556 Correspondence

were listed in the text in the context of overlooked

brain abnormalities on fetal magnetic resonance imaging
(MRI): ‘Subependymal heterotopia is among the most
frequently overlooked brain abnormalities on fetal MRI,
as compared with postnatal MRI2 and postmortem neu-
ropathological findings, e.g. in Chiari II malformation3 .’.
We agree fully that heterotopia may be diagnosed
prenatally using ultrasound. As the authors pointed out,
the diagnosis can be challenging, especially when only
few heterotopia are present. In such cases, fetal MRI
might provide additional information, even though we
are well aware that the diagnosis of subtle heterotopia is
equally challenging on fetal MRI.
In an earlier version of our Letter, this point was elab-
orated on in more detail, but it was eventually shortened
to meet the word limit set by the Journal. The original
text read: ‘In fact, subependymal heterotopia is among the
most frequently overlooked brain abnormalities on fetal
MRI, as compared with postnatal MRI2,4 . The discrep-
ancy is also evident when comparing MRI to postmortem
neuropathology findings, e.g. in Chiari II malformation,
where MRI detects subependymal heterotopia in about
19% of patients5 , while neuropathology studies report
subependymal heterotopia in about 44% of patients3 .
Especially in very early gestation, when an irregular mar-
gin of the lateral ventricle might be the only hint that
heterotopia is about to form, 3-Tesla fetal MRI might help
to improve sensitivity for detecting heterotopia. Detection
of heterotopia at earlier gestational ages might provide
not only earlier diagnosis but also additional diagnostic
information, as for example subependymal nodules of
tuberous sclerosis can be detected by MRI only at around
30 weeks’ gestation6 , while subependymal heterotopia can
be detected as early as 23 weeks on fetal MRI7 ’.
Dr Garel et al. also suggested that the posterior fossa
findings reported in our Letter were more in keeping with
retrocerebellar arachnoid cyst (RCAC) rather than with
megacisterna magna. We thank the authors for pointing
out this possibility. Both megacisterna magna and RCAC
have been described in association with BPNH8,9 . Indeed,
it may be possible that the two fetuses reported in our
Letter display RCAC. However, there was no mass effect,
so the pathological relevance would be limited. Generally,
the differential diagnosis of megacisterna magna and
RCAC on fetal MRI can be challenging because thin
septa can be hard to depict. In such cases, ultrasound can
provide more precise information as even thin cystic walls
can be displayed reliably.

S. Stoecklein1,2*, C. Haberler3 , G. Gruber4 ,

Reply M. Diogo2 , B. Ulm5 , F. A. Laccone6 and D. Prayer2
1
Department of Radiology,
We thank Dr Garel and colleagues for their insightful Ludwig-Maximilians University Munich,
comments on our Letter to the Editor on bilateral Munich, Germany;
2
periventricular nodular heterotopia (BPNH)1 . Department of Biomedical Imaging and Image-guided
Regarding their first remark, we would like to clarify Therapy, Medical University Vienna,
that there is no mention in our article of the frequency Vienna, Austria;
3
of overlooked heterotopia on prenatal ultrasound; in Institute of Neurology,
fact, the two references2,3 referred to by the authors Medical University of Vienna, Vienna, Austria;

Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd. Ultrasound Obstet Gynecol 2019; 53: 554–557.
Correspondence 557

4 3. Gilbert JN, Jones KL, Rorke LB, Chernoff GF, James HE. Central nervous system
Center of Anatomy and Cell Biology, anomalies associated with meningomyelocele, hydrocephalus, and the Arnold-Chiari
Medical University of Vienna, Vienna, Austria; malformation: reappraisal of theories regarding the pathogenesis of posterior neural
5 tube closure defects. Neurosurgery 1986; 18: 559–564.
Obstetrics and Feto-Maternal Medicine, 4. Limperopoulos C, Robertson RL Jr, Khwaja OS, Robson CD, Estroff JA, Barnewolt C,
Medical University of Vienna, Vienna, Austria; Levine D, Morash D, Nemes L, Zaccagnini L, du Plessis AJ. How accurately does
6 current fetal imaging identify posterior fossa anomalies? AJR Am J Roentgenol 2008;
Institute of Medical Genetics, 190: 1637–1643.
Medical University of Vienna, Vienna, Austria 5. Miller E, Widjaja E, Blaser S, Dennis M, Raybaud C. The old and the new:
supratentorial MR findings in Chiari II malformation. Childs Nerv Syst 2008; 24:
*Correspondence. 563–575.
(e-mail: sophia.stoecklein@med.uni-muenchen.de) 6. Mühler MR, Rake A, Schwabe M, Schmidt S, Kivelitz D, Chaoui R, Hamm B. Value of
fetal cerebral MRI in sonographically proven cardiac rhabdomyoma. Pediatr Radiol
DOI: 10.1002/uog.20241 2007; 37: 467–474.
7. Mitchell LA, Simon EM, Filly RA, Barkovich AJ. Antenatal diagnosis of subependymal
heterotopia. AJNR Am J Neuroradiol 2000; 21: 296–300.
8. Teixeira SR, Blondiaux E, Cassart M, Couture A, Moutard ML, Whalen S, Gelot A,
References Ducou le Pointe H, Garel C; GRRIF (Groupe de Recherche Radiopédiatrique en
Imagerie Fœtale); SFFIP (Société Francophone d’Imagerie Pédiatrique et Prénatale).
1. Stoecklein S, Haberler C, Gruber G, Diogo M, Ulm B, Laccone FA, Prayer D. Association of periventricular nodular heterotopia with posterior fossa cyst: a prenatal
Bilateral periventricular nodular heterotopia detected on fetal and maternal MRI case series. Prenat Diagn 2015; 35: 337–341.
attributable to novel filamin A gene mutation. Ultrasound Obstet Gynecol 2018; 52: 9. Lange M, Kasper B, Bohring A, Rutsch F, Kluger G, Hoffjan S, Spranger S, Behnecke A,
678–680. Ferbert A, Hahn A, Oehl-Jaschkowitz B, Graul-Neumann L, Diepold K, Schreyer I,
2. Dhouib A, Blondiaux E, Moutard M, Billette de Villemeur T, Chalard F, Jouannic J, Bernhard MK, Mueller F, Siebers-Renelt U, Beleza-Meireles A, Uyanik G, Janssens S,
Ducou le Pointe H, Garel C. Correlations between pre- and postnatal cerebral magnetic Boltshauser E, Winkler J, Schuierer G, Hehr U. 47 patients with FLNA associated
resonance imaging. Ultrasound Obstet Gynecol 2011; 38: 170–178. periventricular nodular heterotopia. Orphanet J Rare Dis 2015; 10: 134.

Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd. Ultrasound Obstet Gynecol 2019; 53: 554–557.