 0.001) and lack of detectable hepatitis B core antigen in the liver.

The natural history of liver disease in former drug users.

Three hundred and twenty drug-free former narcotic addicts were studied with regard to persistence of abnormalities of liver function and morphology, and their relation to hepatitis B infection. Hepatitis B antibody was present in 52.4 per cent, while HBs antigen was detected in only 6 per cent. Transaminase abnormalities, initially present in 39 per cent, were found in 22 per cent six months after cessation of drug abuse. Abnormalities tended to persist thereafter, although there was some continued return to normal levels. Liver biopsy findings of chronic persistent and aggressive hepatitis correlated with persistence of HBs antigenemia and transaminase elevation. Follow-up liver biopsies in seven subjects showed decreased inflammatory reaction in five. None showed progressive liver disease. We conclude that: (1) 15 to 20 per cent of former narcotics addicts have chronic persistent hepatitis or chronic aggressive hepatitis after cessation of drug absuse for six months or more; (2) serologic evidence of exposure to HBs antigen is frequent, and rapidly develops after the start of needle use; (3) although histologic ad chemical abnormalities usually persist, progression did not occur, and some individuals demonstrated spontaneous improvement.

Corrected transposition of the great arteries simulating coronary heart disease in adults.

Two middle-aged adults with corrected transposition of the great arteries presented with chest pain and dyspnea, initially attributed to coronary arterial disease with left ventricular dysfunction and mitral regurgitation. The clinical diagnosis of coronary arterial disease was supported in these patients by electrocardiographic changes of probable old anteroseptal myocardial infarction and by an abnormal cardiac silhouette on the chest x-ray film suggestive of ventricular aneurysm. Coronary arteriographic studies in both patients showed that the morphologic left coronary artery supplied the venous ventricle, and the right coronary artery supplied the systemic ventricle. Neither patient had coronary arterial luminal narrowing. We propose that anginal chest pain in these patients resulted from inadequate coronary reserve of the morphologic right ventricle functioning as a systemic ventricle.

Duodenal ulcer in rural Haiti I. Clinical features. II. Gastric secretory studies.

I. In a study of 100 duodenal ulcer patients in rural Haiti, pyloric obstruction was present initially in nine per cent, but later developed in an additional 20 per cent. This high incidence of obstruction is similar to that observed in other tropicla populations in Africa and India. II. Standardized tests for basal and stimulated acid secretion in a small group of duodenal ulcer patients in rural Haiti were not elevated. The implication of the study is that either the test must be modified for different populations or the role of acid secretion in the pathogenesis of the disease questioned.

Pericardiocentesis: insertion of a pericardial catheter.

Pericardiocentesis is a lifesaving procedure in cardiac tamponade but is associated with significant and often life threatening complications. A patient is described in whom a catheter was inserted into the pericardium. This provided for prolonged pericardial drainage of purulent fluid and has the potential for decreasing the risk of this procedure.