INDEXING TITLE: RCHAN’S MAR [5-08]

TITLE:  A Blooming Flower Cut Down

PERIOD OF MEDICAL OBSERVATION: September 23, 2005

NARRATION:

         The patient was a 13-year-old female who underwent transverse colostomy for an imperforate anus in another institution soon after birth. When she was 5-months-old, she underwent posterior sagittal anorectoplasty and consequently after a few weeks, closure of the colostomy. However, after a few days, her abdomen enlarged and underwent adhesiolysis.  As if her misfortune was not enough, her incision site developed surgical site infection. She now sought consult at OMMC presenting with a midline intraabdominal hypogastric mass of 3 days duration accompanied by pain.  Upon physical examination, the mass measured 6 x 5 cm, firm, non-movable and tender on palpation.  On rectal exam, there was pararectal tenderness anteriorly.  By location and character of the mass we deuced that it might be an Ob-Gyne case.

        We referred the patient to the Department of Obstetrics and Gynecology. Both departments agreed that a pelvic ultrasound and a transrectal ultrasound was essential in this case. Initial ultrasound was non-contributory, the radiology resident was not able to visualize the left ovary, was only able to partially visualize the uterus and unable to measure the right ovary.  We decided to go for rescanning the following day wherein a Radiology consultant would be present and a bowel preparation can be carried out as well.

         As we were making our rounds that night, my co-resident and I reviewed the history of the patient which revealed that the patient still had no menarche despite the development of secondary sexual characteristics.  The mass was also noted to enlarge every month for the last two years, noting it to be a size of a calamansi initially.  The mass was associated with pain on a cyclical monthly basis. I inspected the vaginal introitus and noted it to be almost closed. It was as if I was looking at an anal opening.  The mother informed us that the patient had labial fusion and the pediatric surgeon at that time even removed the fusion.  So putting the pieces of the puzzle together, we concluded that the mass is an enlarged uterus filled with menstrual blood because of an outflow obstruction- what we call HEMATOMETRA. The cause of obstruction, we still do not know.  She was ovulating every month, which also accounts for her secondary sexual characteristics. She was supposedly menstruating every month as well,  but then because of a congenital anomaly, the blood could not go out, causing it to collect within the uterus.  We referred back to our consultant and she entertained the possibility of such an entity to be affecting the patient.

         The repeat ultrasound revealed a complex ovarian mass on the right, normal left ovary, a small uterus and a hydronephrotic right kidney.  We were back to square one…so hematometra was ruled out.  Anyway since the patient had an ovarian mass and on physical examination and had signs of peritoneal irritation, our consultant advised us to open up the patient.  She will assist us during the procedure which most likely be cystectomy of the right ovary.

         When we opened up the patient, even our consultant had difficulty identifying and making sense of what we saw… there was a midline cystic mass measuring 6 x 6 cm, from where it was coming from we could not determine.  We could not identify the uterus and the right ovary.  The left fallopian tube and ovary was a rudimentary horn with a paratubal cyst, completely separate from the uterus (which we still had difficulty locating). It was attached to the left pelvic brim.  We mobilized the mass and aspirated its contents. It had old blood and chocolaty material within it. Inferior and lateral to the mass was another cystic mass measuring 4 x 4 cm which we accidentally punctured and contained the same material. We were still in the dark as to where the uterus was located. We asked the OB residents to scrub in and refer back to their consultant.  He said that it was a case of pelvic endometriosis. Their plan was to do piecemeal resection of everything- a left salpingiectomy,  removal of the midline mass and the cystic mass inferolateral to it,  and  hysterectomy. Remember that than patient was just 13-year-old and doing such a radical operation would scar her womanhood for life. We had to get consent from the mother.  We had to wait for almost an hour for her to come back. She readily gave us the consent, seemingly oblivious to our repeated explanations and the gravity of the consequences of the procedure.

         We resected the midline mass, which on close inspection was a dilated coiled up right fallopian tube filled with blood.  We dissected the cyst inferolateral to it, which we noted to either be an endometrial cyst or an ovarian cyst.  Finally, we were able to identify a 4 x 5 cm muscular tubular structure, consistent with a uterus.  Hysterectomy was done. Upon inspection of the proximal vaginal canal, it ended blindly in a pouch. Upon doing IE, the distal vaginal canal was less than 2 cm in length. The OB consultant who scrubbed in with us told us that the patient had a transverse vaginal septum.  She was menstruating, but because of the septum, blood could not go out.  Blood back flowed to the right fallopian tube and right ovary, causing the endometriosis. He explained to us the logic in doing the procedures he asked us to do.  The left ovary had to be spared for maintenance of her hormones.  Since the right fallopian tube and right ovary were pathologic, they had to be removed.  There would still be an intact supply of hormonal stimulation from the left ovary, thus, her endometrium would still undergo its usual

cycle.  Since there was no outflow tract, blood would again be trapped within the uterus, rendering it vulnerable to endometriosis again. Her vaginal septum would be surgically addressed later on.

INSIGHTS: (DISCOVERY, Stimulus, Reinforcement, physical, psychosocial, ETHICAL)

         Definitely this case is one for the books.  I am lucky to have witnessed and participated in a case such as this.  I will truly remember and cherish the experience. It’s not everyday that you can encounter a patient with multiple congenital anomalies as extensive and as complicated as she had.  Managing this patient reminded me of a few things… History is very important.  Her past medical history was very relevant to her case.  She had an imperforate anus and labial fusion.  Patients with these anomalies are most likely to have other associated congenital anomalies.  Hers manifested as a reproductive system defect which only became evident during the onset of puberty.  Her menstrual history was very contributory to arriving to a possible diagnosis as well.  She still had no menarche at her age and yet she was fairly developed already for her age, thus ruling out a hormonal cause for the amenorrhea. The mass was said to be enlarging every month for the past two years associated with monthly cyclical pain.  This might be attributed to the presence of menstruation all the while, only for her menses to be trapped within the uterus and adnexa.  Thus, the sequestered blood manifested itself as endometriosis, accounting for the palpable mass.

         As we proudly snagged the diagnosis, here comes the UTZ result of an ovarian mass and a small uterus…instead of a paraclinical diagnostic procedure assisting us in the arrival of a more definite diagnosis, it confused us further.  Oh well, I told myself, anything is possible. True enough, the sonologist too was mislead in interpreting the results.   The anatomic relationships were distorted intraoperatively, which may account for the discrepancy in the initial reading.  In the advent of diagnostic procedures, it is still better to stick to the good ol’ stuff we were taught in medical school- an accurate HISTORY AND PE.  We must not always be swayed and be influenced by laboratory and diagnostic procedures.  They are but adjuncts to diagnosis,  and the clinical eye should always precede first before anything else.

         As we discuss the merits of this case, we must be reminded of the fact that we just rendered a 13-year-old female surgically barren for the rest of her life. Sure it was unusual.  Sure it was a challenge both in arriving at a diagnosis and a surgical plan. We must put in mind however, that we just controlled the future of this patient. She is at her puberty and yet her capacity to reproduce was taken away from her. She will never be able to conceive and bear children.  She might not even find a man who will marry her because of her inability to have children.  But then, not doing the procedure would endanger the patient’s life as well.  It was a tough call indeed which may even have legal implications.  The decision was arrived at soundly and logically.  The disability that the procedure caused was outweighed by the urgency of the complications of not proceeding with the hysterectomy.  I do hope that in time, she will be able to understand and eventually accept it, as hard as it is.