[NSFW] MRKH Syndrome: Legends, Urban and Historical

Disclaimer: Contains strong language, sexually explicit in nature in order to explain bodily functions in a non-jargon manner. Maybe NSFW.

This week we had a fabulous case presentation in the department. The patient was suffering from Atypical Mayer-Rokitansky-Kuster-Hauser Syndrome, as evidenced by the absence of the uterus and cervix, with only the distal part of vagina being present. In addition, she had left renal agenesis. However, she had a hypoplastic left thumb and thus, I was of the opinion that there was a possibility that she could have been suffering from MURCS Association, in which, in addition to the Mullerian Agenesis, the patients suffer from renal dysgenesis, cardiac anomalies and skeletal anomalies. The patient had not been worked up for cardiac anomalies though. It is always interesting to study such complicated cases in the community setting, and gives us a perspective into the psychosocial angle of a multi-faceted pathology.

While doing some background reading on the MRKH Syndrome after the case presentation, I stumbled across two rather remarkable stories connected to the syndrome and thought that it would make a good topic for a comeback post on the blog. There has been a lot of goings on in terms of career and life, and hence, for a few months, the blog kind of took a back seat. Now that I am getting a better handle on things, hopefully, I shall be blogging more often. Now, coming back to the stories. These are two inordinately interesting stories about MRKH Syndrome which reflect diametrically opposed facets. In one, we speak of a pregnancy in a patient with MRKH-like Syndrome and in the other, about the effects of the lack of one. So, onto the stories.

MRKH Syndrome, Infertility and the fall of a Monarchy:

1833: The 10-year war for independence has recently concluded and the first real dynasty of independent Greece was put into place as the 18-year-old King Otto ascended the throne at Athens amidst much fanfare and expectations. The people’s monarchy was supposed to bring peace and stability to the unified independent country. However, in course of his monarchy, he failed to execute one of the most important duties of the crown – to produce an heir to the throne.

Many academics were of the opinion that Queen Amalia, his wife, may have been responsible for this. There is a strong school of thought that supports the hypothesis that the Queen suffered from MRKH Syndrome. (1) Although some court physicians have reported the presence of a uterus, the opinion is divided on it since the clinical examination were not uniform and the reports varied in their accuracy and details as well. The research by Vladimiros and colleagues (2) strongly lobby for the fact that King Otto was not responsible for the failure to produce an heir. Although the reports of the German court physicians tend to support the fact that they believed that Queen Amalia had a uterus, Vladimiros et al chose to follow lines of query that led them to the theory that the queen suffered from an atypical variant of the MRKH Syndrome.

The inability of the royal couple to present the nation with an heir led to a serious fall-out. The newly emancipated people soon turned against the dynasty. Things deteriorated so much that every attack on the monarchy from the Greek Opposition would invariably comprise of some sort of jab at the queen’s inability to bear a child or the king’s anatomic insufficiency.

This lack of  an heir then became a larger issue with the people at large. Ultimately, largely fuelled by the inability to provide the nation with an heir, the dynasty had to abdicate the throne only after a single generation. The Danish dynasty then took over Greece and her colonies in 1863.

Pregnancy in Vaginal Aplasia:

Now that the story of the lack of a pregnancy is done with, it is time to highlight the misfortune of a young lady with an anatomic defect that should have made pregnancy impossible; but she did the impossible anyways! So I came across this rather remarkable article (3) in which the story of how a patient who had vaginal aplasia ended up getting pregnant. Once you read this story, you shall understand why Wikipedia insists that this methodology should not be pursued in common course in order to get MRKH Syndrome patients pregnant. (4) To be entirely accurate this patient was not suffering from MRKH Syndrome, but rather from vaginal aplasia. Anyways, after attempting vaginal sex and being disappointed, she took to oral sex. On one such occasion where she was gratifying a partner, a former lover turned up and the resulting tension spawned a knife fight. In the resulting skirmish, the girl ended up with a stab wound to the abdomen, with her stomach being perforated in two places.

Intraoperatively, her stomach was found to be empty and despite the absence of gastric contents in the peritoneal cavity, it was lavaged and closed in layers. The patient improved subsequently and was discharged following an uneventful 10-day post-operative recovery period.

Exactly 278 days after this event, she presented to the hospital with acute, intermittent abdominal pain. Physical examinations and investigations revealed a term pregnancy. Now this pregnancy was being carried in her uterus, so she was clearly not a case of MRKH Syndrome, and was, rather, suffering from cervico-vaginal aplasia. However, since these conditions are very similar anyways, I decided to include it in the narrative.

A cesarean section was performed and a baby boy, weighing 2800 gms was delivered under spinal anesthesia. The much-surprised mother, the young father and the baby boy thereafter lived happily ever after in their dysfunctional lives.

The interesting thing is that the sperm lived through the period of operative procedure and managed to wend their way through the gastric perforations and then across the peritoneal wastelands and ended up in the right place, at the right time. One hell of a way to get pregnant, if ever!

References:

1. E Poulakou-Rebelakou, C Tsiamis, N Tompros, G Creatsas The lack of a child, the loss of a throne: the infertility of the first royal couple of Greece (1833–62) J R Coll Physicians Edinb 2011; 41:73–7 doi:10.4997/JRCPE.2011.115

2. Vladimiros LE, Diamantis AG, Androutsos GI. [The cause of sterility of the queen Amelia.] Athens: Zeta; 2008. p. 44–60. In Greek.

3. Verkuyl DA. Oral conception. Impregnation via the proximal gastrointestinal tract in a patient with an aplastic distal vagina. Case report. Br J Obstet Gynaecol. 1988 Sep;95(9):933-4. PubMed PMID: 3191066.

4. Wikipedia: Mullerian Agenesis aka MRKH Syndrome

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