Ben W. J. M. Verbeeten

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

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.

Cholesterol granuloma of the frontal bone: CT diagnosis.

Cholesterol granuloma of the frontal bone is a distinct clinical entity. This expanding lesion grows slowly within the diploe of the frontal bone until it extends into the orbit and causes globe displacement. We report the clinical and the CT findings in 11 patients with cholesterol granuloma of the orbit. The CT characteristics of cholesterol granuloma of the frontal bone appear to enable a correct preoperative diagnosis.

Intradiploic Epidermoid Cysts of the Bony Orbit

Epidermoid cysts originating within the diploic space of the bony orbit are rare. The authors retrospectively studied four patients with an intradiploic epidermoid cyst of the orbital bones to investigate the clinical and the computed tomographic (CT) findings. The clinical presentation was dependent on the location of the slowly expanding epidermoid cyst. The sphenoid bone was involved in three patients and the frontal bone in one patient. Proptosis caused by intraorbital extension of the mass was the most common presenting sign. The findings on high-resolution CT scans appeared to be specific for intradiploic epidermoid cysts. The typical sclerotic margin, which is diagnostic of intradiploic epidermoid cysts, can be demonstrated by CT scans with a bone window setting.

Congenital anomalies in the teratological collection of Museum Vrolik in Amsterdam, The Netherlands. III : Primary field defects, sequences, and other complex anomalies

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 morphogenetic views. The original descriptions, if preserved, were compared with the clinical, radiographical, and magnetic resonance imaging findings. In 120 specimens the following midline anomalies, primary field defects, and sequences were diagnosed: holoprosencephaly sequence; aprosencephaly/atelencephaly; cleft lip; Pierre Robin sequence; omphalocele; diaphragmatic hernia; ectopic heart; bladder exstrophy; cloacal exstrophy; caudal dysgenesis; prune belly sequence; sirenomelia; fetal akinesia sequence; and disruption sequences. Polytopic field defects and associations were diagnosed in 25 specimens. We discuss the opinions of Gerardus and Willem Vrolik, who tried to find reasonable explanations for the dysmorphogenetic entities they investigated, rather than accepting the traditional superstitions.

A 100‐year‐old anatomical specimen presenting with boomerang‐like skeletal dysplasia: Diagnostic strategies and outcome

The Museum Vrolik collection of human anatomy comprises 360 recently redescribed specimens with congenital anomalies. The specimen described here dated from 1881 and presented with a general embryonic appearance, disproportionate short stature, brachycephaly, widened cranial sutures, hypertelorism, microphthalmia, bilateral cleft lip and palate, micrognathia, short and curved limbs, polysyndactyly, and abnormal female genitalia. Conventional radiography was hampered by decalcification of the skeleton, due to acidification of the preservation fluid. The use of additional imaging techniques, i.e., mammography, computerized tomography with three-dimensional reconstruction, and magnetic resonance imaging eventually led us to conclude that the condition of our specimen was similar to Piepkorn type skeletal dysplasia, boomerang dysplasia, and a condition described by Carpenter and Hunter [1982: J Med Genet 19:311-315], though none of these diagnoses seemed fully applicable.

Dermolipoma: Characteristic CT appearance

Dermolipomas are choristomas usually located at the external canthus. Clinically they can be confused with dermoid cysts. Preoperative diagnosis of a dermolipoma is important because the surgical approach is less aggressive than for dermoid cysts. We describe the clinical aspects of ten patients with a dermolipoma and demonstrate the posterior extension of these lesions by CT.