Cervical Atresia: A Challenging Diagnosis

hours of dialysis the patient experienced rapid resolu-tion of her bleeding.

Case 3: 12 ½ year old female was admitted withthrombocytopenia, anemia and vaginal bleeding. Shegave a 3-month history of nose and gum bleeds.The patient had her menarche 6 months prior andhad 2 bleeding episodes since then, lasting 5 and 14days respectively. Extensive work up included a nor-mal bone marrow biopsy, low iron levels, normal totaliron binding capacity and normal clotting factors. Achest x-ray was normal. The ANA was elevated andthe complement levels were low. She was diagnosedwith Systemic Lupus Erythematosus and started onimmunotherapy. She has had regular menses for 4consecutive months.

Case 4: 13 year female sent referred for oligome-norrhea with variable flow. Adiagnosis of McCuneAlbright syndrome was made at age 3 when she pre-sented with precocious puberty and vaginal bleeding.She was started on medication to reverse pubertalchanges and it was discontinued at 10 years of age.Since then she has had oligomenorrhea with no othersigns of endocrine dysfunction. On exam, she wasTanner V with no cafe au lait spots or signs of virili-zation. Laboratory evaluation was unremarkable andpelvic ultrasound showed a small left ovarian cyst.

Comments: Not all cases of irregular or heavybleeding in adolescents are related to physiologic an-ovulation. A careful history and physical will indicatethe need for further workup. In addition to the chal-lenge of controlling the uterine bleeding, there is theneed to manage contraceptive needs when hormonalmedications may not be the safest choice.

doi:10.1016/j.jpag.2006.01.042

155NASPAG 20th Annual Clinical Meeting

Cervical Atresia: AChallenging Diagnosis

Anjali Mehta, MD, (submitting and presentingauthor), Andrea Zuckerman, MD, (seniorauthor), and Ann J. Davis, MDNew England Medical Center, Boston, MA

Background: Diagnosing cervical atresia can bea challenging diagnosis, even with the assistance ofMRI. This is a case report of a patient with cervicalatresia that was diagnosed through several imagingstudies and visits to the operating room.

Case: A 15-year-old female presented with primaryamenorrhea and a 2 month history of worsening cy-clic pelvic pain. At age 13, she began having cyclic

pelvic pain lasting for 6 days, worsening over theprior months. The patient never menstruated, but oth-erwise had normal development with no significantmedical history. On exam, the lower abdomen wasdistended with tender bilateral masses. Inspection ofexternal genitalia was tanner stage 5, with a patentand cresenteric hymen. The vagina was found to bepatent, and there was thought to be an bulging trans-verse septum. Patient’s TSH, Prolactin, FSH, Estra-diol and CBC were within normal. MRI showed7�5�4cm hematometra, bilateral hematosalpinges,and two large blood filled masses anterior and poste-rior to the cervix.

Under anesthesia, exam was consistent with a trans-verse vaginal septum less than 1 cm thick. The vaginalapex was incised, and dark clotted blood was ex-pressed. A closed cervix was thought to be palpatedthrough the incision, but the cervix was not visual-ized. Several days after the procedure, the hematocol-pos stopped draining. An MRI was repeated, with newsignificant findings including a cervix that appearedthin and stretched with no endocervical canal seen,and no evident vaginal septum or hematocolpos.The patient and her family were extensively coun-seled on reproductive limitations and options, as wellas surgical outcomes. The patient then underwent anexploratory laparotomy revealing a large right endo-metrioma 10�5cm, two large hematocolpos, and bi-lateral hematosalpinges, and a large uterus withhematometra. A hysterotomy revealed a normal uter-ine cavity, but no patent cervical canal. An intra-oper-ative hysterosalpingogram confirmed the diagnosis ofcervical atresia and the absence of a patent cervix. Re-canalization of the cervix was considered, but this pa-tient was not an ideal candidate due to poor compli-ance with follow-up and challenges accessingmedical care. A total abdominal hysterectomy and bi-lateral salpingectomy were performed without com-plication with the ovaries left intact bilaterally. Finalpathology established the diagnosis of distal cervicalatresia with proximal cervical stenosis, and a normaluterine cavity.

Conclusion: MRI is beneficial in recognizing mul-lerian tract anomalies in most cases. In this case ofcervical atresia, the extent of the anomaly and defin-itive diagnosis could only be reached though anexploratory laparotomy with hysterotomy and intrao-perative hysterosalpingogram. A discussion shouldtake place pre-operatively on possible outcomes,including surgical sterilization or re-canalization re-quiring close follow up and access to specializedmedical care.

doi:10.1016/j.jpag.2006.01.043