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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

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

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

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

Pseudo-Meigs' syndrome caused by paraovarian fibroma.

Meigs' syndrome includes an ovarian tumor, usually fibroma, associated with hydrothorax and ascites. It is accepted that uterine tumors, like fibromas, can also be associated with ascites and hydrothorax, but this is extremely rare. The mechanism of formation of peritoneal and pleural effusion is not well documented. The most likely pathogenesis ascribes the fluid formation to the filtration of interstitial fluid in the peritoneal through the tumor capsule, and the diffusion to the pleural space through the diaphragm lymphatic vessels at the foramen of Bochdalek. Paraovarian fibromas are also extremely rare neoplasms, probably of paramesonephric origin. It has been hypothesised that they can develop by proliferation of connective tissue cells around the Wolfian remnants. In this article, probably for the first time, a case of paraovarian fibroma with ascites and hydrothorax is presented.

Ascites

Hydropic degenerating leiomyoma presenting as pseudo-Meigs syndrome with elevated CA 125.

BACKGROUND: Leiomyomas rarely cause pseudo-Meigs syndrome. Increased levels of CA 125 often are associated with some types of malignancy. No reported case of pseudo-Meigs syndrome presenting with hydropic degeneration of uterine leiomyoma and an elevated CA 125 level could be found on a MEDLINE search. CASE: A 46-year-old woman presented with a pleural effusion and a pelvic mass measuring 30 x 18 cm. Preoperative evaluation was remarkable for a CA 125 level of 254 U/mL. At laparotomy, the diagnosis was a benign leiomyoma with focal hyaline and extensive hydropic degeneration. Her pleural effusion resolved completely by 4 months postoperatively. CONCLUSION: Pseudo-Meigs syndrome can present with an elevated CA 125 level.

CA-125 Antigen

Atypical Meigs' syndrome and bilateral ovarian stromal hyperplasia. A case report.

BACKGROUND: Atypical Meigs' syndrome has been observed in patients with dermoid tumors, struma ovarii, uterine leiomyomata and other benign pelvic tumors except for ovarian fibromas. Meigs' and atypical Meigs' syndrome present management decisions complicated by a high index of suspicion for malignancy. CASE: A 43-year-old woman, gravida 1, para 1, with ascites; a pleural effusion; radiologic evidence of enlarged, cystic adnexa; and a normal CA-125 level was found to have cortical stromal hyperplasia on bilateral ovarian pathologic evaluation. CONCLUSION: This is the first case of cortical stromal hyperplasia presenting with bilateral involvement of small ovaries, ascites and a pleural effusion. Meigs' syndrome and its variants develop with clinical pictures suggestive of malignancy. Thorough evaluation and individualized treatment are necessary.

Adult

[Meigs syndrome presenting as diaphragmatic pleural nodules].

A 52-year-old woman was admitted to our hospital because of non-productive coughing and dyspnea on exertion. A chest X-ray film revealed a moderate pleural effusion. The effusion was persistent and progressive in spite of repeated thoracentesis. Because examinations of the effusion did not result in a specific diagnosis, thoracoscopy was done under local anesthesia. Two nodules were found in the surface of the diaphragmatic pleura. Examination of biopsy specimens revealed mesothelial hyperplasia and lymphatic duct proliferation without malignant or granulomatous lesions. Systemic examinations revealed a tumor in the right ovary. The tumor was removed, and examination revealed that it was a serous adenofibroma without malignant lesions. Meigs syndrome was diagnosed. The pleural effusion disappeared quickly, two weeks after the operation. We know of no previous report of diaphragmatic pleural nodules in a case of Meigs' syndrome. Meigs' syndrome should be considered in the differential diagnosis of pleural nodules.

Adenofibroma

[Meigs syndrome with bilateral hydrothorax].

The co-existence of pelvic tumor, hydrothorax and ascites has been known since the last century. The features of this disease were described by Meigs and Cass in 1937; in the same year Roads named it Meigs syndrome. According to the original description this syndrome only included, as pelvic involvement, an ovarian neoplasm; at present it is accepted that hydrothorax and the ascites can also be associated with a uterine tumor, like a fibroma. The existence of either an ovarian or a uterine neoplasm distinguishes the typical Meigs syndrome from a pseudo-Meigs syndrome. The most likely pathogenesis of Meigs syndrome ascribes the formation of the peritoneal and pleural effusion to the filtration of interstitial fluid in the peritoneum through the tumor capsule, and the diffusion to the pleural space, generally at the right side, through the diaphragm lymphatic vessels and the foramen of Bochdalek. Dockerty reported that at least 40% of ovarian tumors had a diameter of more than 6 cm when associated with hydrothorax and ascites. The entity of pleural and peritoneal effusion can be moderate or massive. The effusions generally derive from a transudative process, but they can occasionally contain blood cells. The connection between the pelvic tumor and the effusion is demonstrated by the regression of the latter when the neoplasm is excised. When the pelvic tumor has an ovarian location it derives from the connective tissue of the hilus, it appears during fertile age and has a slow growth, the clinical signs becoming evident in elder age.

Aged

Spontaneous remission in patients with essential blepharospasm and Meige syndrome.

PURPOSE: To determine the frequency of remission in patients with essential blepharospasm and Meige syndrome. METHOD: Retrospective analysis of patients by means of questionnaire in an institutional setting. RESULTS: Among 238 patients diagnosed in a single institution as having essential blepharospasm or Meige syndrome, 27 (11.3%) claimed to be symptom free without curative surgery. The average time between onset and resolution in the patients who claimed remission was 4.85 years (range, 3 months to 22 years). Among these patients, the duration of remission averaged 6.33 years (range, 1 year to 14 years). CONCLUSION: Patients with essential blepharospasm or Meige syndrome have a small but definite potential for spontaneous remission of symptoms, particularly within the first 5 years after onset of symptoms.

Blepharospasm