Cervical Spine Anomalies: Children in One of the Oldest Churches in Poland

Abstract

This paper presents pathological changes indicative of type II Klippel-Feil syndrome identified in the skeleton of a child uncovered in one of the oldest Christian churches in Poland (first half of the 11th century) with the archaeological layer containing the burial dated to the 13th-15th centuries. The age at death of the child was estimated at 9 to 10 years. The third and fourth cervical vertebrae of this individual were fused asymmetrically, leading to torticollis. The apex of the dens axis (odontoid process) was V-shaped, which is consistent with clinical descriptions of ossiculum terminale. The laminae of the second sacral vertebra were found to be fused but positioned asymmetrically with respect to one another, while the laminae of the third and fourth vertebrae were hypoplastic, suggesting spina bifida of the sacrum. This configuration of anomalies is symptomatic of type II Klippel-Feil syndrome. In addition, the studied individual revealed heterotopic ossification on the supinator crest of the ulna, which may have arisen as a result of dislocation of the elbow joint.

Congenital Synostosis of Cervical Vertebrae: An Osteological Study and Review of the Literature

“…Moreover, minor but intermittent head and neck pain are noted, the trapezii muscles are prominent, the neck movements are restricted, and the hairline is lowered [7, 11-12]. Furthermore, in cases of a pronounced asymmetry between the two halves of the vertebral body of a CFCV, torticollis will likely develop with a markedly titled position of the head [14]. Klosinski et al noted that in atlantoaxial synostosis, the vertebral vessels could be potentially compressed, leading to symptoms like occipital and neck pain, vertigo, neurological deficits, and presumably cerebral ischemic infarction, especially in atlantoaxial synostosis where the head rotation is seriously limited [13].…”

“…Major neurological complications involve quadriplegia or death after minor trauma in high-risk patients [18]. CFCV may be associated with a syndrome known as Klippel-Feil syndrome, which is a complex congenital disease with various clinical manifestations [14]. In 1919, Feil subdivided that syndrome into Type 1 with massive fusion of many cervical and upper thoracic vertebrae into blocks, Type 2 with fusion of only one or two interspaces (usually C2-C3 or C5-C6), and Type 3 with both cervical fusion and lower thoracic or lumbar fusion often associated with multiple organ abnormalities and neurological compromise [19].…”

Fusion of the 2nd with the 3rd Cervical Vertebrae (C2-C3): A Case Series with Possible Clinical Significance

“…KFS clinical features consist of the triad, low posterior hairline, short neck, and decreased range of motion, which is observed in 50% of the patients [8]. In cases of asymmetrical fused CV, torticollis can develop with a markedly titled position of the head [20].…”

Klippel–Feil syndrome with associated cervical rib in a human skeleton from Wągrowiec (Poland, 14th–17th centuries)

“…KFS is a rare genetic disorder with an occurrence estimated at 1:40,000–50,000 live births (Wessell, DeRosa, Cherrick, & Sherman, ). Although since the first description of the syndrome by Maurice Klippel and Andre Feil in 1912 (Henneberg & Otocki, ) a wide range of reports concerning the occurrence of this syndrome has been presented in the clinical literature (e.g., Samartzis et al, ; Wessell et al, ), relatively few have been presented in publications about archaeological material (e.g., Ortner & Putschar, ) and only two cases of KFS were derived from Poland—one of which was diagnosed in the skeleton of a child (Marchewka, Borowska‐Strugińska, Czuszkiewicz, & Kliś, ) and the other involved the case where only two cervical vertebrae were preserved (Gładykowska‐Rzeczycka, ).…”