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of the thoracic spine revealed a 1.1 cm intradural extramedullary mass at the level of the T2 vertebral body (Figure 1a). Spinal neurosurgery was planned due to exacerbation of her chronic back pain and progressive weakness of the lower limbs at 28 weeks’ gestation. Emergent spinal decompression surgery was performed with gross total excision of the tumour. Doppler flow of the umbilical artery was used preoperatively and postoperatively to monitor fetal wellbeing. The histological examination revealed HPC, World Health Organization (WHO) grade 2 (Figure 1b). Complete recovery was seen within 1 week of surgery. Follow-up MRI demonstrated complete removal of the tumour. We recommended adjuvant external radiotherapy to the patient in the 3rd trimester of pregnancy due to HPC’s high risk of recurrence. However, the patient declined radiotherapy. Routine weekly obstetric assessments were performed following surgery. At the 37th gestational week, a 2,850 g, Apgar score 7–8, healthy infant was delivered by caesarean section, without need of admission to the neonatal intensive care unit. Adjuvant radiotherapy was administered to the patient in the postpartum period.

Discussion HPC is an extremely rare tumour located in the CNS (Rutkowski et al. 2012). HPC typically appears as a mixture of spindle-shaped tumour cells with oval nuclei and scanty cytoplasm, mixed with a network of thin-walled blood vessels or sinusoid-like spaces, described as ‘staghorn’ bodies (Enzinger and Smith 1976). The tumour contains well-developed reticulin fibres and diffuse CD34 immunopositivity. HPC is considered a WHO grade 2 neoplasm, with anaplastic variants classified as grade 3 (Louis et al. 2007). An extracranial location is more frequent in HPC than in meningiomas (Bonde and Goel 2009). Meningiomas demonstrate a female predominance and a high incidence of symptomatic progression during pregnancy (Kanaan et al. 2003). Hormonal status may cause accelerated tumour growth, with increased vascularity (Stevenson and Thompson 2005). Symptoms are associated with the location of the tumour in the CNS. In our case, the patient’s major symptom was progressive lower limb weakness at 28 weeks’ gestation. MRI is useful in the diagnosis of spinal HPC during pregnancy. No harmful effects to the fetus have been documented (Simon 1988). Iodine-based compounds used for computed tomography (CT) may rarely induce fetal hypothyroidism. Gadolinium-based contrast agents used for MRI have not been shown to cause fetal abnormalities (Webb et al. 2005). The patient should be informed about the potential risks and benefits of neuroimaging during pregnancy. In our case, MRI with gadolinium was chosen after counselling of the patient. There is much debate in the literature about the necessity for intraoperative fetal monitoring. Rosen (1999) advocated continuous monitoring to identify possible impaired uteroplacental blood flow and fetal oxygenation. However, Horrigan et al. (1999) concluded that it was not required in the absence of maternal hypoxia or hypotension. In our case, we used umbilical arterial Doppler assessment to monitor fetal wellbeing pre- and postoperatively. The diagnosis of malign CNS tumour in the late gestational period may necessitate preterm delivery. Radiotherapy is not associated with an increased risk of birth defects or fetal loss and can be administered in the second half of gestation. However, increased risk of childhood leukaemia and potential neurocognitive deficit may occur after radiotherapy during pregnancy (Pimperl 2005). In our case, considering the high risk of recurrence of HPC, adjuvant external radiotherapy was offered in the 3rd trimester of pregnancy but refused by the patient. Caesarean section was performed in the 37th week of pregnancy. Adjuvant radiotherapy was planned in the postpartum period. This is the first case reported in the literature of HPC located in the spinal cord, diagnosed during pregnancy. Due to the aggressive behaviour of HPC, it should be differentiated from other CNS tumours. Complete tumour resection during the initial surgery and adjuvant radiotherapy play the most important role

for survival. A multidisciplinary approach is suggested for optimal obstetric and neurological care during pregnancy at a tertiary care centre. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

References Annunziato M, Alessio A, Stefano M et al. 2005. Hemangiopericytoma in pregnancy: a case report. Journal of Neuro-Oncology 73:277–278. Bassiouni H, Asgari S, Hübschen U et al. 2007. Intracranial hemangiopericytoma: treatment outcomes in a consecutive series. Zentralblatt fur Neurochirurgie 68:111–118. Bonde VR, Goel A. 2009. Two patients with intracavernous hemangiopericytoma. Journal of Clinical Neuroscience 16:330–333. Hernández-Durán S, Sánchez-Jiménez E, Pérez-Berríos J. 2014. Hemangiopericytoma of the foramen magnum in a pregnant patient: A case report and literature review. Surgical Neurology International 55:13. Enzinger FM, Smith BH. 1976. Hemangiopericytoma: an analysis of 106 cases. Human Pathology 7:61–82. Horrigan TJ, Villarreal R, Weinstein L. 1999. Are obstetrical personnel required for intraoperative fetal monitoring during nonobstetric surgery? Journal of Perinatology 19:124–126. Kanaan I, Jallu A, Kanaan H. 2003. Management strategy for meningioma in pregnancy: a clinical study. Skull Base 13:197–203. Louis DN, Ohgaki H, Wiestler OD et al. 2007. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathologica 114: 97–109. Petignat P, Vajda D, Letovanec N et al. 1998. Pelvic hemangiopericytoma in pregnancy: Report of a case. Journal de Gynecologie, Obstetrique et Biologie de la Reproduction 27:721–724. Pimperl LC. 2005. Radiation as a nervous system toxin. Neurologic Clinics 23: 571–597. Rosen MA. 1999. Management of anesthesia for the pregnant surgical patient. Anesthesiology 91:1159–1163. Rutkowski MJ, Jian BJ, Bloch O et al. 2012. Intracranial hemangiopericytoma: clinical experience and treatment considerations in a modern series of 40 adult patients. Cancer 118:1628–1636. Simon RH. 1988. Brain tumors in pregnancy. Seminars in Neurology 8:214–221. Stevenson CB, Thompson RC. 2005. The clinical management of intracranial neoplasms in pregnancy. Clinical Obstetrics and Gynecology 48:24–37. Ustaalioglu BB, Gumus M, Unal A et al. 2010. Malignancies diagnosed during pregnancy and treated with chemotherapy or other modalities (review of 27 cases): multicenter experiences. International Journal of Gynecological Cancer 20:698–703. Webb JA, Thomsen HS, Morcos SK. 2005. The use of iodinated and gadolinium contrast media during pregnancy and lactation. European Radiology 15:1234–1240.

Prenatal diagnosis of congenital imperforate hymen with hydrocolpos E. Nakajima1,2, T. Ishigouoka2, T. Yoshida2, T. Sato3, T. Miyamoto1, M. Shirai3 & K. Sengoku1 Departments of 1Obstetrics and Gynecology, Asahikawa Medical University, 2Obstetrics and Gynecology and 3Pediatrics, Asahikawa Kohsei Hospital, Asahikawa, Japan DOI: 10.3109/01443615.2014.951608 Correspondence: T. Miyamoto, Department of Obstetrics and Gynecology, Asahikawa Medical University, Midorigaokahigashi 2-1-1-1, Asahikawa, Hokkaido, 0788510, Japan. E-mail: [email protected]

Case report A 36-year-old woman, gravida 6, para 4, was referred to our hospital at 28 weeks’ gestation to evaluate fetal growth. The personal and family histories were unremarkable. Ultrasonography revealed a female fetus with a non-vascular pear-shaped pelvic cyst, which was 46 ⫻ 21 mm in diameter (Figure 1a). A detailed ultrasound scan was then performed and the results were as follows: the coating of the cyst was

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thin and the content of the cyst was liquid. There was no solid component inside the cyst and the front of the cyst was in contact with the back of the bladder. The cyst had no continuity with other organs (Figure 1b). A sonographic examination at 34 weeks’ gestation showed that the cyst had increased in size to 55 ⫻ 35 ⫻ 35 mm and was in contact with the back of the uterus (Figure 1c). Fetal magnetic resonance imaging (MRI) was carried out on the same day and showed a pelvic lesion with homogeneously low (coronal T1-weighted image) and high (coronal T2-weighted image) signal intensity between the bladder and rectum (Figure 1d). The cyst did not have a connection with the spinal cord, bladder or rectum, and the uterus was close to the top of the cyst. The cervix was in contact with the cystic lumen. These findings strongly suggested congenital imperforate hymen with hydrocolpos. The cyst became larger and its size was 81 ⫻ 43 ⫻ 43 mm at 37 weeks. However, no major anomaly in the other organs could be detected by ultrasonography. At 38 ⫹ 5 weeks’ gestation, a 2,880 g female neonate was delivered vaginally with Apgar scores of 8 and 9 after 1 and 5 min, respectively. At birth, the neonate’s abdomen was soft, but was distended above the umbilicus. The perineum appeared normal, with an intact position and a perforated anus. However, the hymen was found to be imperforate and was bulging forwards (Figure 1e,f). No other abnormalities were found by a physical examination. Routine laboratory data were normal and surgery was performed by making an incision of the hymen followed by immediate leakage of white mucinous fluid. The patient was discharged with a good postoperative course thereafter.

Discussion Congenital imperforate hymen is an external urogenital anomaly that usually presents in infancy and early childhood. This condition is mostly a sporadic and isolated malformation, with a reported incidence of 0.014–1% at term (Mor et al. 1986; McCann et al. 1990). The association of an imperforate hymen with other anomalies is well known, including bifid clitoris; polydactyly; duplication of the ureter; hypoplastic kidney with ectopic ureter; multicystic dysplastic kidney; vascular anomalies; imperforate anus; a ureteral membrane; and a low anorectal anomaly (Winderl and Silverman 1995). Prenatal diagnosis of imperforate hymen with hydrocolpos has been reported as early as 22 weeks’ gestation (Adaletli et al. 2007), although most cases are described during late gestation or after birth (Odibo et al. 1997; Tseng et al. 2008). This accounts for 15% of abdominal masses in female infants (Reed and Griscom 1973). The differential diagnosis of such a pelvic mass should include a distended urinary bladder, ovarian neoplasm, reduplication of the sigmoid and sacrococcygeal teratoma (Westerhout et al. 1964). Ultrasonography is useful for evaluating fetal anatomy and it is mostly used for the first diagnosis of abnormalities. Additionally, MRI is also a critical method for identifying some of the equivocal prenatal cases because of recent development in MRI technology. In the present case, the associated hydrocolpos manifested as a distended vagina and appeared as a cystic mass with diffuse low-level echoes. A distal vaginal obstruction was characterised by ultrasonographic findings of a blind pouch covered by a thin membrane at the distal end of the vagina. Therefore, congenital imperforate hymen with hydrocolpos was suspected by ultrasonography examination. Fetal MRI was carried out to confirm our

Figure 1. Transabdominal ultrasonography examination at 28 weeks and 6 days’ gestation in axial (a) and sagittal planes (b). The white arrows indicate hydrocolpos. (c) Transabdominal ultrasonography examination at 34 weeks’ gestation in the sagittal plane. The cranium can be seen on the left side and a foot can be seen on the right side. The white arrow shows that the cervix of the uterus is in contact with the hydrocolpos. (d) Fetal MRI at 34 weeks’ gestation. The black arrows show a characteristic boot-shaped mass in the sagittal plane. The white arrows show that the uterus is in the anterior position and the cervix contacts the hydrocolpos. (e,f) Photographs of the external genitals after birth. (e) The hymen membrane was bulging while the newborn was crying. (f) The mass is reduced without abdominal pressure.

Obstetric Case Reports 313 prenatal diagnosis. The MRI showed the exact location and extension of the cystic mass from the mid-abdomen to the perineum, in addition to the findings observed on sonography. The bladder was also clearly shown on the MRI. The cystic mass was diagnosed as hydrocolpos because it extended to the perineum and because a bladder abnormality could be excluded. In conclusion, we suggest that fetal MRI should be used when imperforate hymen with hydrocolpos is suspected by ultrasonography. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

References

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Adaletli I, Ozer H, Kurugoglu S et al. 2007. Congenital imperforate hymen with hydrocolpos diagnosed using prenatal MRI. American Journal of Roentgenology 189:W23–25.

McCann J, Wells R, Voris J. 1990. General findings in prepubertal girls selected for non-abuse: a descriptive study. Pediatrics 86:428–439.

Mor N, Merlob P, Riensner SH. 1986. Types of hymen in the newborn infant. European Journal of Obstetrics and Gynecology and Reproductive Biology 22:225–228. Odibo AO, Turner GW, Borgida AF et al. 1997. Late prenatal ultrasound features of hydrocolpos secondary to cloacal anomaly: case reports and review of the literature. Ultrasound in Obstetrics and Gynecology 9:419–421. Reed MH, Griscom NT. 1973. Hydrocolpos in infancy. American Journal of Roentgenology 118:1–13. Tseng JJ, Ho JYP, Chen WH et al. 2008. Prenatal diagnosis of isolated fetal hydrocolpos secondary to congenital imperforate hymen. Journal of the Chinese Medical Association 71:325–328. Westerhout FC, Hodgman JE, Anderson GV et al. 1964. Congenital hydrocolpos. American Journal of Obstetrics and Gynecology 89: 957–961. Winderl LM, Silverman RK. 1995. Prenatal diagnosis of congenital imperforate hymen. Obstetrics and Gynecology 85:857–860.

Prenatal diagnosis of congenital imperforate hymen with hydrocolpos.

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