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[Meigs' syndrome and "Meigs' pseudo-syndrome." Report of 2 cases].

BACKGROUND: The aim of this study is to investigate similitudis and differences between the Meigs' syndrome and Meigs' pseudosyndrom. The Meigs' syndrome is an uncommon disease that is characterized by benign ovarian tumor, ascites and pleural effusion. The Meigs' pseudosyndrom is a serious disease that is characterized by malignant ovarian tumor, ascites, pleural effusion. METHODS: We have examined two cases: a case of Meigs' syndrome that is characterized by vomit, abdominal pain, ascites, height serum Ca 125 level; a case of Meigs' pseudosyndrom that is characterized by ovarian adenocarcinoma that is diagnosticated owing to ascites and pleural effusion. CONCLUSIONS: This study suggest that the surgical therapy have a very important role for the complete remission of the disease in the Meigs' syndrome and for the remission of ascites and pleural effusion in the Meigs' pseudosyndrom.

Aged↗

Pseudo-Meigs' Syndrome.

Meigs' syndrome is defined as a hydrothorax with ascites and a pelvic tumor, both of which resolve on removal of the tumor. Pseudo-Meigs' is a variant not possessing the original tumor cell types described by Meigs. Both these syndromes should be considered in otherwise healthy women who present with either new or recurrent hydrothorax and ascites. This case concerns a 21-year-old woman who presented to the emergency department (ED) with a recurrent hydrothorax. After performing a pelvic examination that was suspicious for a pelvic mass, further evaluation by ultrasonography showed ascites and a pelvic tumor. Surgeons from the Obstetrics and Gynecology Department performed an exploratory laparotomy with removal of the tumor. Subsequently, there was no recurrence of the ascites and hydrothorax. The tumor was diagnosed histologically as a benign mature teratoma. The literature of Meigs' and Pseudo-Meigs' is reviewed, focusing on the history, pathology, and clinical characteristics. The clinical significance for the emergency physician is discussed.

Adult↗

Blepharospasm-oromandibular dystonia syndrome (Meige's syndrome): clinical aspects.

Meige's syndrome is a form of cranial dystonia characterized by the presence of bilateral dystonic spasms of the facial muscles and frequently of other cranial muscles as well. Its most common and disabling manifestation is blepharospasm which can render the patient functionally blind. Several types of orbicularis oculi spasms occur in Meige's syndrome: brief clonic spasms, prolonged dystonic spasms, constant tonic contraction, and "apraxia" of lid opening. In the completed form of the syndrome, blepharospasm is typically associated with lower facial or oromandibular dystonia. Spasms of the neck and limb muscles, generally mild, and action tremor not uncommonly accompany the cranial dystonia. In most patients the cause of the spasms is unknown. This so-called idiopathic or primary form of Meige's syndrome is considered an adult form of adult onset dystonia. Secondary Meige's syndrome can be encountered in the context of several neurodegenerative disorders, chronic administration of neuroleptics, levodopa, or other drugs, and in patients with focal brain lesions. These secondary cases of Meige's syndrome suggest that a dysfunction of the basal ganglia or of the mesencephalic/diencephalic region plays an important role in the pathophysiology of this dystonic syndrome. Recent neurophysiologic studies and postmortem findings in some patients also support the notion that disease of the brain stem contributes to the pathophysiology of orofacial dystonia.

Basal Ganglia Diseases↗

Brueghel syndrome: its distinction from Meige syndrome.

A critical historical evaluation of the cranial dystonias supports the separation of the dystonia of the motor trigeminal nerve producing a widely opened mouth (Brueghel syndrome) from the more common facial dystonias with blepharospasm (Meige syndrome). In a patient with Brueghel syndrome, paroxysmal hyperpnea coincided with dystonic gaping; the finding of upbeating nystagmus suggests pontine localization in the pathogenesis of this rare disorder.

Aged↗

[Meigs syndrome--diagnostic difficulties].

Meigs' syndrome in 68 years woman with pleural effusion and ovarian tumour was described. The cause of the pleural liquid in Meigs' syndrome is still not clear. New views on the pathogenesis and diagnostic methods of pleural liquids causes were presented.

Aged↗

[Recurrence of Demons-Meigs' syndrome. A case report].

Meigs' syndrome includes ascites, hydrothorax and benign ovarian tumor. The aim of this study was to report a recurrence of Meigs' syndrome in a 71 year old woman, which occurred 30 years after the initial operation. It seems to be the first reported case of recurrence. The high level of CA125 was suggestive of malignancy. Resection of a peritoneal recurrence of the benign fibrothecoma was followed for the second time by improvement of all the symptoms.

Aged↗

Meigs' syndrome in a young woman.

Meigs' syndrome is a rare entity in women under age 30 years. It is characterized by a benign fibrous ovarian tumor, ascites, and hydrothorax. Complete resolution of symptoms occurs with removal of the tumor. The case of a 20-year-old woman with a fibroma and dermoid cyst is presented and the literature reviewed. The etiology of the fluid accumulations remains unclear, although it appears to be related to lymphatic obstruction. Presenting symptoms may include abdominal distension and pain, menstrual irregularities, cough, pleuritic chest pain, and weight gain. Meigs' syndrome and pseudo-Meigs' syndrome should be included in the different diagnosis in young women presenting with a pleural effusion. Resolution of the fluid accumulation usually occurs within 2 weeks of tumor removal.

Adult↗

Bilateral pallidal stimulation for idiopathic segmental axial dystonia advanced from Meige syndrome refractory to bilateral thalamotomy.

Meige syndrome is an adult-onset dystonic movement disorder that predominantly involves facial muscles, while some patients with this syndrome develop spasmodic dysphonia and dystonia of the neck, trunk, arms, and legs. We report that all dystonic symptoms that had been refractory to both pharmacotherapy and bilateral thalamotomy were markedly alleviated by bilateral pallidal stimulation in a patient with segmental axial dystonia advanced from Meige syndrome.

Dominance, Cerebral↗

[Meigs' syndrome: its clinical picture and treatment].

Meigs' syndrome is a rare clinical entity characterised with ovarian benign tumour, ascites and hydrothorax. Between January '94-September '98 we observed three patients with: ovarian neoformation, light (1 patient), moderate (2 patients) monolateral pleural effusion, moderate (2 patients) and considerable (1 patient) ascites. In all patients the preoperative evaluation (sero-haematologic routine, Ca 125 and other oncologic markers, chest X-ray, abdominal and pelvic ultrasonography, total-body Tc, cytological analysis of pleural and abdominal effusion) was suggestive for malignancy but not confirmed it. So an explorative laparotomy with histological extemporary analysis was performed. The results were: 1 fibrothecomas, 1 fibroma, 1 ovarian inflammation with cystic luteinization areas and fibromatosis uterine. All three patients had a good postoperative course. The symptomatology and the effusions disappeared 7-10 days after operation. The first two cases were diagnosed as classic Meigs' syndrome, the third one, instead, as a pseudo-Meigs's syndrome. In conclusion the A.A., according to literature and their experiences, underline: 1) an ovarian mass with pleural and abdominal effusion not always represents an advanced malignancy; 2) even if elevated Ca 125 value is usually associated to a ovarian malignancy, there are some benign lesions in which we observed elevated level of this marker 3) the removal of the ovarian mass is the only resolutive treatment for these patients.

Aged↗

Meigs' syndrome: the history of the eponym.

Meigs' syndrome is defined as presence of ascites with hydrothorax in association with benign ovarian tumor. The syndrome is named after Joe Vincent Meigs (1892-1963), a Harvard Medical School Professor of Gynecology. However, it was described by several authors in the 19th and the beginning of 20th centuries. Meigs and Cass deserve the credit for awakening the medical profession to the importance of the syndrome in 1937. At the end, the syndrome was coined Meigs' syndrome by Rhodes and Terrell in 1937.

Ascites↗

Meige syndrome: neuropathology of a case.

Primary Meige syndrome is a form of cranial dystonia of unknown cause. Only three postmortem studies have been reported, and the results of these studies have not been consistent. We have examined the brain of a 72-year-old man with typical primary Meige syndrome and found mild to moderate cell loss in the zona compacta of the substantia nigra, locus ceruleus, midbrain tectum, and dentate nucleus of the cerebellum. Also frequent Lewy bodies were present in pigmented nuclei of the brainstem. No abnormalities were detected elsewhere. These pathological findings support the notion that brainstem pathology is important in the pathophysiology of cranial dystonia.

Aged↗

[Perioperative management of patients with Meigs syndrome].

We report our perioperative management of three cases of Meigs syndrome. The major pre-operative problems in Meigs syndrome are physical trouble caused by giant mass in peritoneal space, respiratory distress, and poor nutrition. These problems must be settled before the operation. The important points in the pre-operative management are 1) respiratory care employing the intermittent positive pressure breathing (IPPB) and the pleural effusion drainage, and 2) the correction of intravascular volume and the concentration of albumin and hemoglobin by transfusion of massive lactated Ringer solution and albumin solution and/or whole blood when they are necessary. During the operation, the epidural anesthesia under spontaneous breathing is the best method of anesthesia. According to circumstances, we adopt the intra-tracheal intubation with continuous positive airway pressure breathing (CPAP). We can generally deal with excessive bleeding by transfusion of lactated Ringer solution and plasma expander, during the first half of operation. By the end of the operation, however, the correction of the concentration of albumin and hemoglobin must be made by the fresh frozen plasma and blood transfusion. After the operation, we use epidural analgesia to control the postoperative pain. We have succeeded in the treatment of three cases of Meigs syndrome owing to our perioperative management as described above.

Analgesia, Epidural↗

Conjunctival edema and alopecia of the external third of the eyebrows in a patient with Meige syndrome.

PURPOSE: To describe a patient with Meige syndrome in whom we observed the coexistence of hereditary lymphedema of the lower legs, conjunctival edema and alopecia of the lateral third of the eyebrows. METHODS: Case report. RESULTS: Histological examination of the conjunctival and skin specimens showed dermal edema and a slight reduction in the number of severely ectatic lymphatics in the reticular dermis. The vessel were identified as lymphatics on the basis of immunohistochemical evidence of discontinuity and/or absence of basement membrane. CONCLUSIONS: Clinical and histological findings suggest that the etiopathogenesis of the edema in Meige syndrome is related to a structural ectatic defect of lymphatics. This anomaly seems to involve both skin and other sites, such as conjunctival mucosa.

Adult↗

Pseudo-Meigs' syndrome due to broad ligament leiomyoma: a mimic of metastatic ovarian carcinoma.

Pseudo-Meigs' syndrome is a rare complication of benign leiomyomas of the female genital tract. We report a patient with pseudo-Meigs' syndrome due to a large broad ligament leiomyoma, which also caused bilateral reversible hydronephrosis. This unusual combination of pseudo-Meigs' syndrome, broad ligament leiomyoma and hydronephrosis requiring ureteric stenting does not appear to have been reported previously. Features of the syndrome that led to a diagnostic problem, the mimicking of metastatic ovarian carcinoma, are presented. The little that is known of the pathogenesis of the pleural and ascitic fluids is discussed. An elevated level of serum CA 125 antigen is reported, we believe for the first time in association with pseudo-Meigs' syndrome.

Adult↗