1/7. prenatal diagnosis of Milroy's primary congenital lymphedema.Milroy's primary congenital lymphedema (PCL) (hereditary lymphedema type I, Milroy disease) is present at birth, and mostly affects the dorsal aspects of feet. It is mostly a life-long condition but does not affect longevity. Complications are rare except for chronic discomfort and warmness of affected areas. PCL is an autosomal dominant disease with incomplete penetrance due to a mutation in the gene locus encoding for VEGFR3 with resultant dysgenesis of microlymphatic vessels. We report on two fetuses where ultrasonographic examination at 15 weeks of gestation showed significant edema of the dorsal aspects of both feet with no evidence of other major malformations. Whereas in one fetus the edema resolved completely, it persisted in the second fetus and proved after birth to be of lymphedematous nature. To the best of our knowledge, this is the first report of early prenatal diagnosis of primary congenital lymphedema via fetal ultrasonographic examination and of spontaneous resolution of lymphedema during fetal life.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/7. Coats' disease and bilateral cataract in a child with turner syndrome: a case report.PURPOSE: To report the first case in which Coats' disease was observed with infantile cataract in a girl with turner syndrome (TS). MATERIALS AND methods: We examined a 4-year-old female infant with TS who was referred with a diagnosis of leukocoria in the left eye. RESULTS AND DISCUSSION: Examination under anaesthesia revealed a bilateral punctate cataract and left eye fundus showed vascular retinal abnormalities typical of Coats' disease. cryotherapy was performed on the telangiectatic vessels and the child was followed up for a period of 12 months. Despite cryotherapy resulting in regression of the peripheral exudates, an exudative maculopathy persisted with poor visual outcome. We suggest that Coats' disease should be considered as a rare ocular manifestation in TS.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/7. Pedal hemangioma (venous malformation) occurring in Turner's syndrome: an additional manifestation of the syndrome.Two infants with Turner's syndrome and an unusual vascular tumor or malformation of the feet are described. In one child, the lesion developed in the dorsal metacarpal area, whereas in the other, the lesion was located in a bilaterally symmetrical distribution over the dorsum of the feet. Both lesions were characterized by a proliferation of tortuous, thick-walled veins with imperfectly formed muscular walls. Redundancy of the endothelial lining leading to intimal "webs" and intraluminal vascular channels was prominent. The similarity of these two lesions clinically and histologically suggests that they do not represent a fortuitous occurrence, but an additional manifestation of Turner's syndrome. Furthermore, their existence indicates that the vascular abnormality in Turner's syndrome may be more generalized than previously recognized and may include abnormalities of peripheral blood vessels in addition to those of the heart and aorta.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/7. Treatment of mycotic intracranial aneurysms.Two patients with mycotic intracranial aneurysms were successfully treated with only antibiotic therapy. One patient, who had subacute bacterial endocarditis, rheumatic valvular disease, and an abscessed tooth, sustained a subarachnoid hemorrhage from a ruptured right middle cerebral artery trifurcation aneurysm. The other patient, who had Turner's syndrome and probable congenital aortic stenosis, developed multiple neurological findings during an ipisode of acute bacterial endocarditis precipitated by an infected ingrown toenail; a false aneurysm of the distal left middle cerebral artery and two lesions involving the left superior cerebellar artery were found. A study of the literature shows that only 45 patients with mycotic intracranial aneurysms have received adequate antibiotic therapy and angiographic documentation. Statistically, there does not appear to be a clear-cut advantage to antibiotic plus surgical therpy over antibiotic alone. In fact, in 21 patients who underwent serial angiography, lesions were smaller in six and not visualized in 11. In four patients the aneurysms increased in size; in two others fresh lesions formed. The author proposes the following diagnostic and therapeutic regimen: 1) earliest possible diagnosis of the underlying disorder; 2) appropriate antibiotic therapy; 3) early four-vessel cerebral angiography and follow-up studies every 2 to 3 weeks; study; 5) definitive operation upon completion of antibiotic therapy if the lesion is larger or the same size; and 6) postoperative angiography to evaluate the effectiveness of treatment and to search for interim lesions.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/7. Abnormalities of gut vessels in Turner's syndrome.We describe a 57-year-old patient with Turner's syndrome, iron deficiency anaemia and intestinal vascular abnormalities. colonoscopy revealed 2 widely dilated, tortuous veins in the terminal ileum and several smaller ectatic veins and haemangioma-like malformations throughout the colon. laparotomy for herniotomy showed only minimal vascular abnormalities of the serosal surface. patients with Turner's syndrome and anaemia should be checked for these lesions by endoscopy, and conversely, in patients with such lesions, Turner's syndrome should be considered.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
6/7. Turners's syndrome associated with lymphoedema, diagnosed in the newborns.It is known from literature that about 30% of all female patients with Turner's sydrome have a lymphoedema at the feet and hands. The article discusses as possible reason a congenital defect of the lymphatic system: The vessels are thin-walled, extended and have few valves or none at all. In two phenotypically normal, mature newborn, an analysis of the chromosomes was performed because of marked lymphoedema of the lower extremities, confirming the suspected diagnosis of Turner's syndrome. Hence, it is concluded that Turner's syndrome must always be suspected if there is a congenital lymphoedema of the lower extremities and especially also of the region of the neck.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/7. Two cases of unilateral retinal neovascularization in turner syndrome.PURPOSE: To report two cases of unilateral retinal neovascularization in turner syndrome. METHOD: We examined two female infants, born at 34 weeks' and 33 weeks' gestation, who had received oxygen for 9 days and 3 days, respectively, after birth. Both infants had the 45,XO karyotype of turner syndrome. RESULTS: In each patient, the fundus of one eye showed an avascular area, neovascularization, and multiple branchings with anastomosis of retinal vessels in the temporal periphery. Fellow eyes showed no abnormalities in the fundi. Both infants were treated with argon laser photocoagulation to the avascular area and the neovascularization in the temporal periphery. The neovascularization regressed completely after retinal photocoagulation. CONCLUSION: Abnormalities of retinal vessels may be one of the ocular findings associated with turner syndrome.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |