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Mayer-Rokitansky-Kuster-Hauser syndrome with splenosis. A case report
J A Griggs1, J Rudoff, C C Coddington
1Department of Obstetrics and Gynecology, Portsmouth Naval Hospital, Virginia.
The Journal of Reproductive Medicine
|August 1, 1990
Summary
This case report details the first instance of co-occurring pelvic splenosis and Mayer-Rokitansky-Kuster-Hauser syndrome. Laparoscopic biopsy is recommended for diagnosing splenosis in patients with congenital anomalies and suspected endometriosis.
Area of Science:
- Gynecology
- Surgical Pathology
- Reproductive Medicine
Background:
- Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a congenital disorder affecting the female reproductive system.
- Pelvic splenosis, the presence of ectopic splenic tissue in the pelvis, is rare and often associated with abdominal surgery or trauma.
- Simultaneous occurrence of these conditions has not been previously documented.
Observation:
- A patient presented with pelvic pain, initially suspected to be endometriosis.
- Laparoscopy revealed lesions prompting further investigation.
- The patient had no history of abdominal trauma or surgery and no other congenital anomalies.
Findings:
- Histologic evaluation confirmed splenosis after tissue removal during laparotomy.
- The co-occurrence of pelvic splenosis and MRKH syndrome was established.
- This represents the first reported case of these two conditions occurring simultaneously.
Implications:
- This case highlights the importance of considering splenosis in the differential diagnosis of pelvic masses, especially in the context of congenital anomalies like MRKH syndrome.
- Laparoscopic biopsy can be crucial for accurate diagnosis when endometriosis is suspected but splenosis is a possibility.
- Further research may be needed to understand the potential relationship or predisposition between these conditions.