1/12. leopard syndrome.An 18-year-old girl with leopard syndrome is described. Clinical manifestations include lentigines, ocular hypertelorism, mental and growth retardation, deafmuteness, and several patches of hair loss on her scalp. No family history of skin lentiginosis or any other inherited condition was found.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
2/12. leopard syndrome--report of a variant case.This case report presents a patient with leopard syndrome, with multiple lentigines all over the body and face, ocular hypertelorism, delayed secondary sexual characteristics, mild cardiac abnormalities and supernumerary teeth. Clinical relevance of this syndrome lies in its early recognition and precautions to be taken during any invasive dental procedure, which if not performed under antibiotic prophylaxis and premedication, could lead to infective endocarditis. Additionally, a multidisciplinary approach with pediatric and medical consultants is mandatory during the management of such cases.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
3/12. leopard syndrome.The leopard syndrome is a complex of multisystemic congenital abnormalities characterised by lentiginosis, electrocardiographic conduction abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of genitalia, retardation of growth and deafness (sensorineural). Hypertrophic cardiomyopathy, though not included in the mnemonic, is often associated. Although the leopard syndrome is rare, it is important to recognise it since it can be associated with serious cardiac disease. It is advisable to follow up patients with leopard syndrome for new onset of cardiac abnormalities and to monitor the progression of existing cardiac disease. We present a case report and review of the literature of this syndrome.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
4/12. Cardio-cutaneous syndrome (the "LEOPARD" syndrome). review of the literature and a new family.Numerous reports of familial cardiac disorders associated with abnormalities of cutaneous pigmentation have appeared in the literature. In some of these there have been other somatic malformations including retardation of growth and anomalous development of the genitalia. In this paper we review the literature and describe another family with multiple lentigines occurring in six members out of eight examined. They were reported to occur in 10 other members not seen by us. The trait showed an autosomal dominant mode of inheritance and appeared to be transmitted by affected fathers. In previous reports transmission has usually been through an affected mother. In five of the six patients examined, only skin abnormalities were detected, but in the propositus the lentigines were associated with retardation of growth, hypertelorism, abnormal genitalia and complete atrioventricular block. The possible explanations for the lack of pleomorphy in other members of the family are discussed.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
5/12. leopard syndrome with a new association of congenital corneal tumor, choristoma.A 5-year-old girl with a family history of leopard syndrome had multiple lentigines on the face and trunk, hypertelorism, and growth retardation. In addition, she had congenital corneal tumors on both eyes. Histologically the tumors were choristoma. The neuroectodermal origin hypothesis of leopard syndrome could explain the presence of a congenital corneal tumor in this patient. We suggest that corneal tumor may represent an unrecognized associated finding in this syndrome.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
6/12. Polyarticular pigmented villonodular synovitis in children: evidence for a genetic contribution.Pigmented villonodular synovitis rarely occurs in multiple joints, and only once has been noted in siblings. We identified this disorder in 2 generations and found evidence for its presence in a third. It occurred in 2 sibling children at multiple sites. All affected individuals also had a rare autosomal dominant disorder, the multiple lentigines syndrome. Among the developmental abnormalities in this kindred were fibrous dysplasia, hypertelorism, pectus excavatum, high arched palate, and primary lymphedema. The etiology of pigmented villonodular synovitis is uncertain, and suggested causes include trauma, inflammation, neoplasia, and repeated hemorrhage. Our observations suggest genetic factors might also be important in the development of pigmented villonodular synovitis.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
7/12. Premature cataracts associated with generalized lentigo.Generalized lentigo (leopard syndrome) is an autosomal dominant trait characterized by lentigo, sensorineural deafness, retarded growth (below 25%), ocular hypertelorism, mandibular prognathism, pectus carinatum or excavatum, dorsal kyphosis, winging of the scapulae, valvular pulmonary artery stenosis, electrocardiographic conduction defects, and genitourinary defects. Ocular evaluations of patients with generalized lentigo have revealed the appearance of multiple small white punctate and comma-shaped opacities in the cortex and nuclci of the lenses of affected patients. On the basis of age of the patients examined, it would seem that the corneal opacities first appear in the third decade. Although the opacities may be extensive, the lens opacities do not appear to impair visual function until approximately twenty years after they first appear.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
8/12. Multiple lentigines (Leopard) syndrome with Chiara I malformation.This is a case report of multiple lentigines (leopard syndrome) with Chiari malformation, i.e. a herniation of the cerebellum into the foramen magnum. This male patient had generalized multiple lentigines since birth, and the lesions spread steadily, involving the scalp but sparing all mucous membranes. Organ system involvements included heart murmur, ocular hypertelorism, and retardation of growth.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
9/12. Multiple lentigines syndrome in a Nigerian family.Three cases of the multiple lentigines syndrome are reported in a Nigerian family, involving a father and two of his children. The major abnormalities in this syndrome include: multiple lentigines, cardiac defects, genitourinary malformations, neurological deficits, growth retardation, cephalofacial dysmorphism and a family history consistent with an autosomal dominant mode of inheritance. We hereby report these cases in whom the main features are multiple lentigines, ocular hypertelorism, electrocardiographic defects and a pattern consistent with an autosomal dominant mode of inheritance.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
10/12. Multiple lentigines syndrome (leopard syndrome or progressive cardiomyopathic lentiginosis).The multiple lentigines syndrome is an autosomal dominant condition which has many similarities to noonan syndrome, except in the most striking feature from which its name is derived. The less neutral but very apt mnemonic, leopard syndrome, was first used by Gorlin et al to whom the major debt in the definition of this syndrome lies, that is, Lentigines, ECG abnormalities, Ocular hypertelorism/Obstructive cardiomyopathy, pulmonary valve stenosis, Abnormalities of genitalia in males, Retardation of growth, and deafness. Not previously included in the mnemonic is cardiomyopathy which is an important feature because it is associated with significant mortality.- - - - - - - - - - ranking = 1keywords = hypertelorism (Clic here for more details about this article) |
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