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At least 1,063 records · Page 59Linked to original sources
PubMed1996

Ocular manifestations in Delleman syndrome (Oculocerebrocutaneous syndrome, OCC-syndrome) and encephalocraniocutaneous lipomatosis (ECCL). Report of three cases.

The authors present two patients with Delleman syndrome and one in with possible ECCL. Two boys with Delleman syndrome showed characteristic dysmorphic features with cerebral, ocular and skin malformations. The ocular anomalies consisted of eyelid coloboma, microphthalmia, iris coloboma and epibulbar lypodermoids. A third boy with possible ECCL syndrome had limbal lypodermoids, ectopia pupillae and aberrant iris tissue in the right eye. He showed an ipsilateral focal dermal hypoplastic defect within an area of alopecia of the scalp.

Abnormalities, Multiple↗
PubMed1996

Vertical transmission of the hip rolls due to wearing of TLSO for scoliosis.

Cosmesis is important in the treatment of adolescent patients with idiopathic scoliosis. It has been shown that although bracing reduces the rib hump, it induces several complications. The aim of this study was to describe a new disturbing complication due to wearing a thoracolumbosacral orthosis (TLSO) for adolescent idiopathic scoliosis: the vertical transmission of the hip rolls (trochanteric lipomatosis) in girls and its pathogenetic mechanisms. Trochanteric lipomatosis is common in the female population, particularly after adolescence, and is responsible for the characteristic appearance of the hip rolls. No data have been reported concerning vertical transmission of normal trochanteric lipomatosis with subsequent disturbance of aesthetic appearance while wearing the TLSO. In this prospective study, 300 consecutive adolescent girls who were treated with the TLSO for progressive idiopathic scoliosis were followed and evaluated for development of any vertical transmission of the hip rolls after application of the brace for an average of 2.5 years after termination of the treatment. Almost simultaneously, 290 age-matched, randomly selected adolescent girls served as controls and were examined by two unbiased observers during the school screening program to estimate the prevalence of trochanteric lipomatosis in female adolescents of the same geographic area. In this series, bracing reduced the thoracic scoliosis from 34.4 +/- 5 degrees to 18.5 +/- 6 degrees and the lumbar scoliosis from 29.4 +/- 4 degrees to 16.7 +/- 4 degrees 6 months after initiation of brace wearing. The average prevalence of normal trochanteric lipomatosis in girls in the control group was 26% (range, 12.2-68.4%) for the ages 9-14 years and was increasing with the age and weight. No correlation was found between amount of reduction of scoliosis and prevalence of the complication. Vertical transmission of the hip rolls was observed in 69 (23%) of the girls with scoliosis 6 months after initiation of brace wearing and was positively correlated with the rigidity of the scoliotic curvature (p < 0.05) and the amount of reduction of the curvature (p < 0.05). Seven (8.8%) girls discontinued brace wearing because of psychological distress related to the deformity around the hip rolls, whereas 51 (73%) additional girls underwent suction lipoplasty during or after the termination of the brace wearing, because of persistent and disturbing lipomatosis. Recurrence of significant lipomatosis was observed in 3 (5.8%) of the girls who underwent lipoplasty. Physicians who are involved with treatment of scoliosis with bracing should be aware of this aesthetic complication. Brace wearing should not attempt maximum correction of scoliosis, thus avoiding excessive force on the body, and should be ordered only in patients with immature spines, curves of > 25 degrees, and radiologically justified progression. Suction lipoplasty seems to be a safe method of treatment and should be advised in cases with unaesthetic vertical transmission of the hip rolls.

Adipose Tissue↗
PubMed2006

CT differentiation of distal pancreas fat replacement and distal pancreas agenesis.

We aimed to describe CT signs useful for differentiation of distal agenesis from distal or dorsal pancreas lipomatosis. Multidetector CT (MDCT) studies of five patients with distal pancreas agenesis (n = 2), distal lipomatosis (n = 1), distal short pancreas (n = 1), and distal pancreatectomy (n = 1) were retrospectively reviewed. Agenesis of dorsal pancreas can be diagnosed by the absence of body and tail of pancreas. In the absence of distal pancreas, distal pancreatic bed can be filled by stomach or intestine (dependent stomach or dependent intestine signs), which abut splenic vein. Same findings can be seen in patients with distal pancreatectomy, however, splenic vein is absent in these patients. In case of distal lipomatosis abundant fat tissue is observed anterior to splenic vein. Dependent stomach and/or dependent intestine signs on MDCT imaging can allow differentiation of distal pancreas agenesis from distal lipomatosis obviating further diagnostic studies.

Adipose Tissue↗
PubMed1984

Incidence and diagnostic significance of minor pathologic changes in the adult pancreas at autopsy: a systematic study of 112 autopsies in patients without known pancreatic disease.

In 112 unselected autopsies of adult patients without known pancreatic disease (except adult-onset diabetes mellitus), the pancreas was examined to establish the incidence and degree of such minor pancreatic lesions as lipomatosis, fibrosis, alterations of ducts and ductal epithelium, inflammatory infiltrates, focal necrosis, acinar dilation, and vascular changes. Each lesion was then tested for statistically significant correlations with the age of the patient and a number of clinical conditions, including cholelithiasis, adult-onset diabetes mellitus, adiposity, generalized severe atherosclerosis, chronic alcoholism, severe bacterial infection prior to death, and generalized malignant tumor. This was done in the hope of finding associated or predisposing factors for the pancreatic lesions. The results show, in addition to the unexpectedly high incidence of the various pancreatic lesions, a clear increase of lipomatosis, fibrosis, and both ductal and ductal epithelial alterations with increasing age; these conditions were accompanied by a steady decrease in the mean weight of the gland, starting at the age of about 40 years, except in cases of advanced lipomatosis. The latter condition was associated with adult-onset diabetes mellitus. Severe generalized atherosclerosis was correlated with lipomatosis and fibrosis, but the two latter conditions were found together only rarely. Acute (terminal) lesions, including focal necrosis and acinar dilation, were associated with severe bacterial disease prior to death. Other statistically significant correlations were rare, indicating the lack of specificity of these minor pancreatic lesions rather than offering a clue as to their pathogenesis. The diagnostic significance and the relations of these lesions to clinically relevant chronic pancreatitis are discussed briefly.

Adolescent↗
PubMed2000

Imaging changes in the pancreas in cystic fibrosis: a retrospective evaluation of 55 cases seen over a period of 9 years.

BACKGROUND: Pathologic changes of the pancreas have been observed as early as the recognition of the disease termed initially "cystic fibrosis of the pancreas". Atrophy of the gland and its fatty infiltration were considered as usual features. The aim of this study was to follow-up the evolution of cystic fibrosis pancreas and to define its successive stages in correlation with the clinical, biochemical, and imaging findings. METHODS: Fifty-five patients were followed up during 9 years. The patients' genetic backgrounds were systematically performed. Blood lipase levels were analyzed systematically at each consultation of the patients and in the event of bouts of abdominal pains. Imaging using mainly echograms and tomodensitometric scans were regularly performed: echograms every 6 months, and tomodensitometric scans every 1 to 2 years. Magnetic resonance imaging was performed in four patients. RESULTS: Five groups of patients were identified on the basis of tomodensitometric scan findings: normal pancreas (n = 4), incomplete lipomatosis of the pancreas (n = 9), complete lipomatosis of the pancreas (n = 23), cystic pancreas (n = 5), macrocystic pancreas (n = 1), atrophic pancreas (n = 13). Pancreas exocrine function was not correlated with findings. Forty episodes of pancreatitis were observed in seven patients. They had bouts of abdominal pain and elevation of lipase levels. Five of these patients were composite heterozygotes (D508/other). Incomplete lipomatosis represents an intermediate stage leading toward complete lipomatosis or toward atrophy after pancreatitis. CONCLUSIONS: Studies of pancreatic function should be performed routinely in cystic fibrosis, especially in pancreatic sufficiency or in patients with normal pancreas images. Acute pancreatitis should be diagnosed and properly identified to be differentiated from other acute abdominal syndromes occurring in cystic fibrosis.

Adolescent↗