Fibercolonoscopy for the diagnosis of granulomatous colitis. (1/42)

In order to clarify the significance of fibercolonoscopy in diagnosis of granulomatous colitis, the pathological, radiological, endoscopic and clinical differences between granulomatous colitis and ulcerative colitis were investigated on 12 cases of granulomatous colitis and 23 cases of ulcerative colitis, which underwent colonic resections between 1954 and 1973. The comparative studies have revealed considerable differences in their characteristic features besides the overlapping spectrum. As a consequence of assessments made of the various diagnostic techniques as to usefulness in differential diagnosis between the two colitis conditions, it was noted that fibercolonoscopy and biopsy under direct vision, when applied in conjunction with the conventional procedures, readily facilitate the differential diagnosis without any surgical intervention in all aspects, excepting the depth of inflammatory involvement and the presence of lymphangiectasia. However, the depth of the inflammed lesion as well as lymphangiectasia can also be estimated indirectly. Therefore, it seems that it is possible to make the differential diagnosis between the two colitis conditions even prior to colectomy, with the exception of atypical forms which represent overlapping findings.  (+info)

Lymphangiectatic fibrolipomatous polyp of the palatine tonsil. (2/42)

A rare benign polypoid tumor of the right palatine tonsil is described in a 23 year old male. It contained dilated lymphatic channels surrounded by fibrous tissue and foci of mature fat. The features of this lesion add support to the hypothesis that benign tumors of tonsil may be hamartomas of tonsil rather than true neoplasms. The differential diagnosis of polypoid lesions of the tonsil is discussed.  (+info)

Treatment of symptomatic primary chylous disorders. (3/42)

PURPOSE: Primary chylous disorders (PCDs) are rare. Rupture of dilated lymph vessels (lymphangiectasia) may result in chylous ascites, chylothorax, or leakage of chyle through chylocutanous fistulas in the lower limbs or genitalia. Chyle may reflux through incompetent lymphatics, causing lymphedema. To assess the efficacy of surgical treatment, we reviewed our experience. METHODS: The clinical data of 35 patients with PCDs treated between January 1, 1976, and August 31, 2000, were reviewed retrospectively. RESULTS: Fifteen men and 20 women (mean age, 29 years; range, 1 day-81 years) presented with PCDs. Sixteen (46%) patients had chylous ascites, and 19 (54%) had chylothorax (20 patients), and of these, 10 (29%) had both. In 16 patients, reflux of chyle into the pelvic or lower limb lymphatics caused lymphedema (14, 88%) or lymphatic leak through cutaneous fistulae (11, 69%). Presenting symptoms included lower-limb edema (19, 54%), dyspnea (17, 49%), scrotal or labial edema (15, 43%), or abdominal distention (13, 37%). Primary lymphangiectasia presented alone in 23 patients (66%), and it was associated with clinical syndromes or additional pathologic findings in 12 (yellow nail syndrome in 4, lymphangiomyomatosis in 3, unknown in 3, Prasad syndrome (hypogammaglobulinemia, lymphadenopathy, and pulmonary insufficiency) in 1, and thoracic duct cyst in 1). Twenty-one (60%) patients underwent 26 surgical procedures. Preoperative imaging included computed tomography scan in 15 patients, magnetic resonance imaging in 3, lymphoscintigraphy in 12, and lymphangiography in 14. Fifteen patients underwent 18 procedures for chylous ascites or pelvic reflux. Ten (56%) procedures were resection of retroperitoneal/mesenteric lymphatics with or without sclerotherapy of lymphatics, 4 (22%) were lymphovenous anastomoses or grafts, 3 (17%) were peritoneovenous shunts, and 1 (6%) patient had a hysterectomy. Six patients underwent eight procedures for chylothorax, including thoracotomy with decortication and pleurodesis (4 procedures), thoracoscopic decortication (1 patient), ligation of thoracic duct (2 procedures), and resection of thoracic duct cyst (1 patient). Postoperative mean follow-up was 54 months (range, 0.3-276). Early complications included wound infections in 3 patients, elevated liver enzymes in 1, and peritoneovenous shunt occlusion with innominate vein occlusion in 1. All patients improved initially, but four (19%) had recurrence of symptoms at a mean of 25 months (range, 1-43). Three patients had postoperative lymphoscintigraphy confirming improved lymphatic transport and diminished reflux. One patient died 12 years postoperatively, from causes unrelated to PCD. CONCLUSIONS: More than half of the patients with PCDs require surgical treatment, and surgery should be considered in patients with significant symptoms of PCD. Lymphangiography is recommended to determine anatomy and the site of the lymphatic leak, especially if lymphovenous grafting is planned. All patients had initial benefit postoperatively and two thirds of patients demonstrated durable clinical improvement after surgical treatment.  (+info)

The lungs in lymphangiomyomatosis and in tuberous sclerosis. (4/42)

Two cases of pulmonary lymphangiomyomatosis (PL) are described and 33 other cases from the literature are reviewed. These are compared with one case of tuberous sclerosis with pulmonary involvement (PTS) and 32 other cases from the literature. There are no differences in lung function between these two conditions, both of which show airways obstruction associated with diffuse radiological lung changes. There are, however, both clinical and radiological differences and also differences in the distribution of the lesions and the histological location of the excessive smooth muscle; these indicate that PL and PTS are probably different entities and not polar forms of one condition. Finally, the strictly female incidence of PL suggests a sex-linked disorder, and it is postulated that this may be related to congenital pulmonary lymphangiectases.  (+info)

A case of primary hyperparathyroidism due to an oxyphil cell adenoma. (5/42)

A 59-year-old woman with primary hyperparathyroidism was found to have a parathyroid adenoma behind the left clavicle. Preoperatively, it appeared as a hypoechoic mass on ultrasonography, as a hot nodule on thallium scintigraphy, and as a high signal on T2-weighted magnetic resonance imaging. Histological, immunohistochemical and ultrastructural studies of the surgically resected tumor revealed a parathyroid adenoma composed mainly of oxyphil cells with production of a parathyroid hormone. Moreover, a multilocular lesion of lymphangiectasia was contained. Hypercalcemia was alleviated postoperatively. These observations corroborated a functioning parathyroid oxyphil cell adenoma. This is the first case report of functioning oxyphil cell adenoma of the parathyroid gland with lymphangiectasia in Japan.  (+info)

Acquired lymphangiectasis after breast conservation treatment for breast cancer: report of a case. (6/42)

Acquired lymphangiectasis is a dilatation of lymphatic vessels that can result as a complication of surgical intervention and radiation therapy for malignancy. Acquired lymphangiectasis shares clinical and histological features with the congenital lesion, lymphangioma circumscriptum. Diagnosis and treatment of these vesiculobullous lesions is important because they may be associated with pain, chronic drainage, and cellulitis. We describe patient who had these lesions after treatment for cancer. Although a number of treatment options are available, we have found cryosurgery and electrocautery to be particularly effective.  (+info)

Primary pulmonary lymphangiectasia in infancy and childhood. (7/42)

Primary pulmonary lymphangiectasia (PPL) is a rare disorder of unknown aetiology characterised by dilatation of the pulmonary lymphatics. PPL is widely reported to have a poor prognosis in the neonatal period and little is known about the clinical features of patients who survive the newborn period. The current authors report the outcome in nine patients diagnosed in infancy with PPL over a 15-yr period at a single university-based hospital clinic and followed for a median of 6 yrs. Although all of the patients initially experienced respiratory distress, respiratory symptoms improved in most patients after infancy and were notably better by the age of 6 yrs. Many patients had poor weight gain in the first years of life, which eventually improved. Radiological scans showed progressive resolution of neonatal infiltrates, but were characterised by hyperinflation and increased interstitial markings in older children. Most patients had evidence of bronchitis and grew pathogenic organisms from quantitative bronchoalveolar lavage culture. Pulmonary function tests showed predominantly obstructive disease that did not deteriorate over time. In conclusion, these results suggest that primary pulmonary lymphangiectasia does not have as dismal a prognosis as previously described and symptoms and clinical findings improve after the first year of life.  (+info)

Hypoplasia of the small pulmonary arteries in hypoplastic left heart syndrome with restrictive atrial septal defect. (8/42)

BACKGROUND: Restrictive atrial septal defect (ASD) (including intact atrial septum [IAS]) has been reported to be a risk factor that negatively impacts survival in hypoplastic left heart syndrome (HLHS). Although lymphangiectasia and "arterialization" of the veins of the lung in HLHS with restrictive ASD have been reported, they cannot fully explain the high mortality. We have introduced a new method of evaluating the development of the pulmonary vasculature in histological sections and used it to assess patients' lungs. We tested the hypothesis that the small pulmonary arteries (SPA), which are pulmonary arteries in a histological section whose radii are approximately 25 microm to 250 microm, in HLHS with restrictive ASD are hypoplastic, but that the alveoli are not, to elucidate the mechanism underlying the poor outcome of these patients. METHODS AND RESULTS: Fourteen HLHS patients between 1 day and 40 days of age were studied. In 8 cases, the ASD was restrictive [R(+) group], and in the other 6 cases it was not [R(-) group]. Specimens from 12 autopsies of cases with no congenital heart or pulmonary disease were examined as a control group (C group). As a novel histological parameter, we assessed the size of SPA in relation to the size of accompanying bronchioles to identify SPA underdevelopment. To evaluate the development of alveoli and interstitial tissue, radial alveolar counts (RAC), which reflect alveolar maturity and complexity, were also performed. Statistical comparisons between groups were made by analysis of covariance with age as a covariant factor. When the radius of the accompanying bronchiole was 100 microm, the radius of the SPA was 34.0+/-10.8 microm in the R(+) group, and significantly lower than the 46.6+/-8.5 microm in R(-) group (P=0.0022) and 70.5+/-8.4 microm in the C group (P<0.0001). The RAC was in 3.5+/-0.9 in the R(+) group, 3.4+/-0.6 in the R(-) group, and 3.7+/-0.9 in the C group (no significant differences between groups). CONCLUSIONS: The SPA in HLHS with restrictive ASD were underdeveloped compared with the SPA in HLHS with nonrestrictive ASD and the controls, but their alveoli were not hypoplastic. Based on these results, it is speculated that SPA hypoplasia may be responsible for the poor outcome of HLHS with restrictive ASD.  (+info)