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Query: UMLS:C0033377 (prolapse)
 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This report describes a 56-yr-old man with a dominantly inherited disorder affecting four generations and characterized by bilateral ptosis and dysphagia. Muscle biopsy showed only minor light microscopic abnormalities but electron microscopy revealed fibres containing paracrystalline mitochondrial inclusions. Southern analysis of mitochondrial DNA obtained from muscle did not reveal mitochondrial gene deletions. An extensive search eventually identified the characteristic intranuclear filaments of oculopharyngeal muscular dystrophy (OPMD). Abnormal mitochondria are non-specific epiphenomena in OPMD but a potential source of confusion with a late-onset mitochondrial cytopathy. This case further emphasizes the necessity for a diligent search for the diagnostic intranuclear filaments when oculopharyngeal muscular dystrophy is suspected clinically.
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PMID:Mitochondrial abnormalities in oculopharyngeal muscular dystrophy. 878 3

To date there are many descriptive terms for the tuberous breast deformity but there is no widely accepted nomenclature. A retrospective study was undertaken of 68 tuberous breasts and the operative corrections performed. The deformities were classified into four types. Type I (hypoplasia of the lower medial quadrant), type II (hypoplasia of the lower medial and lateral quadrants, sufficient skin in the subareolar region), type III (hypoplasia of the lower medial and lateral quadrants, deficiency of skin in the subareolar region) and type IV (severe breast constriction, minimal breast base). Areolar prolapse, usually regarded as a major symptom, was only found in 30 (44%) deformed breasts. Postoperative review of 51 breasts in 31 patients showed that type I cases treated by reduction mammaplasty of adequately sized breasts or augmentation of hypoplastic breasts had excellent results. These procedures with additional spreading of the breast tissue in type II deformities give good results. Severe cases (types III and IV) treated by augmentation and tissue spreading procedures have an unsatisfactory shape and have a 'second crease' deformity. For types III and IV, additional skin in the subareolar region by tissue expansion or flap procedures is necessary. There is no one method to correct 'the' tuberous breast but there are many procedures which should be used according to the type of deformity. The classification developed could end the confusion in nomenclature.
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PMID:The tuberous breast deformity: classification and treatment. 888 78

A 82-year-old female was admitted to hospital because of deteriorated general condition, severe diffuse headache and complete left-sided ptosis. A computed tomography scan of the head revealed no subarachnoid haemorrhage. Based on the hypothesis that the symptoms resulted from an infarction in the brain stem, the previous medication with Aspirin was continued. After repeated vomitus hypotensive dehydration developed and was adequately treated. Because of confusion, elevated white blood counts and signs of meningism, a spinal puncture was performed. Only the serology for Borrelia-IgG was positive, therefore the patient received Rocephin. During treatment only the ptosis persisted, therefore the substitution with sodium and the medication with Prednisone were stopped. Afterwards the symptoms reappeared and the laboratory results showed insufficiency of the pituitary. A magnetic resonance scan showed a microadenoma of the pituitary with local bleeding. Nine months after pituitary apoplexy, with hormonal substitution only a divergent strabism on the left side persisted. Clinical findings, course and therapy of pituitary apoplexy are discussed.
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PMID:[Headache, general malaise and left-side ptosis]. 978 50

Although surgical intervention is occasionally required for rectal prolapse (RP), there is both vagueness as to the indications for surgery and confusion as to the technique that should be used for children who need surgical treatment. Using Ekehorn's transanal suture rectosacropexy technique, 56 children with RP were treated surgically between 1987 and 1998 at our hospital. There were 36 boys and 20 girls, the average age was 4.5 years, and the duration of the recurrent prolapse prior to admission ranged from 3 to 8 months. The technique consists of simply inserting one "U"-shaped suture through the rectal ampulla and tying the strands of the suture outside at the level of the sacrococcygeal junction. In this series, follow-up periods ranged from 1 to 10 years and there were no recurrences. We believe that surgical indications for RP need to be defined more clearly and that Ekehorn's technique offers a simple and effective method for the surgical treatment of complete RP in children.
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PMID:Management of rectal prolapse in children: Ekehorn's rectosacropexy. 1007 42

The neurologic manifestations of thallium poisoning include a severely painful ascending peripheral neuropathy, autonomic dysfunction, cranial nerve abnormalities, and a toxic encephalopathy. Although thallium has a short half-life, these neurologic manifestations commonly progress, even as the blood concentration of thallium decreases. This suggests either that thallium persists in neuronal tissues or that it initiates an injury cascade that takes time to fully manifest. As the latter mechanism is consistent with many toxin exposures, the concept of a central nervous system reservoir for thallium is often discounted. A recent case provided a unique opportunity to evaluate this possibility. A 48-year-old man was acutely and chronically thallium poisoned by his common-law wife. During his initial exposures, only gastrointestinal symptoms manifested. Following an acute ingestion, hospitalization was required. Over 3 days, his symptoms rapidly progressed from a severely painful neuropathy to slurred speech, ptosis, confusion, coma, respiratory insufficiency, and death. Because of considerations of alternative diagnoses, 2 lumbar punctures were performed, one on admission and another on the day of his death. Serum thallium concentrations obtained from stored blood samples were paired with spinal fluid concentrations from the same days. On day 1, serum and spinal fluid concentrations were 8700 mu/L and 1200 mu/L, respectively. On day 3, although the serum concentration had fallen to 7200 mu/L, the spinal fluid concentration had increased to 2100 mu/L. This case provides evidence to support the hypothesis that thallium distributes into the central nervous system more slowly than the blood compartment, and this may in part account for the progression of neurologic findings in the setting of decreasing serum concentrations.
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PMID:Cerebrospinal fluid analysis in fatal thallium poisoning: evidence for delayed distribution into the central nervous system. 1516 69

During the last 10 Years, the cesarean section (CS) rate was increased despite of the recommendations of the World Health Organization to keep it below 10-15%. The purpose of this review of the literature was to demonstrate how the concept of CS rate limitation has become obsolete. The increase in the CS rate is mainly justified by the decrease in maternal mortality and morbidity following elective CS: surgery-related risks have decreased and the confusion that was made between the risks of vaginal delivery and those of trial of labor has to be clarified to show that maternal mortality and morbidity are not increased by elective CS. However, instrumental delivery and CS during labor remain two situations at high risks both for the mother and her fetus. There is also an association between the increase in the CS rate and the decrease in perinatal mortality and morbidity, but this effect would only become clinically significant after a dramatic increase in the CS rate: this is the preventile principle of "marginal death". Numerous articles have been published reporting on the effects of vaginal delivery for the pelvic floor: urinary incontinence, pelvic organ prolapse, and especially fecal incontinence. All these publications concluded that CS has a protective effect. The rising duty to provide information to patients in high risk obstetrical situations such as a history of CS also contributes to the overall increase in CS rate mainly through the elective CS rate. Indeed, when faced with the alternative choices of potentially severe complications either for themselves or their child, women are likely to choose what appears to be the safest mode of delivery for their child and thus to opt for a CS. Finally, widespread delivery of information to the patients about trial of labor itself and the risks of vaginal delivery is the first step towards a "principle of preference", which consists in giving an important place to the patient's choice in the decision-making process, and thus to recognize her right to ask for an elective CS.
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PMID:[Evaluation of cesarean rate: a necessary progress in modern obstetrics]. 1548 Feb 87

Prolapse of subconjunctival intraconal orbital fat is a rare cause of an intraorbital mass lesion. Over the past several years, we have seen a number of cases in which this prolapsed fat was confused pathologically with a neoplasm of adipocytic lineage, specifically pleomorphic lipoma and atypical lipomatous neoplasm (well-differentiated liposarcoma). We report the clinical, histopathologic, and immunohistochemical findings in 21 specimens from 17 patients, all of whom presented with prolapsed intraconal orbital fat. All specimens were routinely examined and processed for light microscopy. Immunohistochemistry for CD34, CD68, S100 protein, vimentin, alpha-smooth muscle actin, and Ki-67, and Giemsa, Masson trichrome, and alcian blue histochemical stains were performed. Clinical and follow-up information was extracted from a chart review. The mean age (+/-SD) of the patients was 65.6+/-11.9 years (range: 41 to 85 y); 2 were women and 15 were men. Subconjunctival prolapsed orbital fat was localized in the superotemporal quadrant or lateral canthus around the rectus muscle below the lacrimal gland. The lesions were unilateral in 10 and bilateral in 7 patients. No recurrence was clinically evident over a mean (+/-SD) follow-up time of 2.5+/-3.2 years (range: 1 mo to 13.5 y). Histopathologically, all specimens showed an admixture of mature fat, fibrous septae lacking hyperchromatic cells, adipocytes with intranuclear vacuoles (Lochkern cells), multinucleated giant cells with a wreathlike configuration of normochromatic nuclei (floret cells), and varying numbers of histiocytes, lymphocytes, plasma cells, and mast cells. "Control" sections of normal orbital fat showed occasional Lochkern cells but lacked floret cells. By immunohistochemistry, the floret cells expressed only CD34 and vimentin, whereas the Lochkern cells expressed CD34, S100 protein, and vimentin. We conclude that subconjunctival herniated orbital fat commonly contains multinucleated floretlike giant cells, fibrous septae, and Lochkern cells, features that may result in diagnostic confusion with pleomorphic lipoma and atypical lipomatous neoplasms. Importantly, specific diagnostic features, such as aggregates of bland spindled cells associated with wiry collagen, as seen in pleomorphic lipoma, and enlarged hyperchromatic cells within fibrous septae, as in atypical lipomatous neoplasms, are entirely absent in herniated orbital fat. Multinucleated floret cells present in prolapsed orbital fat likely represent a reactive phenomenon, as they are not present in normal orbital fat.
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PMID:Subconjunctival herniated orbital fat: A benign adipocytic lesion that may mimic pleomorphic lipoma and atypical lipomatous tumor. 1725 63

Whipple disease is a granulomatous infectious disease caused by Tropheryma whipplei. The bacteria accumulate within macrophages, preferentially in the intestinal mucosa. Disease manifestation seems to be linked to immunological abnormalities of macrophages. We describe a patient with cerebral Whipple disease who presented with changes in mental status, confusion, inverse sleep-wake cycle, bilateral ptosis and vertical gaze palsy. Endoscopic biopsy sampling revealed Whipple disease in the gastric antrum but not in the duodenum. Whole blood stimulation displayed reactivity to T. whipplei that was at the lower end of healthy controls while reactivity of duodenal lymphocytes was not diminished. We propose that in cases of neurological symptoms suspicious of Whipple disease with normal duodenal and jenunal findings, biopsy sampling should be extended to the gastric mucosa. The robust reactivity of duodenal lymphocytes may have prevented our patient from developing small bowel disease, whereas the impaired reactivity in peripheral blood lymphocytes might yet explain the bacterial spreading to the central nervous system leading to the rare case of predominant neurological symptoms without relevant systemic involvement.
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PMID:A patient with cerebral Whipple disease with gastric involvement but no gastrointestinal symptoms: a consequence of local protective immunity? 1737 3

We report a case of pituitary apoplexy occurring in a 74-year-old patient 6 hours after cardiac surgery. The patient presented with confusion, unilateral ptosis and ophthalmoplegia. Neurological examination revealed right oculomotor nerve palsy and decreased level of consciousness. Magnetic resonance imaging showed a hemorrhagic and necrotic pituitary macroadenoma. After prompt endocrinological replacement therapy with hydrocortisone and levothyroxine, the confusion of the patient resolved. Removal of a non-functional macroadenoma with large necrotic areas resulted in full recovery. The physician should be aware of pituitary adenoma infarction after open cardiac surgery and should remember that it can be fatal or cause permanent neurological or endocrine damage without proper treatment. Surgical and endocrine treatment can be life-saving procedures.
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PMID:Pituitary apoplexy after cardiac surgery in a patient with subclinical pituitary adenoma: case report with review of literature. 2108 98

Ocular abnormal head posture (AHP) or torticollis is a frequent sign in pediatric pathology The incidence is 5.6% in ophthalmological practice and 3.19% in pediatric ophthalmological practice. The abnormal head posture is adopted to improve visual acuity maintain binocular single vision, center residual visual field with the body or for cosmetic reasons. Face turn is the most frequent abnormal head posture in pediatric ophthalmology. The more common diseases causing face turn are Duane syndrome, congenital fibrosis of extraocular muscles, nystagmus, refractive errors, visual field defects. The most frequent ocular causes of head tilt in children are congenital nystagmus, superior oblique paresis, dissociated vertical deviation, Brown syndrome, refractive errors. Chin-up or chin-down abnormal head postures are most commonly caused by "A" and "V"-pattern strabismus, palpebral ptosis, nystagmus, refractive errors. Torticollis is not a diagnosis, but it is a sign of an underlying disease. There are ocular diseases which diagnosis is straightforward for general practitioner, pediatricians or pediatric surgeons (horizontal nystagmus, lateral rectus paralysis, ptosis, esotropia), but others less obvious (superior oblique paralysis, Duane syndrome, A and V-pattern strabismus, torsional nystagmus) because of the compensatory head posture, and these last disorders are predisposed to confusion with congenital AHP Interdisciplinary collaboration between ophthalmologist, pediatrician, pediatric surgeon, ENT specialist and neurologist is mandatory in establishing the etiology of AHP Every child with AHP must be examined by an ophthalmologist.
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PMID:Understanding ocular torticollis in children. 2177 81


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