Concurrent diffuse dermal angiomatosis and granuloma inframammary adultorum of the breast.

Diffuse dermal angiomatosis (DDA) is a cutaneous reactive angiomatosis. Typically presenting as ulcerated, erythematous, violaceous, or purpuric plaques on the breast or lower extremities, DDA is believed to be a reaction to tissue ischemia. Granuloma inframammary adultorum (GIA) is a type of irritant dermatitis of multifactorial etiology, clinically presenting as papules and nodules. Herein, we report an interesting rash presenting as fungiform papulonodules overlying a large violaceous plaque on the left breast. Biopsy revealed an exuberant epidermal proliferation and a diffuse and deep dermal proliferation, consisting of small slit-like blood vessels in between collagen bundles. In light of these clinical and histopathologic findings in the setting of an indurated plaque on a pendulous breast of a woman with multiple risk factors for local tissue ischemia, a diagnosis of concurrent diffuse angiomatosis of the breast (DDAB) and GIA was rendered. This case highlights the critical importance of clinicopathologic correlation in the diagnosis of multiple diagnostic entities.

Primitive retinal vascular abnormalities: tumors and telangiectasias.

Primitive retinal vascular abnormalities are benign conditions of the retinal circulation that comprise vascular tumors and telangiectasias. The principal vascular tumors of the retina include retinal capillary hemangioma, cavernous hemangioma of the retina, racemose hemangiomatosis of the retina and retinal vasoproliferative tumor, while primary retinal telangiectasias include Coats' disease, Leber's miliary aneurysms and idiopathic juxtafoveal telangiectasias. In most cases, these alterations result in significant visual impairment due to exudation determined by the structural abnormalities of the retinal vasculature. The aim of this review is to assess the different clinical and diagnostic features of the single pathological entities and to discuss the available treatment modalities including the onset of intravitreal antivascular endothelial growth factor therapy.

[Retinal angiomatous proliferations]. / Retinale angiomatöse Proliferationen.

Retinal angiomatous proliferations, also known as type 3 neovascularisation, are a common entity amongst patients with age-related macular degeneration. Their prevalence is being estimated at around 12-15% in this group of patients. Certain funduscopic signs like an extravofeal, intraretinal haemorrhage, cystoid macular oedema or a retinal anastomosis of the lesion are considered to be pathognomonic. Verification of the diagnosis should be based on ICG angiography, although OCT is gaining popularity. Interestingly, RAP lesions seem to have distinctive demographic characteristics and respond differently to established therapies, differentiating them from regular type 1 or type 2 neovascularisation. Current therapies of choice are VEGF inhibitors. Nonetheless, combination therapies, combining different approaches like anti-VEGF treatment and photodynamic therapy, have received more attention recently.

[Treatment of cutaneous vertebral medullary angiomatosis].

OBJECTIVE: Cutaneous vertebral medullary angiomatosis, also known as Cobb's syndrome, is a part of spinal arteriovenous metameric syndromes (SAMs), is derived from the vascular malformation triad of skin, bone, and spinal cord involvement. It is poorly managed with current treatment modalities. We reviewed the treatment of Cobb's syndrome series and summarized the experiences. METHODS: A total of 61 cases of Cobb's syndrome with spinal cord dysfunction were treated at our department from February 2003 to December 2007. The treatment followed the same strategy: First step-investigating the pathogenic mechanisms by symptom onset, MRI, angiography and the response to initial treatment. Second step-treating the problematic parts of the lesions with embolization alone, surgery alone or combination of both. Forty-eight cases were embolized, 3 cases treated with surgery and 10 cases treated with a combination of both. Nidus involving pathogenic mechanism for spinal cord were eliminated, completely and nearly disappeared in 36 cases and partially in 25 cases. All patients were followed up regularly. RESULTS: Among these 61 cases, the pathogenic mechanisms were identified as hemorrhage, mass effect, ischemia and venous hypertensive myelopathy. Two or more mechanisms could coexist in the same patient. More than 1-year's spinal cord function follow-up showed: excellent in 9 cases, good in 26, fair in 23 and worse in 3. CONCLUSION: Not all lesions of Cobb's syndrome can be or should be cured anatomically. Pathogenic mechanism should be analyzed carefully and the treatment should focus on the special cord-affecting targets. Long-term improvement or stabilization may be achieved.

Diffuse dermal angiomatosis.

Diffuse dermal angiomatosis (DDA) is a benign and rare acquired, cutaneous, reactive, vascular disorder. We report a rare case of a 43-year-old man who presented with a large (15-cm diameter), indurated, hyperpigmented plaque covering the left buttock for 6 years. This report further discusses DDA with a review of the literature, including its classification, epidemiology, pathophysiology, etiology, histopathology, differential diagnosis, and current therapeutic approaches.

Kasabach-Merritt syndrome caused by giant hemangiomas of the spleen in patients with Proteus syndrome.

Proteus syndrome is a rare hamartomous disorder with highly variable manifestations of disproportionate overgrowth of body parts, epidermal nevi and vascular malformations. We describe two patients who had typical clinical and imaging Proteus syndrome features. One of most characteristic findings in these two patients was that they had giant hemangiomas of the spleen that caused Kasabach-Merritt syndrome, demonstrating clinical bleeding and disseminated intravascular coagulation laboratory alterations. The present two cases are the first report of this complication in Proteus syndrome. The patients' bleeding tendency and hemostatic defects were completely corrected after they successfully underwent splenectomy.

Solitary mediastinal angiomatosis: report of two cases and review of the literature.

Angiomatosis is a rare benign vascular lesion, usually seen in females in the first two decades of life. It commonly involves the lower extremities. Angiomatosis of mediastinum is very rare and we report two such cases with a review of the literature on solitary mediastinal angiomatosis. Both of our patients were female, aged 34 and 57 years. One patient presented with left-sided subcutaneous supraclavicular swelling. Magnetic resonance imaging showed a mass extending from the left upper mediastinum to the left supraclavicular area. The other patient presented with pleural effusion. Computed tomography of the chest disclosed an infiltrative mass in the mediastinum. Both patients were treated by excision of the lesions, histologically confirmed to be angiomatosis. Despite the rarity of mediastinal angiomatosis, this clinicopathological entity must be taken into consideration when the treatment of mediastinal tumours is planned.

COBB syndrome treated by staged intravascular embolisation and surgery.

A 28-year old Malay man with evidence of an upper motor neuron cord lesion was diagnosed to have a C7 to T2 spinal arterio-venous malformation and associated cutaneous vascular lesion. He finally agreed for treatment after 5 years of progressive spastic right lower limb weakness leading to inability to mobilize. A two staged intravascular procedure was done followed by surgery with recovery of ASIA impairment scale grade B.

Treatment of retinal angiomatous proliferation in age-related macular degeneration: a series of 104 cases of retinal angiomatous proliferation.

OBJECTIVE: To report the management of retinal angiomatous proliferation (RAP), a recently described intraretinal neovascular lesion occurring in age-related macular degeneration. METHODS: This was a retrospective review of consecutive patients with age-related macular degeneration who underwent treatment of RAP from January 1, 2000, through January 31, 2003. Inclusion criteria were age 55 years or older, signs of age-related macular degeneration, and diagnosis of RAP based on dynamic indocyanine green angiography. Baseline angiograms were reviewed and RAP was classified into the following 3 stages: stage 1, intraretinal neovascularization, early stage; stage 2, subretinal neovascularization, middle stage; and stage 3, choroidal neovascularization, late stage. Treatment and concomitant treatment results were assessed separately for each RAP stage. The clinical data were statistically analyzed (chi2 test and analysis of variance) for 2 main outcome measures--complete obliteration of the lesion and final visual acuity. RESULTS: Eighty-one patients (99 eyes) with 104 RAPs were identified. Forty-two lesions were at stage 1, 42 at stage 2, and 20 at stage 3. The following 5 treatments were performed: direct laser photocoagulation of the vascular lesion, laser photocoagulation of the feeder retinal arteriole, scatter "gridlike" laser photocoagulation, photodynamic therapy, and transpupillary thermotherapy. Complete obliteration of RAP was achieved in about 24 (57.1%) of the stage 1 lesions (direct laser photocoagulation of the vascular lesion, 73% success rate; photodynamic therapy, 45%), 11 (26.2%) of the stage 2 lesions (scatter gridlike laser photocoagulation, 38% success rate; direct laser photocoagulation of the vascular lesion, 17%), and only 3 (15.0%) of stage 3 lesions (P = .001). Predictive factors with a significant effect on final visual acuity were initial visual acuity (P = .003) and early lesion stage (P = .04). Best final visual acuity was 0.41 (mean, direct laser photocoagulation of the vascular lesion in stage 1) and 0.39 (mean, photodynamic therapy in stage 1), with a mean decrease of 2.5 and 3 lines from baseline, respectively. CONCLUSIONS: Treatment of RAP remains difficult. Early detection of the lesion and subsequent direct conventional laser photocoagulation seems to be associated with better anatomical and functional outcome. Once the vascular complex is well established, anatomical closure is rarely achieved. Further study is warranted to assess the long-term efficacy and the need for re-treatment.

Combined surgical ablation and intravitreal triamcinolone acetonide for retinal angiomatous proliferation.

PURPOSE: Neovascular age-related macular degeneration (ARMD) with retinal angiomatous proliferation (RAP) has a poor natural history and the efficacy of any treatment has not yet been established. The authors describe a combined surgical treatment. METHODS: A 76-year-old woman presented with a best-corrected visual acuity (BCVA) of 20/600 in the right eye and macula with stage 3 RAP as identified by fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT). After a standard three-port pars plana core vitrectomy (PPV), endodiathermy of the arteriolar and venous feeder vessels of each lesion was performed, intraretinal RAP feeder vessels were cut with manual vertical intraocular scissors, and 0.1 mL of triamcinolone acetonide (TAAC) was injected intravitreally. At 1 and 4 weeks and at the sixth month, the patient underwent a complete eye examination, FA, ICGA, and OCT to assess outcomes and complications. RESULTS: Six months later, BCVA was stable at 20/300, intraocular pressure was 15 mmHg, anterior segment and vitreous cavity were clear without evidence of TAAC granules, and retina was attached. FA and ICGA showed a complete occlusion of the RAP and absence of leakage or ischemia and OCT demonstrated decreased macular thickness with resolution of both intraretinal edema and pigment epithelium detachment, and the restoration of the normal macular profile. At the end of follow-up, the authors did not observe any ocular or systemic complication. CONCLUSIONS: Surgical approach to RAP stage 3 with intravitreal injection of 4 mg of TAAC was safe and anatomically effective.

Uterine stromatosis: a report of 33 cases.

Uterine stromatosis is an uncommon intramyometrial lesion. The present study reviews the clinicopathologic aspects of 33 cases, 9 of which were treated at The Johns Hopkins Hospital, whereas 24 were referred to the gynecologic pathology laboratory. In the series it was noted that progesterone therapy was able to control the spread of the condition and to cause regression of extrauterine disease for periods up to 9 years. Criteria for the diagnosis of malignancy in stromatosis are discussed.

Cobb's syndrome and its treatment with embolization. Case report.

A case of Cobb's syndrome, which is characterized by spinal and vertebral angiomas and a skin nevus at the same metameric level, is reported. The spinal angiomas were treated with liquid embolization using ethylene vinyl alcohol.

Vascular and lymphatic tumors in infancy, childhood and adulthood: challenge of diagnosis and treatment.

The essential features of the major benign and malignant vascular tumors affecting infants, children and adults have been presented. Major emphasis has been placed upon the classification and treatment of hemangiomas, although malignant vascular tumors are discussed, and extensive and selected references are cited. The classification system of hemangiomas presented in this monograph is based upon the clinical prognosis of the angioma. The principles of treatment for hemangiomas based upon our clinical classification are discussed and may be summarized as follows: 1. The diagnostic features and prognosis vary with the variety of the angioma. 2. Spontaneous regression occurs only with juvenile hemangiomas, and the major involution occurs before age 5. 3. Patients with rapidly growing capillary cavernous hemangiomas should be treated with steroids. 4. Treatment modalities that increase scarring of the surface epithelium are contraindicated. 5. Intradermal and cirsoid angiomas do not spontaneously regress. 6. Congenital A-V fistulas require aggressive surgery. 7. The ultimate deformity resulting from angiomas may be greatly reduced by plastic surgical techniques. 8. Radiation therapy is never indicated for the treatment of hemangiomas. 9. The psychologic impact of the congenital deformity on patients is generally more detrimental than comparable postsurgical or traumatic deformities.

The Klippel-Trenaunay syndrome. A case report and review.

The Klippel-Trenaunay syndrome presents with a vascular nevus extending over an entire extremity, ipsilateral varicose veins dating from infancy and hypertrophy of all the tissues of the affected limb. This paper illustrates the problems that can be encountered in the management of this disorder. Congenital angiodysplasias should be suspected in all young individuals presenting with unilateral varicose veins. We recommend that modern methods of investigation be used in these cases to precisely define the anatomic pathology. In this manner, the appropriate treatment may be tailored to the patient's specific disorder.

Embolisation in tumours and vascular malformations of the head and neck.

The principles underlying the use of embolisation in the management of tumours and vascular malformations of the head and neck are reviewed. The percutaneous angiographic technique is employed to embolise the lesion, producing the effects of haemostasis, reduction of tissue mass and relief of pain. Specific clinical applications are discussed with illustrative examples.

Skeletal angiomatosis in association with gastro-intestinal angiodysplasia and paraproteinemia: a case report.

Skeletal-extraskeletal angiomatosis is defined as a benign vascular proliferation involving the medullary cavity of bone and at least one other type of tissue. It has also been known as cystic angiomatosis in which multiple cystic lesions are scattered diffusely throughout the skeleton often with similar angiomatous changes in other tissues, usually the spleen. A case of skeletal angiomatosis in association with gastro-intestinal angiodysplasia and paraproteinemia is reported.

[Superficial venous malformations]. / Malformations veineuses superficielles.

Mature vascular malformations of the venous compartment are haemodynamically inactive and persist throughout the patient's life. They have no systemic repercussions, except in patients with disorders of coagulation (e.g. disseminated intravascular coagulation), but they have local, cosmetic and functional repercussions. Percutaneous embolization, notably with Ethibloc, has modified their prognosis favourably. This simple and effective procedure is mainly used for superficial venous malformations of the face.

[Congenital subfold hemangioma in combination with hemangiomatosis of the head, face and neck]. / Vrozhdennaia podskladkovaia gemangioma v sochetanii s gemangiomatozom golovy, litsa, shei.

The treatment was made of 15 children aged 3-6 months with advanced hemangiomatosis of the head, face, neck and larynx. The treatment combined hormone therapy, sclerotherapy of the tumor, staged cryodestruction of the external and endolaryngeal nodes. In one case the arterial occlusion was performed. Good cosmetic results and recovery of adequate respiration were achieved in 13 children.