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Reduced fetal movements at 27 weeks: An alarm for maternal–fetal listeriosis? S. Matsubara, Y. Takahashi, T. Yoshiba, R. Usui & Y. Koike To cite this article: S. Matsubara, Y. Takahashi, T. Yoshiba, R. Usui & Y. Koike (2014) Reduced fetal movements at 27 weeks: An alarm for maternal–fetal listeriosis?, Journal of Obstetrics and Gynaecology, 34:8, 740-740 To link to this article:

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Obstetrics Case Reports

testosterone peripherally. Aromatase deficiency causes increased excretion of 16α-hydroxyandrostenedione metabolites (16α-hydroxyandrosterone, 16-OHAn, and 16α-hydroxyetiocholanolone, 16-OHEt) in the maternal urine (Marshall et al. 2003; Jones et al. 2007). Congenital hypopituitarism is characterised by deficiency of two or more pituitary hormones. It has a wide range of causes, including mutations of genes encoding pituitary transcription factors. Maternal steroid urine profile in cases of congenital hypopituitarism is coincident with adrenal insufficiency; molecular genetic testing is necessary for further differential diagnosis (Marshall et al. 2003). Maternal urine steroid profile analysis is useful for Smith–Lemli–Opitz syndrome (SLOS) suggestion. SLOS is a congenital multiple anomaly syndrome, characterised by growth retardation, feminisation of the fetus, microcephaly, intellectual disability, cleft palate, cardiac defects and syndactyly/polydactyly. It is caused by a deficiency of the 7-dehydrocholesterol reductase, which catalyses the final step of the cholesterol biosynthesis. Lack of cholesterol results in low uE3 levels. SLOS can be suspected because of distinctive fetal anomalies in addition to specific maternal urine steroid profile (8-dehydroestriol excretion) (Marshall et al. 2003; Schoen et al. 2003). Maternal urine steroid profile analysis is very useful in the differential diagnosis of these rare causes of low maternal uE3, because it leads to the clinical diagnosis suggestion and indicates the direction of molecular genetic investigations. In cases of fetal feminisation or virilisation, karyotyping of the fetus is another useful procedure. Therefore, amniocentesis is necessary in the differential diagnosis of rare causes of low uE3, while XLI diagnosis confirmation can be performed postnatally.

Conclusion The most common cause of low uE3 in viable fetuses is steroid sulfatase deficiency, a clinical manifestation of which is XLI. Nevertheless, it is important to keep in mind other possible causes of low uE3. Each case of low maternal oestriol requires careful examination of the patient and evaluation of individual needs for invasive prenatal diagnosis. Maternal urine steroid profile analysis is recommended in every differential diagnosis due to its informativity and non-invasiveness. However, confirmation of the diagnosis is possible only after molecular genetic investigations. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

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Marshall I, Ugrasbul F, Manginello F et al. 2003. Congenital hypopituitarism as a cause of undetectable estriol levels in maternal triple-marker screen. Journal of Clinical Endocrinology and Metabolism 88:4144–4148. Mevorah B, Frenk E, Müller CR et al. 1981. X-linked recessive ichthyosis in three sisters: evidence for homozygosity. British Journal of Dermatology 105: 711–717. Minsart AF, Onderbergen AV, Jacques F et al. 2008. Indication of prenatal diagnosis in pregnancies complicated by undetectable second-trimester maternal serum estriol levels. Journal of Prenatal Medicine 3:27–30. Nussbaumer P, Billich A. 2004. Steroid sulfatase inhibitors. Medicinal Research Reviews 24:529–576. Robledo R, Melis P, Schillinger E et al. 1995. X-linked ichthyosis without STS deficiency: clinical, genetical, and molecular studies. American Journal of Medical Genetics 59:143–148. Sarikaya E, Bozdag S, Deveer R et al. 2012. Possible association of medications during pregnancy with low estriol level of the triple antenatal screening test. Journal of Maternal-Fetal and Neonatal Medicine 25:930–933. Schoen E, Norem C, O’Keefe J et al. 2003. Maternal serum unconjugated estriol as a predictor for Smith-Lemli-Opitz syndrome and other fetal conditions. Obstetrics and Gynecology 102:167–172. Shwayder T. 2004. Disorders of keratinization: diagnosis and management. American Journal of Clinical Dermatology 5:17–29. Siegel DH, Sybert VP. 2006. Mosaicism in genetic skin disorders. Pediatric Dermatology 23:87–92. Thauvin-Robinet C, Lambert D, Vaillant G et al. 2005. X-linked recessive ichthyosis in a girl: strategy for identifying the causal mechanism. British Journal of Dermatology 152:191–193. Tiepolo L, Zuffardi O, Fraccaro M et al. 1980. Assignment by deletion mapping of the steroid sulfatase X-linked ichthyosis locus to Xp223. Human Genetics 54:205–206. Wells RS, Kerr CB. 1965. Genetic classification of ichthyosis. Archives of Dermatology 92:1–6. Zalel Y, Kedar I, Tepper R et al. 1996. Differential diagnosis and management of very low second trimester maternal serum unconjugated estriol levels, with special emphasis on the diagnosis of X-linked ichthyosis. Obstetrical and Gynecological Survey 51:200–203.

Reduced fetal movements at 27 weeks: An alarm for maternal–fetal listeriosis? S. Matsubara1, Y. Takahashi1, T. Yoshiba1, R. Usui1 & Y. Koike2 Department of 1Obstetrics and Gynecology, and 2Pediatrics, Jichi Medical University, Tochigi, Japan DOI: 10.3109/01443615.2014.925859 Correspondence: S. Matsubara, Department of Obstetrics and Gynecology, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi, 329-0498 Japan. E-mail: [email protected]

Introduction Reduced fetal movements sometimes indicate fetal jeopardy (RCOG 2011). Cardiotocography at the time of reduced fetal movements was shown to be a reliable predictor of fetal outcome (Daly et al. 2011). Reduced fetal movements at 27 weeks, although the cardiotocography showed fetal wellbeing, heralded the clinical manifestation of listeriosis.

Case report

At 27 ⫹ 4 weeks’ gestation, a 42-year-old 1-parous woman complained of reduced fetal movements. Ultrasound and cardiotocography revealed an appropriate-for-date fetus with normal movements, reassuring pattern with baseline rate 120 bpm and normal velocimetry of the umbilical artery and middle cerebral artery. After 8 h, she re-presented due to abdominal pain without vomiting. Her body temperature was 40°; blood pressure 117/55 mmHg; pulse 117 bpm; white blood cell count 19,100/μl; haemoglobin 10.8 g/dl; and CRP 3.4 mg/dl, with fetal tachycardia (180 bpm), without deceleration. Due to breech presentation with possible intrauterine infection, we performed a caesarean section, with flomoxef (2 g/day) and panipenem/ betamipron (1 g/day) administered before caesarean section, which yielded a female 1,110 g infant: Apgar score 1/7 (1/5 min); cord blood

Obstetrics Case Reports 741 pH 7.26 and base excess –8.5 mEq/l; WBC 13,800; haemoglobin 14.8 g/dl; platelets 14.2 ⫻ 104/μl; CRP 2.98 mg/dl. Small abscesses were observed in the placenta. Administration of flomoxef (2 g/day for the mother) and panipenem/betamipron (60 mg/kg per day for the neonate) were continued. Two days later, Listeria monocytogenes was cultured from the maternal blood, chorionic membrane, the placenta and fetal gastric juice, leading to the diagnosis of maternal– fetal listeriosis. Flomoxef or panipenem/betamipron was changed to aminobenzyl penicillin, 12 g/day for the mother and 200 mg/kg per day for the infant. The infant was under a respirator for 26 days due to respiratory distress syndrome and is now under the care of pediatricians, without major sequelae. The mother recovered well. The transmission route of the Listeria was not identified.

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Discussion The clinical course of this patient suggested that reduced fetal movements can occur before the clinical manifestations of maternal– fetal listeriosis. According to the Royal College of Obstetricians and Gynaecologists guidelines (RCOG 2011), a woman can be reassured in the absence of objective reduced fetal movements and risk factors for stillbirth. Daly et al. (2011) showed that cardiotocography well predicted fetal outcome at reduced fetal movements after 28 weeks: resuscitation at the time of delivery was required in 2.2% (11/497) and 22.2% (6/27) in reassuring group and non-reassuring group, respectively. In this patient, objective reduced fetal movements and risk factors for stillbirth were absent and cardiotocography showed a reassuring pattern at the time of reduced fetal movements. However, symptoms of listeriosis appeared 8 h later. Listeriosis is mainly a food-borne infection with a relatively long incubation period (RCOG 2011). Considering that many small abscesses were observed in the placenta at the time of caesarean section and Listeria were cultured from various specimens from the mother and fetus, it is natural to assume that the infant may have already been infected by listeriosis at the time of reduced fetal movements. Fetal movement requires intact neurological activity (RCOG 2011), which fetal infection may have suppressed. However, routine check-ups at the first visit showed fetal wellbeing, therefore, the ‘threshold’ for the occurrence of discernable non-reassuring signs and reduced fetal movements may differ. Whether reduced fetal movements heralding the clinical manifestation of fetal infection is specific to listeriosis is unclear. We wish to add another point regarding reduced fetal movements before 28 weeks’ gestation. Reduced fetal movements may indicate imminent fetal jeopardy even before 28 weeks. Many fetal movement-counting charts start at 28 weeks (Holm Tveit et al. 2009; Winje et al. 2011). A previous study by Daly et al. (2011) also confined study subjects to women after 28 weeks. RCOG (2011) guidelines recommend only checking the fetal heart rate and historytaking regarding stillbirth risk to pregnant women with reduced fetal movements at 240/7–280/7 weeks. Previously, we described reduced fetal movements at 26 weeks due to cord hypercoiling; caesarean section saved the infant’s life (Matsubara et al. 2013). We once again experienced reduced fetal movements before 28 weeks, which indicated the imminent fetal jeopardy. Although this single case does not allow any conclusion, we believe that it may be cautious that a pregnant woman with reduced fetal movements but without discernible ominous signs may be advised to visit an obstetrician when she has fever even before 28 weeks. Reduced fetal movements can be initial sign of maternal– fetal listeriosis. Fetal movement count may better start before 28 weeks; however, an appropriate week to start is unclear.

Holm Tveit JV, Saastad E, Stray-Pederson B et al. 2009. Reduction of late stillbirth with the introduction of fetal movement information and guidelines – a clinical quality improvement. BMC Pregnancy Childbirth 9:32. Matsubara S, Kuwata T, Fukui S. 2013. Fetal movement count may prevent fetal death as early as 26 weeks. Acta Obstetricia et Gynecologica Scandinavica 92:1426–1426. RCOG. 2011. Green-top Guideline 57: Reduced fetal movements. London: Royal College of Obstetricians and Gynaecologists. Available at: files/rcog-corp/GTG57RFM25022011.pdf (Accessed 28 April 2014). Winje BA, Saastad E, Gunnes N et al. 2011. Analysis of ‘count-to-ten’ fetal movement charts: a prospective cohort study. British Journal of Obstetrics and Gynaecology 118:1229–1238.

Absent ductus venosus in the fetus F. Oztunc1, S. Gokalp1, M. A. Yuksel2, M. Imamoglu2 & R. Madazli2 1Divison of Pediatric Cardiology, Department of Pediatrics and 2Division of Perinatology, Department of Obstetrics and Gynecology,

Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey DOI: 10.3109/01443615.2014.930105 Correspondence: F. Oztunc, Division of Pediatric Cardiology, Department of Pediatrics, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey. E-mail: [email protected]

Introduction The development of the connection between umbilical vein and ductus venosus is of primary importance in the development of the venous drainage system and supply of oxygenated blood to the fetus (Hofstaetter et al. 2000). The ductus venosus (DV) connects the portal and umbilical venous circulation to the inferior vena cava. Oxygenated blood in umbilical vein (UV) enters the DV, bypasses the liver and reaches directly to the left heart through foramen ovale (Fasouliotis et al. 2002). Thus, DV plays a major role in the regulation of unrestricted blood flow of the umbilical vein and protects the fetus from high output heart failure (Berg et al. 2006). Absence of ductus venosus is still a rare congenital anomaly. It is mostly characterised with hydrops fetalis and cardiomegaly and these features are usually detected on fetal ultrasonography. The diagnosis is suspected by demonstration of abnormal course of UV and absence of DV.

Case report 1 A 29-year-old, G2P1 woman was referred to our unit for fetal echocardiography, at the 26th gestational week, with previously diagnosed fetal cardiomegaly. Fetal echocardiography revealed a dilated umbilical vein (11 mm) with an abnormal course draining directly into the right atrium. No DV was detected. The course and sizes of the hepatic and portal veins and inferior vena cava, were normal. All

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

References Daly N, Brennan D, Foley M et al. 2011. Cardiotocography as a predictor of fetal outcome in women presenting with reduced fetal movement. European Journal of Obstetrics, Gynecology, and Reproductive Biology 159:57–61.

Figure 1. Direct connection between the umbilical vein and the right atrium by colour Doppler ultrasonography. UV, umbilical vein; RA, right atrium; TA, transverse aorta; DA, descending aorta.

Reduced fetal movements at 27 weeks: an alarm for maternal-fetal listeriosis?

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