[Frequency of lymphoscintigraphic anomalies in the contralateral limb and progression of unilateral primary lymphedema in children]. / Fréquence des anomalies lymphoscintigraphiques infracliniques controlatérales et évolution des lymphœdèmes primaires unilatéraux de l'enfant.

BACKGROUND: Primary lymphoedema (LE) is the consequence of lymphatic insufficiency, usually associated with hypoplasia of the lymph vessels and/or nodes. Lymphoscintigraphy allows early diagnosis. OBJECTIVES: To assess the frequency of contralateral LE by lymphoscintigraphy in children with unilateral primary LE of one limb; to assess facets of clinical bilateralization of LE. METHODS: This retrospective single-centre study included children with unilateral LE followed up at the Tours university hospital centre between 2004 and 2014. Parents were contacted by phone to obtain follow-up data. RESULTS: Of the 13 children included, 6 (46%) displayed bilateral lymphatic anomalies on lymphoscintigraphy. Within a median follow-up period of 6 years, clinical bilateralization was observed in one girl after progression of her LE for 7 years; in her case, bilateral lymphatic insufficiency was detected at the initial lymphoscintigraphy. CONCLUSION: While contralateral subclinical lymphatic insufficiency is frequent in children with unilateral primary LE of the limbs, clinical bilateralization appears only rarely.

Serial lymphoscintigraphic findings in a patient with Gorham's disease with lymphedema.

Gorham's disease is a rare disorder characterized by vascular, "lymphangio- matous" and/or "hemangiomatous" lesions in bone and surrounding soft tissues. Associated lymphedema has not been reported and clinical evolution is unpredictable. Plain radiographs, CT, MRI, and occasionally bone scintigraphy, are used to detect the bone and soft tissues changes. Biopsy is a major component of the diagnostic process. We report the findings of serial lymphoscintigraphy in a young boy with a polyostotic Gorham's disease associated with lymphangioma of the thigh and lower limb lymphedema. In this patient, lymphoscintigraphy was useful for diagnosis and follow-up of primary lymphedema. It provided valuable information concerning the occurrence, location, and progression of lymphatic lesions in both bone and soft tissues.

Contributions of SPECT/CT imaging to the lymphoscintigraphic investigations of the lower limb lymphedema.

Lymphoscintigraphy is a safe and reliable technique for investigating lymphedema. However, interpretation of delayed planar conventional imaging may be questionable due to the superimposition of soft tissues. Therefore, the aim of this retrospective study was to evaluate the contribution of single photon emission computed tomography/computed tomography (SPECT/CT) to lymphoscintigraphic investigation in a selected population with abnormal interstitial activity. Forty one patients with lower limb lymphedema either primary (n=17) or secondary (n=5), or associated with chronic venous insufficiency (n=19) underwent lymphoscintigraphy according to a standard protocol. SPECT/CT imaging that started immediately after planar imaging covered the part of the lower limbs with the most significant interstitial activity. The CT images were also analyzed separately to identify the typical honeycomb pattern of lymphedema. SPECT/CT identified additional abnormalities in vessels and soft tissues in 19 patients (46%). The additional information, primarily related to dermal collateralization of flow, dermal backflow, lymphangioma, and lymphorrhea, aided in understanding the physiopathology of edema and may have influenced management in 35 patients (85%). The honeycomb appearance of the soft tissues was noted on CT in most patients (90%). This study suggests that SPECT/CT is a promising addition to planar imaging in lymphoscintigraphic investigations of lymphedema.

[Limb lymphedema: Diagnosis, explorations, complications. French Lymphology Society]. / Les lymphoedèmes des membres: diagnostic, explorations, complications.

Lymphedema results from impaired lymphatic transport with increased limb volume. Primary and secondary forms can be distinguished. Secondary lymphedema of the upper limb is the most frequent in France. A 2-cm difference on any segment of the limb confirms the diagnosis of lymphedema. Calculated lymphedema volume using the formula for a truncated cone is required to assess the efficacy of treatment and to monitor follow-up. Primary lymphedema is sporadic but rarely familial. Lymphoscintigraphy is useful in the primary form to evaluate precisely lymphatic function of the two limbs. Erysipelas is the main complication,but psychological or functional discomfort may occur throughout the course of lymphedema. Lipedema is the main differential diagnosis, defined as an abnormal accumulation of fat from hip to ankle and occurs almost exclusively in obese women.

[Urbach-Wiethe disease (lipoid proteinosis) with neurological involvement]. / Maladie d'Urbach-Wiethe (lipoïdo-protéinose) avec manifestations neurologiques.

INTRODUCTION: Lipoid proteinosis is a rare autosomal recessive disease that has recently been shown to result from mutations in the ECM1 gene. Some cases are associated with a more severe mucocutaneous phenotype. OBSERVATION: We report the case of a 38-year-old woman who had clinical and histological skin features typical of Urbach-Wiethe disease. Physical examination revealed neurological abnormalities including dizziness, ataxia, slight psychomotor retardation and amnesic impairment. The patient reported sudden left brachiofacial paralysis one month earlier that regressed spontaneously after one week. CT scan and MRI were normal. Cerebral scintigraphy displayed bilateral hypoperfusion of the frontal areas, of the anterior and internal right temporal lobe (which includes amygdala), and of the left thalamic core. DISCUSSION: We considered these abnormalities as neurological features of lipoid proteinosis in the absence of evidence of any other cause. Our observation as well as other recent reports suggests the need for neurological and neuropsychological investigations in patients with Urbach-Wiethe disease.

[Lymphedema of the limb: predictors of efficacy of combined physical therapy]. / Lymphoedème de membre. Critères prédictifs de l'efficacité du traitement physique combiné.

BACKGROUND: Limb lymphedema results from incompetence of the lymphatic system, and treatment of both primary and secondary forms involves manual lymphatic drainage and support. The effectiveness of treatment varies from patient to patient and can be unpredictable. OBJECTIVE: To investigate clinical and paraclinical criteria able to predict responses to combined physical treatment (lymphatic drainage and multilayered support) during hospitalization. PATIENTS AND METHODS: A retrospective study was performed in 45 patients admitted for one week's intensive treatment of limb lymphedema in the Lymphology Unit of the Department of Dermatology of the University Hospital of Tours. Lymphoscintigraphy was performed for all patients on admission. Loss of volume in affected limbs was studied to evaluate the effectiveness of treatment. RESULTS: Two criteria were predictive of resistance to combined treatment, i.e. venous insufficiency and continuing lymph node evidence of scintigraphic activity four hours after lymphoscintigraphy. The two factors were significantly related. CONCLUSION: Failure of combined physical treatment for lymphedema appears to be related to venous insufficiency. It is therefore essential to investigate and treat venous insufficiency, particularly in patients with continuing evidence of scintigraphic activity in lymph nodes.

[Contribution of lymphoscintigraphy in the exploration of lymphedema in children]. / Intérêt de la lymphoscintigraphie dans l'exploration des lymphoedèmes de l'enfant.

OBJECTIVES: The objectives of this work were a) to assess the feasibility of lymphoscintigraphy in very young children, and b) to assess its usefulness in understanding the underlying pathophysiology. PATIENTS AND METHODS: Forty-one children (age range 3 months-16 years) with primary lymphedema of the lower and/or upper limbs underwent lymphosinctigraphy using 99mTC-labeled colloid (Nanocis or Nanocol) injected subcutaneous at the foot and/or hand level. Recording time, which depended on the child's age, included at least static images during the thirty minutes following injection and four hours later. RESULTS: Abnormal activity of the lymph collectors, nodes, and interstitial space were analyzed according to age (> or < 2 years). Abnormal findings were classed as simple hypoplasia (n=18) or other type of lymph disease: diffuse lymphatic dysplasia (n=6), mixed vascular dysmorphism (n=5), amniotic bride disease (n=1), and hypoplasia revealed by occurrence of a minor factor (n=11). These observations demonstrate the role of lymphoscintigraphy in the diagnosis and prognostic evaluation of lymphedema in children. CONCLUSION: Lymphoscintigraphy is a useful technique for very young children with lymphedema providing further information contributing to overall exploration.

The value of immunoscintigraphy in the detection of recurrent colorectal cancer.

The aim of this prospective study was to determine whether anti-carcinoembryonic antigen (anti-CEA) scintigraphy is a useful additional technique in the diagnosis recurrence of colorectal cancer. Forty patients with suspected recurrence of colorectal cancer, underwent immunoscintigraphy (IS) and helical computed tomography (CT) in the 2 weeks before surgery. Surgical findings were used to evaluate the performance of the imaging techniques. Suspected areas on IS and CT were systematically explored. Helical CT was found to be superior to IS for the liver, the sensitivity and specificity of CT being 100% and 90%, respectively, vs 53% and 100% for IS. However, IS was better than CT for the detection of extra-hepatic abdominal recurrence: sensitivity and specificity of IS were 100 and 82% respectively vs 33 and 82% for CT. Seven cases of peritoneal carcinomatosis were overlooked by helical CT. Our results indicate that IS improves detection of extra-hepatic abdominal recurrence of colorectal cancer. Immunoscintigraphy is valuable as a guide to the treatment strategy and operative procedures.

[Incidental detection of a rare liver tumor: angiomyolipoma]. / Une rare lésion hépatique de découverte fortuite: l'angiomyolipome.

We report the case of a woman who presented a single liver lesion with no evidence of specific findings at Doppler US, CT, nuclear studies and MRI to suggest angiomyolipoma. The final diagnosis was confirmed at anatomopathology and immunohistochemistry which demonstrated positive anti-HMB 45 aspect.

Technetium-99m ECD single photon emission computed tomography in brain trauma: comparison of early scintigraphic findings with long-term neuropsychological outcome.

OBJECTIVE: The aim of this study was to assess the prognostic value of early brain scintigraphy in head injury in relation to long-term neuropsychological behavior. Twenty-four patients underwent technetium-99m (Tc-99m) ethyl cysteinate dimer single photon emission computed tomography (SPECT) within 1 month of the trauma. Scintigraphic abnormalities were evaluated both visually and semiquantitatively using the brain-to-cerebellum ratio method. The clinical neuropsychological investigation was conducted to evaluate abnormalities related to motor deficit, frontal behavior, and memory and language disorders. All patients had abnormalities on SPECT scan. One year after trauma, 14 patients (58%) had neuropsychological sequelae. The brain-to-cerebellum ratios in the left basal ganglia and brain stem were significantly decreased in patients with memory disorders (P = .03 and P = .02, respectively). Moreover, SPECT visual analysis indicated that low uptake in the basal ganglia, thalamus, and brain stem was associated with subsequent motor deficit, frontal behavior, and language and memory disorders. The authors conclude that brain SPECT can be valuable in predicting the neuropsychological behavior of survivors of severe head injury.

[Value of esophageal scintigraphy in exploration of a gastro-esophageal reflux in a respiratory patient]. / Intérêt de la scintigraphie oesophagienne dans l'exploration d'un reflux gastro-oesophagien chez le patient respiratoire.

Patients with respiratory pathologies in the forms of tracheitis, rhino-sinusitis, and asthma sometimes have symptoms of gastro-oesophageal reflux, that should be taken into consideration in order to treat the co-factors associated with the etiopathology. However, these patients, because of their respiratory handicaps, are frequently unwilling to submit themselves to traumatic investigations. Oesophageal scintigraphy, because of its perfect tolerance and reliability, seems to be an examination that is adapted to the situation to resolve this dilemma.

[Scintigraphic detection of gastrointestinal hemorrhage. Value and limitations]. / Localisation scintigraphique des saignements digestifs. Intérêts et limites.

A technetium-99m labelled red blood cell scintigraphy was performed in 51 patients with an unexplained gastro intestinal bleeding. Static images were acquired on the injection day then at 24 hours. Forty one patients had a positive scintigraphy. In 13 patients with a positive scintigraphy, on the injection day (5 cases) and later (8 cases), no intestinal bleeding was identified in spite of further investigations guided by scintigraphy. In 24 patients, with a positive scintigraphy on the injection day (20 cases) and later (4 cases), an intestinal lesion was identified and treated; data were confirmed in 19 cases: there were 15 colon lesions, localized on caecum in 10 cases and 4 small bowel lesions. Clinical value and limitations of the investigation are discussed. The scintigraphic localization is usually accurate if the abnormal focus activity is noticed on the injection day. It helps to guide further investigations of the bleeding site. This non invasive method should be performed more quickly to help to localize intestinal bleeding which poses a diagnostic problem.

The potential usefulness of condensed image processing of sequential lymphoscintigrams in patients with lymphedema.

Condensed image processing (CIP), a computerized technique of scintiscans that generates a vertical distribution of activity as a function of time in a single image, was applied to isotopic lymphoscintigrams of four patients with leg lymphedema. By "condensing" information gained from multiple images into a single image, CIP better evaluates ascending progression of radiolabeled colloid in peripheral lymph and better localizes stagnant areas. In "high lymph flow failure," CIP also depicts radioactive "bursts" suggestive of increased lymphatic truncal contractility. This data processing method improves interpretation of dynamic scintiscan recordings and seems ideally suited for evaluation of peripheral lymph kinetics.

[The current role of lymphoscintigraphy in the study of lymphedema of the limbs]. / Place actuelle de la lymphoscintigraphie dans l'étude d'un lymphoedème des membres.

After a brief review of the classical methodology and results of lymphoscintigraphy, the technique is considered in terms of an original kinetic study approach. Immediately after distal subcutaneous injection of a technetium colloid (rhenium sulfocolloid), 40 1-min serial images of the limbs are recorded followed 4 h after injection by recording of static images. The main abnormalities detected by scintigraphy concern the lymphatic vessels, nodes and interstitium. They reveal the presence of lymphedema and provide information about its mechanism, indicating, particularly in the case of primary lymphedema, whether there is predominant hyperplasia or hypoplasia in vessels or nodes. Synoptic study of the initial dynamic recording based on simple examination of serial images is difficult. Complementary data provided by activity curves determined from manually selected regions of interest (ROI'S) are of limited value since the ROI'S correspond to superimposed spatial structures. Two data-processing techniques--the condensed image (CI) and factorial analysis (FA)--were therefore applied to dynamic scintigraphic recordings in order to study lymphatic progress of the radiotracer in the legs. A complete dynamic series is described in two IC, one for each leg. The CI, obtained by mounting the 40 serial images in vertical strips 1 pixel in width, describes the spatial distribution of radioactivity along the leg during recording. Factorial analysis provides automatic extraction of pure kinetic components or factors from the dynamic series despite their spatial superposition. The factors are described by factorial curves and factorial images representing the spatial identity of the factor. Three-factor FA was applied successively to each leg.(ABSTRACT TRUNCATED AT 250 WORDS)

Factorial analysis in radionuclide lymphography: assessment of the effects of sequential pneumatic compression.

The effects of intermittent pneumatic compression (IPC) in 12 patients with lower or upper limb lymphedema were studied using a computer-based technique (factorial analysis) of dynamic lymphoscintigraphy. After subcutaneous injection of radiocolloid into the first interdigital web space of the arm or leg, scintigraphic recordings consisted of 40 consecutive one-minute frames of both lower extremities or an edematous upper extremity. Pneumatic compression by Euroduc (6 patients) or by Lymphapress (6 patients) was applied during the final 20 minutes of the recording. A three factors factorial analysis (FA) was performed successively for each extremity. FA allowed "uncontaminated" curves to be displayed distinct from neighboring structures and corresponded to dynamic tracer activity in the interstitium, at the injection site, and within lymphatic vessels. Based on the results of lymphatic vascular factorial analysis, a beneficial effect of IPC was detected in 18 of 22 limbs examined. FA processing suggested that IPC facilitated radiocolloid transport in the proximal portion of the limb and also propelled tracer from the injection site toward the lymphatics. The effect of IPC was evident as soon as external compression therapy began and was similar with either mechanical unit. Residual tracer activity in peripheral lymphatics 20 minutes after application of IPC was higher in the edematous as compared with the normal limbs. FA processing is a new and potentially valuable method to evaluate the effects of external pneumatic compression in the management of peripheral lymphedema.

Factorial analysis of dynamic lymphoscintigraphy in lower limb lymphoedema.

The purpose of this work is to assess the contribution of factor analysis to the interpretation of dynamic lymphoscintigraphy. Twenty six adult patients with a lower limb lymphoedema and five control patients were studied. The technique involved a 3 mCi lymphovectoscint* 99Tcm-labelled ammonium sulphur colloid, bilateral pedal subcutaneous injection. Recording consisted of 40 consecutive 1-min frames of both legs followed by static pictures of each leg and pelvis at 40 min and 4 h after injection. The analysis of static pictures allowed a classification of patients into three groups: enhanced pattern, hypoplasia and dermic reflux. To evaluate better the different kinetic components of the sequential pictures, a factorial analysis (FA) software package was used. FA processing enabled the extraction from the dynamic series of different dynamic components, factorial curves and their associated factorial images which represent the anatomic structures with the same temporal behaviour. In each pattern the results of dynamic recording and of three FA factors were compared. FA provides an analytical temporal and spatial description of the dynamic sequence by extracting the different kinetic components; it yields a better differentiation of vessel and interstitium activity. It also provides some clues towards the understanding of oedema physiopathology and is of potential interest for the management of lymphoedema.

Therapeutic effectiveness of iodine-131 MIBG metastases of a nonsecreting paraganglioma.

This case report describes the treatment of the bone metastases of a nonfunctioning sympathetic paraganglioma, with [131I]MIBG. After primary tumor excision and unsuccessful external radiotherapy, the patient received three therapeutic doses of [131I]MIBG, resulting in a reduction of the number and volume of metastases, and an improvement of the general condition. At 3 yr following [131I]MIBG therapy, the patient remained in remission. [131I]MIBG appears to be an efficient and safe agent for treating malignant sympathetic paraganglioma.

Radioiodinated meta-iodobenzylguanidine uptake in medullary thyroid cancer. A French cooperative study.

Fifty meta-iodobenzylguanidine (MIBG) scintiscans were performed in three groups of medullary thyroid cancer (MTC) patients. Group 1 (n = 11) included treated patients with normal calcitonin levels; Group 2 (n = 24) included patients with elevated calcitonin levels due to sporadic and isolated MTC; Group 3 (n = 15) included patients with elevated calcitonin levels due to familial MTC or multiple endocrine neoplasia Type IIA syndrome (MEN). In Group 1 three pheochromocytoma were depicted by MIBG scintiscan. In Group 2 MTC was seen in a small number of patients (3 of 24). In Group 3, besides adrenal hyperplasia and pheochromocytoma four patients, MIBG scintigraphy showed where MTC had localized and spread in almost half of patients (7 of 15). MIBG uptake occurred in patients with relatively high calcitonin level (greater than 0.6 nmol/l). These data indicate that in patients with familial MTC or MEN syndrome, MIBG scintiscan can be useful not only in detecting associated pheochromocytoma, but also in showing MTC.