1/5. Perioperative management of infants with the linear naevus sebaceous syndrome of Jadassohn: a report of two cases.Neurofibromatosis and tuberous sclerosis are the most well-recognized of the congential phakomatoses, a group of six hereditary neuro-oculo-cutaneous disorders. Although easily diagnosed at birth by a parasagittal line of facial sebaceous naevi, the linear naevus sebaceous syndrome (LNSS) of Jadassohn is the rarest phakomatosis, one often characterized by airway and anaesthetic considerations that do not apply to the other phakomatoses. In addition to its obvious cutaneous manifestations, LNSS is characterized by hemifacial asymmetry, an anatomic predictor of difficult trachael intubation, and intractable seizure activity, a condition that limits selection of anaesthetics. The perioperative management challenges of LNSS are depicted in the presentation of two cases of LNSS with different outcomes and contrasted with the major anaesthetic considerations in the perioperative management of other, more common phakomatoses.- - - - - - - - - - ranking = 1keywords = naevus (Clic here for more details about this article) |
2/5. Late occurrence of additional ocular and intracranial pathologies in the linear naevus sebaceous (Feuerstein-Mims) syndrome.The linear naevus sebaceous syndrome of Feuerstein-Mims belongs to the phacomatoses and classically is composed of a linear cutaneous lesion associated with mental retardation and epilepsy, sometimes also with ocular dystrophies. During an unusually long follow-up of 32 years of such a case, which is reported here, the late occurrence of additional ocular and intracranial pathology are demonstrated: conjunctival and bulbar tumours with progressive deterioration of visual acuity, bilateral intracranial arachnoidal cysts, dilatation and tortuosity of the middle cerebral artery. As a consequence of these observations the prognosis in cases with this syndrome has to be given with special caution.- - - - - - - - - - ranking = 1keywords = naevus (Clic here for more details about this article) |
3/5. Ocular findings in linear sebaceous naevus syndrome.The case of a 5-month-old black female child with a linear sebaceous naevus syndrome and multiple congenital anomalies is presented. Ocular malformations consisted of colobomatous changes of the lid and retina, dermoid of the conjunctiva, chorioretinal changes, and peripapillary atrophy of the optic nerve. Systemic findings included midline cleft of the secondary palate with involvement by the naevus, bilateral hearing loss, asymmetrical skull bones, ventricular septal defect, epidermal inclusion cyst, and developmental delay without seizures.- - - - - - - - - - ranking = 1.2keywords = naevus (Clic here for more details about this article) |
4/5. adult form of basal cell naevus syndrome: a family study.A 32-year-old patient had marked reduction of visual acuity due to falciform folds of the retina and retinal detachment, and severe neurological abnormality: bilateral pyramidal involvement, fasciculation in all limbs and gait ataxia. skull radiographs showed internal frontal hyperostosis; CT scan showed calcification of the falx cerebri, and multiple arachnoid cysts were shown by myelography. A naevoid lesion had previously been removed from the left forearm. There was a history of ophthalmological symptoms in the mother and the daughter of the propositus. His son has "cafe au lait" spot on the abdomen and dentigerous cysts. The diagnosis of an adult form of basal cell naevus syndrome with an autosomal dominant mode of inheritance is discussed.- - - - - - - - - - ranking = 1keywords = naevus (Clic here for more details about this article) |
5/5. sturge-weber syndrome associated with naevus of Ota.The association of sturge-weber syndrome with naevus of Ota is an infrequently reported phenomenon and there are only four previously described cases in the literature. In this paper we briefly review the literature regarding the coexistence of vascular and pigmentary naevi and present an additional patient with the association of the sturge-weber syndrome and naevus of Ota.- - - - - - - - - - ranking = 1.2keywords = naevus (Clic here for more details about this article) |