Congenital anomalies in the teratological collection of museum Vrolik in Amsterdam, the Netherlands. v: conjoined and acardiac twins

Abstract

The Museum Vrolik collection of the Department of Anatomy and Embryology of the Academic Medical Center, University of Amsterdam, founded by Gerardus Vrolik (1775-1859) and his son Willem Vrolik (1801-1863), consists of more than 5,000 specimens of human and animal anatomy, embryology, pathology, and congenital anomalies. Recently, the collection of congenital anomalies, comprising 360 specimens, was recatalogued and redescribed according to contemporary perspectives. The original descriptions, as far as preserved, were compared with the clinical, radiographic, and magnetic resonance imaging findings. We diagnosed 30 symmetrical conjoined twins (CTs), 11 parasitic CTs, and 16 acardiac twins. Within the group of symmetrical CTs, the following concomitant external anomalies were found in 15 specimens: neural tube defects, holoprosencephaly, cleft lip/palate, umbilical hernia, omphalocele, cloacal exstrophy, peromelia, polydactyly, and facial abnormalities suggestive of a chromosomal abnormality. We discuss the results in the light of historical and contemporary explanations regarding conjoined twinning, including the opinions of Gerardus and Willem Vrolik and of Louis Bolk, one of their successors.

Bladder exstrophy‐epispadias complex

“…The first medical description is probably Aldovrandus' Historia Monstrum , published in 1646, which references scientific details documented by Scheuke von Grafenberg in 1595 (Hall et al, 1953). A dried pelvis specimen of a 6‐year‐old boy showing marked diastasis of the pubic rami was recorded by Gerardus and Willem Vrolik in their “Tabulae” of the Museum Vrolik (Oostra et al, 1998). Vrolik further portrayed an ectopic bladder that was visible through an infraumbilical defect of the abdominal wall, a phenotype characteristic of classic bladder exstrophy.…”

“…Examination of the collection confirmed the observations of Gerardus and Willem Vrolik and revealed cloacal exstrophy to be diagnosed in 16 specimens, 14 of which had an additional omphalocele, whereas one did not. In one case (M677) the condition was present in an infant with otocephaly‐holoprosencephaly (Oostra et al, 1998).…”

Are Encephaloceles Neural Tube Defects?

“…Two embryologic models of neural tube development exist: one involving a bidirectional "zipper"-like closure and the other involving multiple closure sites. [6][7][8] Although the multisite closure model may help explain the variation in the anatomic location of the defect in the skull found in patients with encephalocele, this model may not account for other genetic and embryologic discrepancies between SB and anencephaly as compared with encephalocele. Genetic studies examining probands with nonsyndromal NTDs found that sibling concordance and prevalence of unrelated congenital anomalies differed between SB and anencephaly as compared with encephalocele.…”

Absence of the spleen(s) in conjoined twins: a diagnostic clue of laterality defects? Radiological study of historical specimens

“…We recently re-investigated anatomical specimens of conjoined twins in the Museum Vrolik collection in Amsterdam, The Netherlands, with CT and MRI. The external presentations of these specimens have been described previously [3]. We describe presumed laterality defects on imaging in a specific type of conjoined twinning in which these defects are considered uncommon.…”

“…These specimens are part of the Museum Vrolik collection of the Department of Anatomy and Embryology of the Academic Medical Centre, University of Amsterdam, The Netherlands. The collection, which was founded by Gerardus Vrolik (1775–1859) and his son Willem Vrolik (1801–1863), consists of more than 5,000 dried or fixed specimens of human and animal anatomy, embryology, pathology and congenital anomalies [3]. All three CTIP specimens had one fairly complete compound face and one hypoplastic face.…”