PEMPix is the American Academy of Pediatrics Section on Emergency Medicine’s annual visual diagnosis competition. This year, in addition to the 10 finalists Maneesha Agarwal will be presenting at the National Conference and Exhibition we will be sharing four cases online in advance of the conference. This is the first of the four cases.

This case was submitted by…

A 12-year-old female was brought to your emergency department by her mother for 1 month of diffuse abdominal and back pain. During that initial visit, her physical exam was notable for generalized abdominal tenderness. She had a normal urinalysis and the abdominal x-rays seen here.

She was diagnosed with constipation. The family declined an enema in the ED, and she was discharged home on a miralax cleanout to follow up with her PCP.

However a few weeks later, she returned to the ED with worsening abdominal and back pain. Pain was now worse on the right side of her abdomen, and her back pain associated with anal pressure. The patient completed the miralax cleanout followed by daily miralax, and started having soft, formed stools daily without any pain (Bristol type 4). She also noted cramping with her menses that resolve with warm compresses; her menstrual cycles occur at regular intervals, last 3 days, and there was no heavy bleeding.

Physical exam

The patient had stable vital signs and an exam notable for tenderness to palpation over the right lower quadrant and suprapubic region without any peritoneal signs. She also had a palpable mass in the right lower quadrant.

Her workup included normal abdominal x-rays, CBC, CMP, and UA.

She also had a pelvic ultrasound with relevant image seen here:

Based on those findings, she then got a CT with relevant images seen here:

A. Ovarian cyst
B. Imperforate hymen
C. Cervical atresia
D. OHVIRA syndrome
E. Bladder neoplasm

D. OHVIRA Syndrome

The patient’s ultrasound revealed an obstructed hemivagina. Her CT was notable for uterine didelphys with obstructed right hemivagina with right hematosalpinx and ipsilateral right renal agenesis. The patient was diagnosed with OHVIRA syndrome: Obstructed Hemivagina with Ipsilateral Renal Anomaly (aka Heryln-Wener-Wunderlch syndrome).

This syndrome can occur in the setting of uterine didelphys or a septate uterus with associated renal abnormality, most commonly associated with renal agenesis. It develops in utero and highlights the close relationship between the development of urinary and reproductive systems. Female patients with renal malformations may benefit from evaluation later in life for reproductive structural anomalies and vice versa.

Patients typically present with nonspecific abdominal pain or dysmenorrhea from progressive distention of obstructed hemivagina after menarche. Diagnosis is typically delayed due to normal regular menstruations from incomplete vaginal outlet obstruction and slow progression of hematocolpos.

Based on the findings, the patient underwent a partial resection of the right hemivagina septum with drainage of the associated hematocolpos. Her imaging also revealed an incidental ovarian teratoma that was also resected.

References

Bajaj SK, Misra R, Thukral BB, Gupta R. OHVIRA: Uterus didelphys, blind hemivagina and ipsilateral renal agenesis: Advantage MRI. J Hum Reprod Sci. 2012 Jan;5(1):67-70. doi: 10.4103/0974-1208.97811. PMID: 22870020; PMCID: PMC3409925. Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3409925/