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Query: UMLS:C0027960 (mole)
 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case presented here is a three-month-old male infant with the Beckwith-Wiedemann's syndrome. Clinically, the patient was characterized by macroglossia, visceromegaly, umbilical hernia, microcephalus and other multiple malformations such as facial flame nevus or ear lobe grooves. The patient died of bronchopneumonia at the age of three months, and an autopsy was performed. Morphological examination revealed adrenal cytomegaly, hyperplasia and hypertrophy of the pancreatic islets, adrenal rest tissue in the right testis or hypertrophy of muscle fibers of the tongue associated with fibrous degenerative change, in addition to bronchopneumonia of the lung, causing his death. On electron microscopical examination, the cytomegalic cell of the adrenal was characteristic of large pleomorphic nucleus and granular substances with high density in the cytoplasm. In this case, thorough histologic search revealed no evidence of tumorous growth in the organs, though the exomphalos-macroglossia-gigantism syndrome has been of interest in its relationship to the occasional occurrence of Wilms tumor, adrenal carcinoma or other tumors.
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PMID:Beckwith-Wiedemann's syndrome--a report of an autopsied case--. 43 92

Nevus flammeus (teleangiectaticus) is the most common variety of congenital nevus. Among 3961 infants checked at the ages of 3--7 days and again at 6 weeks 42% showed nevi flammei mediales in the nape and/or in the eyelids and glabella. The mothers of the infants presenting with n.fl. tended to be overweight, to have circulatory distrubances and prolonged duration of laor. The infants with n.fl. frequently had concomittant edema, navel hernia and minor degenerative stigmata. However malformations did not occur more commonly. This investigation suggests that exogenous factors such as diseases during pregnancy, intake of drugs and difficulties in delivery do not influence the genesis of n.fl. Only constitutional factors could be correlated with the incidence of n.fl. Since there was a higher frequency in female than in male infants and a lower frequency in premature than in term newborns it may be that hormonal inffluences increase the incidence of n.fl., too. These results were obtained from the prospective study: Schwanger-schaftsverlauf und Kindesentwickling (DFG).
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PMID:[Correlation of obstetrical and clinical data with nevi flammei in the newborn (author's transl)]. 112 20

In 1979 Cohen et Hayden and in 1983 Wiedemann et al. delineated a syndrome consisting of partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affections. Hitherto the literature pertaining to this syndrome consists of somewhat more than 100 cases of which some, that have been described previously or subsequently under other headings, were rediagnosed as being Proteus syndromes. Of these, more than half show vascular anomalies closely resembling those observed in the Klippel-Trenaunay syndrome, but in the Proteus syndrome appear to be more haphazardly distributed over the integument. We report 3 pediatric patients with the Proteus syndrome, all showing cutaneous angiodysplasias. These patients were initially diagnosed as suffering from "severe or atypical Klippel-Trenaunay syndrome". In one of these, cardiac tumors were observed soon after birth which subsequently showed spontaneous involution and were therefore considered to be rhabdomyomas. In the Proteus syndrome cardiac pathology is rare, and cardiac tumors have not been described previously. Moreover, we observed umbilical hernia in two of our patients, a feature which has hitherto not been reported in patients with the Proteus syndrome. In all our patients a broad thoracic cage resembling a "body-builders chest", asymmetrical and disproportional macrodactyly and broad, flat feet were conspicuous. These broad, flat feet with macrodactyly and large spaces between the first and second digits were designed by the parents of one of our patient as "chimpanzee's feet". Macrodactyly, "chimp's" feet and a broad thoracic cage are considered by us to be clinical hallmarks of the Proteus syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Proteus syndrome. Expansion of the phenotype. Apropos of 3 pediatric cases]. 133 4

Presented are 3 cases of tumors affecting the papilla of the optic nerve. On the basis of the ophthalmoscopic picture, of fluorescein angiography and of clinical symptoms the authors identified a capillary haemangioma, a pigmentary naevus and a meningeal hernia. In all the cases observation did not detect any increase in the changes.
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PMID:[3 cases of tumors of the optic disk]. 181 64

19750 school children, ages 6 to 15 years, were examined by the authors of this study, 1,220 (6.18%) had congenital abnormalities. In this group, 4.23% were boys and 1.88% were girls. Case histories revealed inbreeding amongst the parents (families) of children with congenital malformation to be 8.9% and 8.2% for the rest of the families in this study. There were 27 different congenital abnormalities identified, with prevalence rates of 0.05/1,000 to 15.85/1,000. The most prevalent abnormalities were umbilical hernia (15.85/1000), inguinal hernia (14.50/1,000), pectus carinatum and excavatum (7.68/1,000), undescended testes (9.00/1,000 boys), congenital nevus (3.54/1,000), retractile testis (4.45/1,000 boys), pilonidal sinus (2.63/1,000), pes planus (2.28/1,000), and hemangioma (1.16/1,000). Of the 19,750 children, 70 had multiple anomalies (3.75/1,000).
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PMID:Prevalence of congenital abnormalities in Turkish school children. 824 91

Interest in day-care surgery is on the increase world-wide, with various surgical specialties embracing this mode of health service. In a period of 5 years (January 1989-December 1993), 286 patients attending a Plastic Surgical Unit were operated on a day care basis. This represented 22.7% of all cases done within the period. The most frequently performed procedure was keloid excision and suture/flap cover (29.7%) followed by inguinal hemiorrhaphy (10.8%) ganglionectomy (8%), excision of lipoma (8%), scar revision (5.2%), suture of skin lacerations (4.5%), breast lumps biopsy (5.9), release, grafting or plasty of flexion deformity of fingers (3.4%). Other problems dealt with on day care basis included repair of human bite losses of face (3.2%); Excision of gynaccomastia (3.1%) umbilical hernia repair (2.4%); breast augmentation with implant 0.3%. Excision of planter wart, hairy naevus, chronic neck folliculitis, sebaceous cyst, dermoid cyst and polydactylism constituted the rest of problems dealt with as day cases. Readmission represents failure of day care surgery and constituted 2.4% of all cases. This was due mainly to social factors and bleeding at home. Significant wound breakdown occurred in 0.69% of cases. We conclude that day care plastic surgery in our subregion is safe and effective.
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PMID:Scope and problems of day-care surgery in a plastic surgical unit. 902 Jun 4

A girl born with a left chest wall hamartoma, macroglossia, nevus flammeus of the middle forehead, and a small umbilical hernia developed left lower extremity hemihypertrophy by 1 year of age and is assumed to have Wiedemann-Beckwith syndrome. Hamartoma of the bladder and a cardiac fibrous hamartoma have been reported previously in association with Wiedemann-Beckwith syndrome. Infantile hamartomas are exceedingly rare and add to the spectrum of tumor formation in the syndrome.
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PMID:Chest wall hamartoma with Wiedemann-Beckwith syndrome: clinical report and brief review of chromosome 11p15.5-related tumors. 1142 37