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Query: UMLS:C0019270 (hernia)
 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An 8-year-old boy with an uncorrected ventricular septal defect, pulmonary stenosis, mental retardation, and gigantism died 24 hours after partial resection of a large right-sided Wilms' tumor. The presence of other abnormalities, including a small umbilical hernia and overgrowth of the external genitalia, raises the possibility that this case represents a variant of the Beckwith-Wiedemann syndrome. The typical facial features of Sotos' syndrome were not present. Gross examination of the surgical specimen revealed that tumor was present in the resected margin of left renal vein. Necropsy showed that death resulted from extensive cerebral infarction due to occlusion of the left internal carotid artery and its branches by tumor emboli. Paradoxical embolism had occurred during or soon after partial resection of the tumor mass due to passage of tumor fragments into the systemic circulation through the ventricular septal defect.
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PMID:Fatal paradoxical embolism to the left carotid artery during partial resection of Wilms' tumor. 132 57

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

Giant inguino--scrotal hernia is largely a problem of developing countries. A case of an unusually giant inguino-scrotal hernia is reported highlighting the problems encountered in management. Orchidectomy, bowel resection and relaxing epigastric incision were successfully employed in repairing the hernia. Hernias may not be difficult to manage if they ar not neglected. Efforts of health education need intensifying in this direction. We propose thorough peri-operative pulmonary exercises to cut down the post-operative pulmonary morbidity.
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PMID:Giant inguino scrotal hernia: a case report. 151 21

A pair of monozygotic twins discordant for the Wiedemann-Beckwith syndrome (WBS) associated with congenital central hypothyroidism is described. During pregnancy, the mother was noted to have marked polyhydramnios and severe abdominal distension. At birth, a large diamniotic, monochorionic placenta was noted. According to a study of the minor blood groups and HLA typing, the possibility of monozygosity is 0.985. The mode of inheritance of the syndrome is debatable, and according to observations of discordance in monozygotic twins, single gene control seems unlikely. The thyroid function was noted to be subnormal when the proband was 3 days old. Oral thyroxine has been supplied since infancy. Repair of the umbilical hernia, the bilateral inguinal hernia, and a bilateral orchidopexy were done. A partial glossectomy was later performed to prevent progressive orofacial maldevelopment. The postnatal gigantism persisted as he grew.
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PMID:Wiedemann-Beckwith syndrome with congenital central hypothyroidism in one of monozygotic twins. 197 21

In 1963 Beckwith and Wiedemann reported a syndrome, which was characterized by umbilical hernia, macroglossia and somatic gigantism. In the literature, early intervention by tongue reduction is recommended in order to prevent both mandibular prognathism and open bite. In this paper seven cases were presented showing that early functional treatment of the stomatognatic system leads to pleasing results, both functionally and aesthetically. Therefore it is suggested, that functional treatment should replace surgical management of macroglossia in the Beckwith-Wiedemann-syndrome.
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PMID:[Early orthodontic treatment measures in infants with the EMG syndrome]. 258 28

Giant omphaloceles are usually treated in stages. The skin is closed first and the ensuing giant ventral hernia is repaired when the patient is 1 year old or older. This attitude has many advantages, but treatment of the giant ventral hernia may be very difficult and often requires many operations. We have used a new approach to this problem in two cases. A polyamide mesh is glued over the skin of the abdominal wall, thorax, and lumbar regions. The distance between the apposed rectus muscles is then progressively decreased by repeated infolding of the polyamide mesh with running longitudinal sutures on the mesh itself. This progressively reduces the hernial content. When the rectus muscles are sufficiently approximated, a definitive procedure is carried out. In the first patient, the giant hernia was completely closed in 3 weeks and in the second patient, it was closed in 5 weeks. A third patient is presently undergoing the same treatment. This simple bedside technique does not require any antiseptic measure and may replace advantageously the use of prosthetic material in a majority of cases.
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PMID:The giant omphalocele: a new approach for a rapid and complete closure. 294 88

Giant inguinal herniae present a major challenge in management. This case details clinical features of an enormous inguinoscrotal hernia associated with septic gangrene and elephantiasis of the scrotum. Two initial operations were required for control of sepsis, followed by a two-staged hernial repair, involving a total colectomy and a subsequent neoscrotal repair. The problems of loss of domain within the abdominal cavity and the special features presented by this case are discussed.
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PMID:Giant inguinal hernia. 325 Apr 19

An infant with an incomplete expression of Wiedemann-Beckwith syndrome during the neonatal period was suspected to suffer from hypothyroidism. However, after exclusion of this tentative diagnosis the phenotypic characteristics lead to the correct diagnosis. In addition to the macroglossia, the typical facial signs of this syndrome such as capillary haemangioma of the glabella, soft tissue folds under the eyes and linear indentations of the ear lobes are demonstrable. Gigantism and umbilical hernia are absent. A tendency to hypoglycaemia and the increased risk of malignancy are the important aspects of this syndrome, which may be expressed in a variety of clinical forms. So far the aetiology of this syndrome is unknown although familial cases implicate the involvement of genetic factors.
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PMID:[Wiedemann-Beckwith syndrome. Study of an oligosymptomatic form]. 377 23

Giant incisional hernias are treated in many different ways involving risks and various degrees of mutilation. We prefer the use of polypropylene mesh (PPM) which is manufactured as Prolene or Marlex mesh. It is easy to handle, has great tensile strength and produces minimum tissue reaction. These qualities make PPM a good substitute for abdominal fascia and the procedure becomes simpler and less extensive than with the use of dermal grafts, flap transfers or other methods. This series consists of twenty-four patients. The results were good in 20 patients (85%). Recurrence of the hernia occurred in two patients (7.5%) and weakness of the abdominal muscles in two others.
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PMID:Giant incisional hernias closed with polypropylene mesh. 377 80

An association between trisomy 11p15 and Beckwith-Wiedemann syndrome is described in two brothers. The first presented at birth with gigantism and macroglossia, umbilical hernia and abdominal distention, hypoglycemia and atresia of the pulmonary artery, leading to the diagnosis of Beckwith-Wiedemann syndrome. Facial dysmorphism also included: a hypoplastic midface, hypertelorism, and a short nose with a flattened bridge. The karyotype showed a trisomy 11p15 with a monosomy 18p11, due to a t(11;18)(p154;p111)pat. His brother, born a year later, showed the same signs. The association between trisomy 11p15 and Beckwith-Wiedemann syndrome is in certain cases well established.
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PMID:Trisomy 11p15 and Beckwith-Wiedemann syndrome. Report of two new cases. 387 70


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