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• The girl had cystic adenomyosis (also known as accessory cavitated uterine masses or ACUM), a congenital Müllerian anomaly that develops as a tiny uterus in the main uterus.
• The patient, a medical student, developed severe dysmenorrhoea since past three years which was increasing and of late, could be controlled with an injectable painkiller only.
• ACUM is a rare anomaly with only 75 reported cases so far and a handful of them were treated laparoscopically.
• The surgery was conducted by a team of doctors led by Padmashree, Ex- Prof AIIMS, Dr. Alka Kriplani, Head of Minimally Invasive Gynaecology at Paras Hospitals a pioneer in advanced laparoscopic surgeries in gynaecology and Dr. Kusum Bhardwaj, Consultant, Minimally Invasive Gynaecology.

Gurgaon, 11.04.2019: A team of doctors at Paras Hospitals, Gurgaon has conducted a successful laparoscopic surgery on a 22-year-old girl to treat an extremely rare uterine malformation that appears as a tiny uterus inside the main uterus. Cystic adenomyoma, also known as accessory cavitated uterine masses (ACUM) is a rare and highly under diagnosed condition with only 75 reported cases so far.

The 22-year old patient, a medical student, was experiencing severe dysmenorrhoea or painful periods since past three years with the condition progressively becoming worse. Despite seeking multiple opinions and treatments from a series of doctors – from gynaecologists to psychologists, her condition remained undiagnosed.

Her rare condition was finally diagnosed by doctors at Paras Hospitals, Gurgaon who used extensive imaging tests to identify the malformation in her uterus.

“She developed dysmenorrhoea 5 years after attaining menarche (occurrence of the first menstruation) due to gradual collection of blood in the accessory uterine which became swollen and caused pain. She sought consultations of gynaecologists and received treatment for polycystic ovarian disease (PCOD) and fibroids. However, despite normal blood parameters, her pain did not subside. So, ultrasound  and MRI were done and the reports shared query subserosal fibroid. Reports were reviewed by the experts where doubt of ACUM was raised – it is a rare disease with only 75 reported cases so far and a handful of them were treated laparoscopically,” says Padmashree, Ex-Prof AIIMS, Dr. Alka Kriplani, Head of Minimally Invasive Gynaecology, Paras Hospitals, Gurgaon.

While initially, the pain experienced by the patient had subsided by usual remedies such as painkillers, nonsteroidal anti-inflammatory drugs or NSAIDs and oral contraceptive pills, of late her condition could be relieved only with an injectable painkiller. Incidentally, a large number of women experiencing this condition fail to find appropriate help as the condition remains undiagnosed.

Accessory and Cavitated Uterine Mass (Uterus in Uterus) is a rare form of developmental Mullerian anomaly. The development of a cavitated mass inside a normal uterus presents as severe dysmenorrhoea and pelvic pain. It requires a high level of expertise to diagnose this rare condition. The cavitated mass is lined by a functional endometrium and surrounded by smooth muscle cells, lending it significant similarity to the uterus.

“We opted for this advanced process (laparoscopic surgery) since it has a better precision and allows the patient to go back to work sooner than before – laparoscopy provides a minimally invasive approach to such young girls who come to us with medically refractory pain,” adds Dr Kriplani, a pioneer in advanced laparoscopic surgeries in gynaecology.

The patient recovered fully and was discharged the next day. She was guided on post-operative care i.e. a USG in every six months. She came for the follow up visit after a month and her previous pain was completely cured.

The other doctors who formed the team to treat the young girl were Dr. Kusum Bhardwaj, Consultant, Dr. Seema Sharma, Consultant.

This Case Highlights –

• ACUM(Accessory and Cavitated Uterine Mass or Uterus in Uterus) is a very rare congenital disease but may lead to severe incapacitating pain.
• In young girls, severe dysmenorrhea should not be taken as normal and probability of ACUM should be always kept in mind as medical treatment in not helpful.
• Patient suffers from extreme pain leading to severe mental stress culminating in psychological stress.
• Awareness must be spread among patients as well as physicians (Gynaecologists / Radiologists). Accurate diagnosis and surgical management gives complete cure and can change quality of life forever.

Paras Hospitals Gurgaon is among the leading providers of treatment and care in the field of neurosciences, cardiology, orthopedics, nephrology, gastroenterology, and critical care in India.