Chiari Frommel Syndrome

Chiari Frommel Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Chiari Frommel Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Frommel-Chiari Syndrome
  • Lactation-Uterus Atrophy
  • Postpartum Galactorrhea-Amenorrhea Syndrome

Disorder Subdivisions

  • None

General Discussion

Chiari-Frommel Syndrome is a rare endocrine disorder that affects women who have recently given birth (postpartum) and is characterized by the over-production of breast milk (galactorrhea), lack of ovulation (anovulation), and the absence of regular menstrual periods (amenorrhea). In Chiari-Frommel Syndrome, these symptoms persist long (for more than six months) after childbirth. The absence of normal hormonal cycles may result in reduced size of the uterus (atrophy). Some cases of Chiari-Frommel Syndrome resolve completely without treatment (spontaneously); hormone levels and reproductive function return to normal.

Symptoms

Chiari-Frommel Syndrome is a rare disorder characterized by the abnormal production of breast milk (galactorrhea), and the absence of regular menstrual periods (amenorrhea) and ovulation (anovulatory) for more than 6 months after childbirth. These symptoms occur even though the mother is not nursing the baby. The pregnancy which precedes the onset of Chiari-Frommel Syndrome is usually normal, and childbirth and initial lactation are uneventful. However, normal menstrual periods and ovulation do not resume, and persistent discharge from the nipples occurs, which can sometimes last for years. Other symptoms may include emotional distress, anxiety, headaches, backaches, abdominal pain, impaired vision, and occasionally obesity. Women who have Chiari-Frommel Syndrome for a long time may also have a loss of muscle tone in the uterus and diminished uterine size (atrophy).

Causes

The exact cause of Chiari-Frommel Syndrome is not fully understood but may be related to an abnormality of the hypothalamus and/or pituitary glands. Some research suggests that microscopic tumors of the pituitary gland (microadenomas), stimulated by the hormones associated with pregnancy (e.g., prolactin, a stimulator of lactation) are responsible. When such microtumors grow, they may be detected by imaging techniques (CT scan or MRI). Approximately 50 percent of affected women eventually resume normal menstruation over a period of months or years.



The cause of the abnormal hormonal relationship between the pituitary and hypothalamus gland associated with Chiari-Frommel Syndrome is not known. Some studies suggest that microscopic lesions of the hypothalamus may also cause Chiari-Frommel Syndrome. An association with the use of oral contraceptives has also been suggested.

Affected Populations

Chiari-Frommel Syndrome is a rare disorder that affects females who have recently given birth (postpartum).

Standard Therapies

Some women with Chiari-Frommel Syndrome may have abnormally high levels of prolactin in the blood. Other women have normal prolactin levels. Additional laboratory findings may include abnormally low levels of estrogen and other hormones (gonadotropins) in the urine.



The drug bromocriptine may be prescribed to help reduce prolactin levels. When these levels are reduced, normal ovulation cycles may be restored along with regular menstrual periods.



If the symptoms persist for a long period of time, affected individuals should be monitored (CT scan or MRI) for the presence of a pituitary tumor. If a tumor is discovered, it may be difficult to treat if it is very small. Larger tumors may be surgically removed.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:1933-38.



Frantz AG, Wilson JD. Endocrine Disorders of the Breast. In: Wilson JD, Foster DW. Eds. Textbook of Endocrinology. 8th ed. W.B. Saunders Company. Philadelphia, PA; 1992:959.



JOURNAL ARTICLES

Matsuno A, Ogino Y, Itoh J, et al. detection of a silent pituitary somatotroph adenoma in a patient with amenorrhea and/or galactorrhea: paradoxical response of GH in TRH or GnRH provocation test. Endocr J. 2000;47 Suppl:S105-09.



Whitman-Elia GF, Windham NQ. Galactorrhea may be clue to serious problems. Patients deserve a thorough workup. Postgrad Med. 2000;107:165-68, 171.



Olive D. Indications for hyperprolactinemia therapy. J Reproduct Med. 1999;44(12 Suppl):1091-94.



Luciano AA. Clinical presentation of hyperprolactinemia. J Reproduct Med. 1999;44(12 Suppl):1085-90.



McLennan MK. Radiology rounds: Pituitary microadenoma (prolactinoma). Can Fam Physician. 1998;44:2396-98.



Forsbach G, Olivares F, Vazquez J, et al. [Disappearance of radiographic image of a macroprolactinoma after treatment with bromocriptine] Ginocol Obstet Mex. 1998;66:170-72.



Katznelson L, Klibanski A. Prolactinomas. Cancer Treat Res. 1997;89:41-55.



Tartagni M, Nicastri PL, Diaferia A, et al. Long-term follow-up of women with amenorrhea-galactorrhea treated with bromocriptine. Clin Exp Obstet Gynecol. 1995;22:301-06.



FROM THE INTERNET

McKusick VA, Ed. Online Mendelian Inheritance In Man (OMIM). The Johns Hopkins University. Entry Number; 104600: Last Edit Date; 8/15/1994.



Dambro MR. Chiari-Frommel syndrome. nd. 1p.

www.5mcc.com/Assets/SUMMARY/TP0189.html



Wysolmerski J, Van Houten JN. Disorders of Breast Development. In: Burrow GN. Ed. Endocrinology of Pregnancy. 8pp.

www.endotext.org/pregnancy/pregnancy5/pregnancy5_2htm



Chiari-Frommel Syndrome. HONselect. Last Modified: Jan 29 2003. 2pp.

www.hon.ch/cgi-bin/HONselect

Resources

Abraham Low Self-Help Systems

105 W. Adams St., Ste. 2940

Chicago, IL 60603

USA

Tel: (312)337-5661

Fax: (312)726-4446

Tel: (866)221-0302

Email: info@lowselfhelpsystems.org

Internet: http://www.lowselfhelpsystems.org/



RESOLVE: National Infertility Association

1760 Old Meadow Rd

Suite 500

McLean, VA 22102

Tel: (703)556-7172

Fax: (703)506-3266

Email: info@resolve.org

Internet: http://www.resolve.org



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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