Scoliosis in Herlyn–Werner–Wunderlich Syndrome

Herlyn–Werner–Wunderlich syndrome (HWWS) is a congenital Müllerian duct anomaly characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Little is reported about spinal deformity associated with this syndrome. This study presents a case of scoliosis occurring in the setting of HWWS and explores the possible association between the 2 diseases. A previously unreported scoliosis in HWWS is described. The patient is a 12-year-old Chinese female with scoliosis that underwent a posterior correction at thoracic 5–thoracic 12 (T5–T12) levels, using the Moss-SI (Johnson & Johnson, American) spinal system. At 24-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. Six months after scoliosis correction surgery, the patient went to our clinics for the treatment of HWWS. She was performed a vaginal septum resection and detected with pyocolpos. Her follow-up was symptom free at the fourth postoperative month. The prevalence of scoliosis among patients with HWWS was 8.57% that is much higher than the incidence of congential scoliosis among general population (1/1000). To the best of our knowledge, this is the first report of HWWS with thoracic scoliosis. During surgery, surgeons and anesthesiologists must pay particular attention to the Müllerian duct anomaly and renal agenesis associated with HWWS. There is a potential association between congenital scoliosis and HWWS. (Medicine 93(28):e185) Abbreviation: HWWS = Herlyn–Werner–Wunderlich syndrome. INTRODUCTION H erlyn–Werner–Wunderlich syndrome (HWWS) is a rare congenital Müllerian duct anomaly characterized by uterShen, MD, and Jinqian Liang, MD incidence of obstructive Müllerian duct anomalies can range from 0.1% to 3.8%. HWWS is usually discovered at puberty with nonspecific symptoms, such as increasing pelvic pain, dysmenorrheal, and palpable mass due to the associated hematocolpos or hematometra, which result from retained, longstanding menstrual flow in the obstructed vagina. The exact cause, pathogenesis, and embryologic origin of HWWS remain a subject of discussion. There are limited reports regarding the diagnosis and management of HWWS with its possible resultant congenial scoliosis. We here present a case of HWWS in a 12-year-old girl with unusual presentation— congenital scoliosis. CONSENT Written informed consent was obtained from the patient’s parents on behalf of the child for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal. CASE REPORT We present the case of a 12-year-old asylum seeker who was admitted for an elective correction of her progressive scoliotic deformity. Her plain radiographs of the spine showed that the thoracic scoliosis was progressive with the Cobb angles increasing from 368 to 568 (Figure 1), suggesting the need for surgical correction. Her past medical history was only remarkable in that she had complained about a lot of menses amounts after 10 months of menarche. The patient denied any recent abdominal trauma, abdominopelvic pain, abnormal vaginal bleeding, nausea, vomiting, or diarrhea. She had undergone an ultrasound in a small medical clinic that revealed oblique vaginal septum and a didelphic uterus. Ultrasounds in our hospital revealed a didelphic uterus and double cervix and absent right kidney. Atrial septal defect was also present while the cardiac function was normal. Magnetic resonance imaging (MRI) was further performed to evaluate other possible genitourinary anomaly. MRI imaging showed a uterine–vaginal anomaly consisting of didelphys uterus, double cervix, and double vagina, one of which was obstructed, and consequently, there was accumulation of fluid exhibiting a signal intensity similar to methemoglobin in the right uterus (slightly dilated, 14 mm diameter of the lumen) and in the right obstructed vagina. The diagnosis of HWWS was confirmed based on the points of didelphic uterus, unilateral renal absence, and hematometra. In February 2012, a posterior correction and fusion at thoracic 5–thoracic 12 (T5–T12) levels was performed, using the Moss-SI spinal system. The total operation time was 3 hours and 15 minutes. Total amount of blood loss was 400 mL. During al of this patient was normal on intraoonitoring. Postoperatively, there was nction. Postoperative plain x-ray film www.md-journal.com | 1