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PubMed1976

[Nevus telangiectasis of the facial skin: possibilities and problems of plastic surgery].

If a portwine stain (capillary hemangioma) or a cavernous hemangioma of the facial skin is not camouflaged by cosmetics, surgical excision and full-thickness skin grafting appears to be the method of choice. Exact planning (donor areas) and perfected technique are prerequisites for results in which the grafted skin matches in color, texture, contour and thickness the unaffected facial side.

Facial Neoplasms↗
PubMed1993

Hereditary hemorrhagic telangiectasis involving the ampulla of Vater presented with recurrent gastrointestinal bleeding.

We report a Chinese lady with hereditary hemorrhagic telangiectasia who presented with multiple episodes of gastrointestinal bleeding requiring transfusion. Gastroscopy, colonoscopy, isotope-labeled red blood cell scan, and selective arteriography were performed, but could not locate the source of bleeding. A bleeding lesion in the ampulla of Vater was seen when endoscopic retrograde cholangiopancreatography was attempted. She had an uneventful pylorus-preserving Whipple's operation with no further evidence of gastrointestinal bleeding. This is the first case report to show that the vascular ectasia of patients with hereditary hemorrhagic telangiectasia could manifest at the ampulla of Vater.

Ampulla of Vater↗
PubMed1997

[Hereditary hemorrhagic telangiectasis and the development of an arteriovenous fistula after 24 years].

The authors describe a unique pulmangiographic picture taken 24 years after the first angiographic examination made on account of repeated haemoptysis associated with Rendu-Osler-Weber s disease. Angiography revealed enlargement of the aneurysm in the lungs and dilatation of the afferent and efferent vessel of the aneurysm. The only treatment of pulmonary arteriovenous fistulas is surgical.

Arteriovenous Fistula↗
PubMed2004

Partial breast irradiation with interstitial 60CO brachytherapy results in frequent grade 3 or 4 toxicity. Evidence based on a 12-year follow-up of 70 patients.

PURPOSE: To investigate the radiation-induced toxicity and cosmesis of brachytherapy (BT) alone in early stage breast cancer. METHODS AND MATERIALS: A total of 70 women diagnosed with Stage I or II breast carcinoma participated in a BT study at the Municipal Oncoradiological Center, Uzsoki Hospital, Budapest, Hungary, between November 1987 and June 1992. They had undergone breast-conserving surgery with an unknown surgical margin. The postoperative tumor bed irradiation was performed with interstitial (60)Co sources with an active length of 4 cm, with 10-mm center-to-center spacing arranged in a single plane. The median number of inserted sources was 5 (range, 2-8), with a linear activity of 133-137 MBq/cm at the beginning of the study. The 50 Gy delivered dose at 5 mm from the surface of the (60)Co sources was administered during 10-22 h to the virtual postoperative lumpectomy cavity (i.e., plane). For radiobiologic considerations, the clinical target volume (CTV) was calculated retrospectively with a 10-mm safety margin, resulting in a 72-cm(3) median CTV (range, 36-108 cm(3)) irradiated with a reference dose of 28 Gy. In the assessment of the skin and subcutaneous toxicity, the RTOG late radiation morbidity scoring system was applied. The radiosensitivity of the cultured fibroblasts was determined by clonogenic assay to check whether individual radiosensitivity played a role in the development and course of radiation-induced side-effects. RESULTS: The median follow-up was 12 years (range, 10-15 years). The population of the final study (34 cases) comprised all survivors with tumor-free breasts (27 cases) and patients with breasts erroneously ablated/excised for misinterpreted radiation-induced sequelae (7 patients). A total of 97% of the cohort (33/34) had grade > or =2, and 59% (20/34) had grade > or =3 radiation-induced toxicity. By the end of the follow-up, 85% of the patients experienced Grade > or =2 telangiectasis and 41% had Grade 3 telangiectasis. Eighty-eight percent had fibrosis of some form, and 35% had grade > or =3 fibrosis. Forty-one percent of the cohort displayed fat necrosis, which was always accompanied by Grade > or =3 fibrosis or telangiectasis. The cosmetic results were poor in 50% (17/34) of the patients. The radiosensitivity of the fibroblasts was increased in only 2/24 patients (8% of the investigated cases, in agreement with data published for the general population). Comparisons of our fibrosis prevalence data with those of others allowed an estimate of 0.47 h(-1) for the rate of recovery of DNA damage in the fibroblasts. CONCLUSIONS: Interstitial (60)Co BT of the breast tumor bed alone with a limited CTV (median, 72 cm(3)) and a total dose of 28 Gy is associated with a high rate (59%) of grade > or =3 radiation-induced toxicity and a high rate (50%) of poor cosmetic outcome at the end of a median follow-up of 12 years. A relatively high BT dose rate (1.3-2.8 Gy/h) applied during a short overall treatment time (10-22 h) and a possible geographic miss (close to skin implantation) might have contributed to the development of these sequelae.

Adult↗
PubMed1987

Retinal vascular abnormalities in facioscapulohumeral muscular dystrophy. A general association with genetic and therapeutic implications.

Because of occasional reports of exudative retinal detachment with facioscapulohumeral muscular dystrophy (FSH) and deafness, we sought to determine by fluorescein angiography whether there is any general relationship between FSH muscular dystrophy and retinal vascular disease. Peripheral retinal capillary abnormalities, comprising telangiectasis, closure, leakage and microaneurysm formation, were demonstrated by angiography in 56 out of 75 individuals with clinical or genetic evidence of FSH. Only 3 patients had relevant ophthalmoscopic abnormalities of the posterior pole and in only 1 was there consequent visual loss. This study included one FSH family in which the propositus was treated for exudative retinopathy and 13 other subjects had telangiectasis, and 8 cases (including 3 parents of apparently 'sporadic' FSH cases) in which fluorescein angiography confirmed the abnormal genotype, even though clinical examination of skeletal muscle revealed no clear abnormality. There was no correlation between the severity of the muscle disease and the extent of the retinal vascular abnormality. Visual complications of telangiectasis, although rare, may present early in life and before there is overt evidence of muscle disease. Since visual loss may be preventable, ophthalmic examination should be undertaken on infants at risk of having the abnormal gene. The findings support the hypothesis that retinal capillary abnormalities are an integral part of the FSH muscular dystrophy syndrome and raise the question as to whether analogous capillary abnormalities could be implicated in the pathogenesis of FSH muscle disease.

Adolescent↗
PubMed1984

Retinal telangiectasia, retinoschisis and retinal detachment.

A 32-year-old woman had progressive bilateral peripheral and central retinal telangiectasis with secondary exudative, nonrhegmatogenous, retinal detachment, and secondary retinoschisis. This is unique constellation of clinical findings, retinal telangiectasis itself being an uncommon disease, and usually occurring in young males, unilaterally. Theories for the etiology of this disease process are presented. The disease was successfully treated bilaterally with retinal cryopexy extensively applied to the retinal telangiectasis, both peripheral and paracentral, with both scleral buckling and drainage of copious amounts of thick subretinal exudation.

Adult↗
PubMed2001

[Rendu-Osler-Weber disease. Descriptive study of 17 cases].

OBJECTIVE: To study the prevalence and presentation forms of hereditary hemorrhagic telangiectasia (HHT) among the population in our hospital area. MATERIALS AND METHODS: Descriptive, retrospective study of patients with the diagnosis of HHT from to 1999 at La Fe Hospital, Valencia. Family history, repeated epistaxis, mucocutaneous telangiectasis and visceral lesions, as well as administered treatments, and clinical course were evaluated. RESULTS: Seventeen patients were diagnosed during such time period, with ages ranging from 23 to 80 years. Eight patients had family histories. The most common symptoms included epistaxis and iron-deficiency anemia, followed by muco-cutaneous telangiectasis. Six patients had neurological, five pulmonary, five gastrointestinal, and three hepatic manifestations, usually secondary to vascular malformations at those levels. CONCLUSIONS: The prevalence of the disease maybe higher than previously reported. This disease should be considered in patients with repeated epistaxis and/or iron-deficiency anemia. The presence of telangiectasis that can suggest the diagnosis should be investigated. An early diagnosis of HHT can be useful for the early control of associated visceral malformations. Treatment of HHT should be tailored according to the clinical manifestations of the patient.

Adult↗
PubMed2000

Idiopathic juxtafoveolar retinal telangiectasia: a review and case report.

Idiopathic juxtafoveolar retinal telangiectasis is a group of retinal vascular anomalies characterized by retinal vessel dilation and tortuosity, multiple aneurysm formations, varying degrees of vascular leakage and lipid exudate deposition. Idiopathic juxtafoveolar retinal telangiectasis may occur as a primary disorder (either congenital or acquired), or may be caused or accompanied by other systemic or ocular diseases. The visual prognosis and effectiveness of therapy is dependent upon the etiology of the retinal telangiectasis. Included in this review is a case report, as well as the classification system used to identify idiopathic juxtafoveolar retinal telangiectasia.

Journal Article↗
PubMed2006

Efficacy of commercially available topical cyclosporine A 0.05% in the treatment of meibomian gland dysfunction.

OBJECTIVE: To investigate the efficacy of topical cyclosporine A 0.05% (tCsA) (Restasis, Allergan Pharmaceuticals) in the treatment of meibomian gland dysfunction (posterior blepharitis). METHODS: Thirty-three patients with symptomatic meibomian gland dysfunction were randomized in a prospective study to either tCsA or placebo (Refresh Plus preservative-free artificial tears), 2 times daily for 3 months. They were evaluated at baseline and at 1, 2, and 3 months for subjective symptoms and objective signs including meibomian gland inclusions, lid margin vascular injection, tarsal telangiectasis, fluorescein staining, tear breakup time, and Schirmer scores. RESULTS: Twenty-six patients completed the study. All patients were tested for ocular symptoms, lid margin vascularity, tarsal telangiectasis, meibomian gland inclusions, tear breakup time, and fluorescein staining. At the 3-month visit, the tCsA group showed a greater improvement in ocular symptoms than the placebo group, but this difference was not statistically significant. At the 3-month visit, several objective examination findings were statistically significantly (P < 0.05) improved in the tCsA group compared with the placebo group. These differences included lid margin vascular injection, tarsal telangiectasis, and fluorescein staining. The most significant finding (P = 0.001) was the greater decrease in the number of meibomian gland inclusions in the tCsA group compared with the placebo group. CONCLUSIONS: Topical CsA may be helpful in the treatment of meibomian gland dysfunction (posterior blepharitis). Topical CsA did not induce an improvement in the symptoms, but it did decrease the number of meibomian gland inclusions in patients with meibomian gland dysfunction.

Adult↗
PubMed1985

Relationship between nailfold capillary abnormalities and organ involvement in systemic sclerosis.

Nailfold capillary abnormalities in 42 consecutive patients with systemic sclerosis were studied by wide field capillary microscopy, and capillary abnormalities were correlated with organ involvement. Twenty-eight patients hd diffuse skin disease, and 14 had the CREST variant of systemic sclerosis (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasis) with anticentromere antibodies. Nailfold capillary enlargement and loss were graded from photographs. There was no correlation between the severity of either nailfold capillary loss or enlargement and duration of disease, number of organ systems involved, or acroosteolysis. The presence of telangiectasis correlated with extreme capillary enlargement (P less than 0.025). Based on these findings it can be concluded that nailfold capillary changes in individual patients with systemic sclerosis are not useful in predicting organ involvement.

Adult↗
PubMed1979

Gill lesions in Cichlid fishes after intoxication with the insecticide Fenthion.

The toxic effects of Fenthion on the gills of Cichlid fishes were investigated. In Herotilapia multispinosa hyperplasia and separation of the respiratory epithelium, lamellar telangiectasis and thrombosis occurred. The lamellar epithelium of surviving fish regenerated in all cases. In Tilapia leucosticta no telangiectasis was found, but all the secondary lamellae were fused due to the severe hyperplasia of the gill epithelium.

Animals↗
PubMed2003

Late skin and subcutaneous soft tissue changes after 10-gy boost for breast conserving therapy.

PURPOSE: To evaluate the influence of boost irradiation for breast conserving therapy on skin and subcutaneous tissue. MATERIALS AND METHODS: Between 1989 and 1995, 468 patients were treated with breast conserving surgery (quadrantectomy or wide excision with axillary dissection) followed by 50 Gy whole breast irradiation. Among them, fifty-eight patients with positive or close margins were treated with 10 Gy external beam boost irradiation. Skin and subcutaneous soft tissue changes during 5-years of follow-up were examined by inspection and palpation and evaluated using the BCT follow-up form based on EORTC late effect toxicity scoring. RESULT: Four percent (20/468) of the patients showed grade 2 late changes in skin and soft tissue. Four of them had skin telangiectasis, which was limited to within the boost field. Boost irradiation had no definite influence on other late changes. Patients' age, extent of surgery, and pT size had no significant relation to the late changes. The cosmetic score 5 years after BCT was not significantly different between the patients with and without boost irradiation. CONCLUSION: Although 10 Gy boost irradiation after 50 Gy whole breast irradiation increased skin telangiectasis, the late skin and soft tissue changes caused by the boost irradiation were generally mild and there was no substantial deterioration of cosmetic outcome.

Adenocarcinoma↗
PubMed2004

Spontaneous improvement in late rectal mucosal changes after radiotherapy for prostate cancer.

PURPOSE: To describe the natural history of mucosal changes, in particular the development of telangiectases in the rectum, after radiotherapy (RT) for prostate cancer. METHODS AND MATERIALS: Twenty patients undergoing local-field, nonconformal RT for prostate cancer underwent flexible sigmoidoscopy every 6 months for up to 3 years after RT completion. Telangiectasis was scored as absent, single, multiple, or multiple confluent. The site and circumferential extent were also documented. The patients filled in a diary to document the symptoms they were experiencing, including rectal bleeding. RESULTS: Of the 20 patients, 12 developed multiple telangiectases, and 10 of these had rectal bleeding, which in all cases was mild. Telangiectasis was most commonly seen between 4 (anorectal junction) and 8 cm from the anal verge. In 5 patients, 4 of whom had multiple telangiectases, spontaneous resolution occurred. CONCLUSION: Late radiation effects in the rectum do not appear to be permanent in all cases. This first prospective prolonged evaluation may provide an explanation for the observation that rectal bleeding resolves in a large proportion of patients with mild symptoms after RT. The reasons for improvement in the late radiation changes in the rectum compared with the permanent changes seen in organs such as the skin are unknown.

Follow-Up Studies↗
PubMed1996

Myelinated nerve fibers and retinal vascular abnormalities.

BACKGROUND/PURPOSE: Abnormal retinal vessels may develop in a region of myelinated nerve fibers, and these vessels may cause vitreous hemorrhages. METHODS: The clinical histories of seven patients with retinovascular abnormalities in a patch of myelinated nerve fibers are presented. None of the reported patients had other evidence of systemic disease. The cases were traced by a multicentric retrospective study. RESULTS: Retinal vascular abnormalities ranged from mild telangiectasis to frank neovascularization, with or without obstruction of the capillary network and signs of branch artery and vein occlusion. Age at diagnosis ranged from 15 to 68 years. Vitreous hemorrhages occurred in the four youngest patients and occurred at 15, 27, 27, and 43 years of age. Laser photocoagulation was applied in three patients and vitrectomy was performed in one. CONCLUSION: The authors' findings suggest that the abnormal structure of the myelinated nerve fibers and the thickened nerve fiber layer of the affected portions of retina may play a role in the onset of retinal vascular abnormalities and eventually cause telangiectasis, branch artery and vein occlusion, neovascularization, and vitreous hemorrhages. This suggestion is based on the absence of other causes of neovascularization or vitreous hemorrhage in all seven patients, and on the relatively young age of four of the patients with this association.

Adolescent↗
PubMedDate not supplied

New findings in the Parry-Romberg syndrome: a case report.

PURPOSE: To describe further findings in the Parry-Romberg syndrome that might contribute towards a better understanding of the disease. DESIGN: Case report. METHODS: The clinical history of a patient with the Parry-Romberg syndrome was studied, and fluorangiography and echography were carried out. RESULTS: Clinical observation revealed classic hemifacial atrophy; fluorescein angiography showed telangiectasis, light staining of the retina, and leakage from the largest telangiectasis and the optic disc vessels; echography showed shrinkage of the eyeball and thinning of the extraocular muscles. CONCLUSIONS: Enophthalmos in this disease is caused not only by progressive fat atrophy but also by shrinkage of the eyeball and thinning of the extraocular muscles; the shrinkage of the eyeball helps explain the presence of choroidal and retinal folding and hyperopia, confirming the primary involvement of connective tissue in this disease.

Child↗
PubMed1996

Coats' disease and central nervous system venous malformation.

Primary retinal telangiectasis or Coats' disease is a non-hereditary retinal vascular abnormality consisting of incompetent telangiectatic and aneurysmal retinal vessels. It is characteristically found unilaterally in boys and occasionally may be associated with other systemic disorders. The authors report the first case of primary retinal telangiectasis with a concomitant diffuse central nervous system venous abnormality.

Aneurysm↗
PubMed1999

Diagnostic imaging of angiographically occult vascular malformations.

Occult vascular malformations of the central nervous system include cavernous malformations, capillary telangiectasis, and developmental venous anomalies (DVAs). Cavernous malformations are prone to multiple small hemorrhages and may enlarge with time. They have a heterogeneous appearance on CT and MR imaging with areas of hemorrhage in various states of evolution and sometimes calcifications. Developmental venous anomalies and capillary telangiectasis are usually incidental findings on imaging studies. They usually are not the cause of pathology by themselves, although DVAs can be associated with cavernous malformations in 8% to 33% of cases.

Arteriovenous Malformations↗
PubMedDate not supplied

Selective photocoagulation in Coats' disease: ten-year follow-up.

PURPOSE: The diagnostic hallmark of Coats' disease is development of "light bulb" telangiectasis in the retinal periphery, leading to posterior pole intraretinal and subretinal exudation. Even after complete obliteration of all abnormal vessels and resorption of all exudates, follow-up examinations are mandatory for several years. METHODS: We retrospectively analyzed the charts, pictures and/or fluorescein angiographies of a series of 32 consecutive patients, in which the diagnosis of Coats' disease was made. All patients underwent selective photocoagulation of the telangiectasis using a yellow-dye laser. Efficacy of treatment was monitored with drawings and/or fluorescein angiographies. RESULTS: All our patients had unilateral disease, with macular involvement and exudative retinal detachment. Visual acuity improved in one patient, decreased from light perception to blindness in another, and was unchanged in the remainder. CONCLUSIONS: This is the first report of anatomical benefits from treatment with a yellow-dye laser (i.e., selective photocoagulation without cryotherapy or drainage) despite the presence of a severe form of Coats' disease. Our young Coats' patients responded quickly to treatment. With successful obliteration of the abnormal vasculature, exudates began to absorb in about 4 to 8 weeks although, in some cases, 10 to 12 months were needed to obtain resolution of the exudative detachment. Poor visual outcome of 20/100 or worse was common. In Coats' patients, the key issue is continuous control and long-term follow-up.

Child↗