Caroline Crachami, the Sicilian dwarf (1815-1824): Was she really nine years old at death?

• Published in: American journal of medical genetics 1998-04, Vol.76 (4), p.343-348
• Main Authors: Berkovitz, B.K.B., Grigson, C., Dean, M.C.
• Format: Article
• Language: English
• Subjects: Age Determination by Skeleton; Age Determination by Teeth; Biological and medical sciences; Bird-headed dwarf; Caroline Crachami; Child, Preschool; Dental Enamel; Dentition; dentition; enamel; Diseases of the osteoarticular system; Dwarfism - history; enamel; Female; History of medicine; History, 19th Century; Humans; Italy; Malformations and congenital and or hereditary diseases involving bones. Joint deformations; Medical sciences; Microscopy, Electron; osteodysplastic primordial dwarfism; perikymata; Radiography; Royal College of Surgeons of England; Seckel's syndrome; Skull
• ISSN: 0148-7299; 1096-8628
• DOI: 10.1002/(SICI)1096-8628(19980401)76:4<343::AID-AJMG10>3.0.CO;2-O

Caroline Crachami (C.C.) considered to have had the Seckel syndrome was one of the most extreme cases of dwarfism ever recorded. Reputedly born in Sicily in 1815, she attracted much attention when exhibited in England before her death on 3 June 1824. Although she is said to have been 9 years old at death, published descriptions give her a dental age varying from 2 to 7 years. Examination of her skull in the Royal College of Surgeons of England demonstrated a more or less erupted complete deciduous dentition, with no erupted permanent teeth. Radiographs showed agenesis of several permanent teeth. It was concluded that the dental age of C.C. was 3 years (± 6 months). Perikymata were evident in the surface enamel encircling the crown of the partially exposed maxillary left first permanent molar. Their distribution and spacing were normal, with no evidence of developmental retardation. The distance between the periradicular bands in the root near the cement‐enamel junction was also normal. We conclude that the dental age of C.C. was similar to her chronological age and that at death she was about 3 and not 9 years old. The reason that she was said to be nearly 9 when exhibited in England we believe was related to financial considerations, as people would be generally less impressed with a dwarf only 3 years old. The new age we give C.C. has implications on the diagnosis of her medical condition. Am. J. Med. Genet. 76:343–348, 1998. © 1998 Wiley‐Liss, Inc.