Spinal pathology in the Medici family, Grand Dukes of Florence (XVI-XVII centuries)

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24 Paleopathology Newsletter SPINAL PATHOLOGY IN THE MEDICI FAMILY, GRAND DUKES OF FLORENCE (XVI-XVII CENTURIES) Valentina Giuffra, Angelica Vitiello, Sara Giusiani, Antonio Fornaciari,* Natale Villari,** and Gino Fornaciari Department of Oncology, Transplants and Advanced Technologies in Medicine, Division of Paleopathology, History of Medicine and Bioethics, University of Pisa, Italy *Department of Archaeology and History of Arts, Section of Medieval Archaeology, University of Siena, Italy **Department of Clinical Physiopathology, Section of Clinical Radiology, University of Florence, Italy Correspondence to: Prof G Fornaciari, Department of Oncology, Transplants and Advanced Technologies in Medicine, Division of Paleopathology, University of Pisa, Via Roma 57, 56126 Pisa, Italy tel.: 0039 050 992894 fax: 0039 050 992706 [email protected] Key words: Florence, Renaissance, Medici, Klippel-Feil’s syndrome, DISH, osteoarthritis, Pott’s disease, scoliosis Abstract Study Design : A paleopathological study of spinal diseases in a high-social class Renaissance sample. Objective : To detect the incidence of vertebral pathology affecting the members of the Medici family in Florence and to investigate the possible causes. Summary of Background Data : except for a case of tuberculosis attested by archival documents no data about the spinal diseases of this sample were known before the exploration of the Medici burials in San Lorenzo (Florence). Methods : the skeletal remains were submitted to macroscopic investigation and radiological examination. Results : a series of spinal diseases were diagnosed, including congenital lesions (Klippel-Feil’s syndrome, scoliosis, spina bifida occulta, coccyx sacralization, coccyx angulation), degenerative changes (osteoarthitis and Schmorl’s nodes), metabolic disorders (Diffuse Idiopathic Skeletal Hyperostosis) and infectious disease (Pott’s disease). Conclusions : we have a complete picture of severe spinal pathology affecting an aristocratic class of the Italian Renaissance. Some of these spinal diseases are related to the lifestyle of the members of this family, which practiced horse back riding and hunting, with consequent mechanical stress. They also consumed a high-calorie diet. Introduction The “Medici Project”, a multidisciplinary research following a scientific agreement between the University of Pisa, the University of Florence and the Superintendent of Florentine Museums, was designed to perform an archaeological and paleopathological study on the 49 funerary depositions of the Medici Grand Dukes, in the famous Medici Chapels of the Basilica of San Lorenzo in Florence. After permission was given by the Superintendent Antonio Paolucci in 2002, the Medici Project, which represented a unique opportunity to reconstruct the

Transcript of Spinal pathology in the Medici family, Grand Dukes of Florence (XVI-XVII centuries)

24 Paleopathology Newsletter

SPINAL PATHOLOGY IN THE MEDICI FAMILY, GRAND DUKES OF FLORENCE (XVI-XVII CENTURIES)

Valentina Giuffra, Angelica Vitiello, Sara Giusiani, Antonio Fornaciari,* Natale Villari,** and Gino Fornaciari Department of Oncology, Transplants and Advanced Technologies in Medicine, Division of Paleopathology, History of Medicine and Bioethics, University of Pisa, Italy *Department of Archaeology and History of Arts, Section of Medieval Archaeology, University of Siena, Italy **Department of Clinical Physiopathology, Section of Clinical Radiology, University of Florence, Italy Correspondence to: Prof G Fornaciari, Department of Oncology, Transplants and Advanced Technologies in Medicine, Division of Paleopathology, University of Pisa, Via Roma 57, 56126 Pisa, Italy tel.: 0039 050 992894 fax: 0039 050 992706 [email protected] Key words: Florence, Renaissance, Medici, Klippel-Feil’s syndrome, DISH, osteoarthritis, Pott’s disease, scoliosis Abstract Study Design: A paleopathological study of spinal diseases in a high-social class Renaissance sample. Objective: To detect the incidence of vertebral pathology affecting the members of the Medici family in Florence and to investigate the possible causes. Summary of Background Data: except for a case of tuberculosis attested by archival documents no data about the spinal diseases of this sample were known before the exploration of the Medici burials in San Lorenzo (Florence). Methods: the skeletal remains were submitted to macroscopic investigation and radiological examination. Results: a series of spinal diseases were diagnosed, including congenital lesions (Klippel-Feil’s syndrome, scoliosis, spina bifida occulta, coccyx sacralization, coccyx angulation), degenerative changes (osteoarthitis and Schmorl’s nodes), metabolic disorders (Diffuse Idiopathic Skeletal Hyperostosis) and infectious disease (Pott’s disease). Conclusions: we have a complete picture of severe spinal pathology affecting an aristocratic class of the Italian Renaissance. Some of these spinal diseases are related to the lifestyle of the members of this family, which practiced horse back riding and hunting, with consequent mechanical stress. They also consumed a high-calorie diet. Introduction

The “Medici Project”, a multidisciplinary research following a scientific agreement between the University of Pisa, the University of Florence and the Superintendent of Florentine Museums, was designed to perform an archaeological and paleopathological study on the 49 funerary depositions of the Medici Grand Dukes, in the famous Medici Chapels of the Basilica of San Lorenzo in Florence. After permission was given by the Superintendent Antonio Paolucci in 2002, the Medici Project, which represented a unique opportunity to reconstruct the

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health and life style of the members of this important family of the Italian Renaissance, was begun. Up until now 15 tombs, including the burials of nine children, have been investigated (Fornaciari et al., 2005, 2006, 2007).

The Medici were one of the most powerful and influential families of the Italian Renaissance. Their successful commercial and banking activity allowed them to build long-lasting social power and to gain political prominence, initially in Florence, and later in the entire region of Tuscany. The Medici promoted art and science; they were patrons of several great artists of the period, such as Leonardo, Michelangelo, Benvenuto Cellini. Materials and Methods

Cosimo I (1519-1574) was the son of Giovanni delle Bande Nere (1498-1526) and

Maria Salviati (1499-1543). After the murder of Alessandro de’ Medici, Cosimo, who belonged to a secondary branch of the family, succeeded in becoming Duke of Florence (1537-69) and, in 1569, he was named 1st Grand Duke of Tuscany by Pope Pius V and governed until his death. In 1539 he married a Spanish noblewoman, Eleonora from Toledo (1519-1562). They had 11 sons, of whom Ferdinando outlived the others. Cosimo was buried in a lateral chapel of the Basilica with his wife Eleonora, their second son Cardinal Giovanni (1543-1562) and their seventh son Don Garzia (1547-1562).

Francesco I (1541-1587), son of Cosimo I and Eleonora from Toledo, was the 2nd

Grand Duke of Tuscany. He married Giovanna from Austria (1548-1578), daughter of Ferdinand I of Hapsburg (1503-1564) and had six daughters and one son, who died at the age of 5 years. Francesco was buried in a lateral chapel with his wife Giovanna.

Ferdinando I (1549-1609), the sixth son of Cosimo I, was the 3rd Grand Duke of

Tuscany from 1587 until his death in 1609. In 1589 he married Cristina from Lorraine (1565-1636) and they had 9 sons, of whom the firstborn, Cosimo II, succeeded to the throne. Ferdinando was buried in a lateral chapel with his wife Cristina and their sons Cardinal Carlo (1595-1666) and Francesco (1594-1614).

The remains of the Grand Dukes, their wives and sons were not found in their primary

depositions, but had been placed in zinc boxes by anthropologists, who explored some depositions of the Medici family in San Lorenzo after the Second World War (Genna, 1948). The skeletons were studied by macroscopic observation and submitted to X-ray examination performed at the Careggi Hospital of Florence. Results

Cosimo I (1519-1574) (55years old)

Examination of the spinal column of Cosimo revealed the ossification of the anterior longitudinal ligament on the right side, at the level of the vertebral bodies of T6, T7 and T8, forming a bony bridge between the vertebrae that appears as a continuous line of bumps. The intervertebral disks and the articular surfaces were preserved. Partial ossification of the right anterior ligament, but without vertebral fusion, was present in other thoracic and lumbar vertebrae. A bony bridge on the left side merged L2 and L3.

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The entire column showed diffuse and severe arthritis, in particular in the lower thoracic and lumbar spine, at the level of the spinous processes, of the superior and inferior articular processes and of the margins of the vertebral bodies. Most of the ligamenta flava were ossified. Schmorl’s nodes were observed on the vertebral plates of T8, L2 and L3.

Eleonora from Toledo (1522-1562) (40 years old)

Eleonora displayed slight arthritis in her lower spine, with arthritic changes most notably at the level of the superior and inferior articular processes and slight ossification of the ligamenta flava. The posterior sacral canal was open from the 3rd to the 5th sacral vertebra.

Cardinal Giovanni (1543-1562) (19 years old)

Schmorl’s nodes were observed on the lower thoracic and lumbar vertebral plates, in particular on T8, T9, T10, T11, T12, L2, L3, L4, L5 (fig.1a, b).

Francesco I (1541-1587) (46 years old)

Francesco I suffered from moderate vertebral arthritis, with osteophytosis at the level of the vertebral plates, the superior and inferior articular processes and the transverse processes. The spinous processes were markedly deformed and the ligamenta flava were ossified (fig.2a, b, c). A congenital angulation of the coccyx was observed, at about 90° (fig.3).

Giovanna from Austria (1548-1578) (31 years old)

The macroscopic and radiological examination revealed that Giovanna was afflicted by a severe scoliosis of the lumbar spine with impressive deformity of the pelvis. The lumbar spine presented a marked right scoliosis in addition to a compensatory left curve of the thoracic segment. The sacrum participated in the lumbar deviation, which appeared to be rotated and inclined towards the left (fig.4a, b). Severe diastasis of the pubic symphysis is a consequence of the seventh delivery, which caused the death of Giovanna.

Fig. 1 Schmorl’s nodes of Giovanni: a) defect on the inferior plate of a thoracic vertebra; b) defect on the superior plate of a lumbar vertebra

Fig. 2 Osteoarthritis of the thoracic spine in Francesco I: a) osteophytosis of the vertebral body; b) osteophytosis of the right inferior articular process; c) degenerative changes of the spinous process

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Ferdinando I (1549-1609) (60 years old) Ferdinand I suffered from diffuse arthritis of the spine. The anterior surfaces of the bodies of C3, C7, T1, T2, T3 and T4 presented ossification of the right anterior ligament, but without formation of bony bridges between the vertebrae. The vertebral bodies from T5 to T9 were fused in a block due to the ossification of the right anterior ligament, conferring the typical aspect of “candle wax” to this spinal segment (fig.5a, b). Another fusion was observed at the level of the T10 and T11 bodies, also linked by a bony bridge on the left-hand side. The intervertebral spaces and the apophyseal joints were normal. Several vertebrae showed ossification of ligamenta flava, interspinal and supraspinal ligament insertions. Furthermore, the coccyx was fused with the sacrum and the posterior sacral canal was open from the 3rd to the 5th sacral vertebra (fig.6).

Fig. 3 Malformation of the coccyx of Francesco I, with angulation at 90° (white arrow)

Fig. 4 Scoliosis of Giovanna from Austria: a) severe scoliosis and deformed pelvis; b) antero-posterior projection of the column and pelvis

Fig. 5 DISH of Ferdinando I: a) anterior view of the column with ossification of the anterior right vertebral ligament, at the level of the 5th-11th thoracic vertebral bodies, conferring the typical “candle wax” appearance; b) antero-posterior projection of the column

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Cristina from Lorraine (1565-1636) (72 years old)

The spine of Cristina was affected by an impressive left scoliosis of the lower thoracic and lumbar column, forming an angle of about 90°. This deformity was caused by the lateral wedge-shaped collapse of the bodies of the 12th thoracic and the 1st lumbar vertebrae, with partial fusion on the concavity. All the lumbar vertebrae were deformed like “fish vertebra”. The 12th thoracic, 1st and 2nd lumbar vertebrae presented ankylosis of the right articular posterior facets. The body of the 5th lumbar vertebra presented a lateral edge-shaped flattening with marked left lipping, and the left articular process was deformed, very widened and affected by arthritic changes. It can be inferred that the lumbar tract presented a curve as compensation for the right convexity. The spinous processes of the 3rd and 4th lumbar vertebrae had an additional facet, (kissing spines) at the point where they are in contact (fig.7a, b).

Fig. 6 Posterior view of the sacrum of Ferdinando I with the defect involving the last three sacral vertebrae. The sacralization of the coccyx is also visible

Fig. 7 Scoliosis of Cristina from Lorraine: a) left scoliosis of the lower thoracic and lumbar column, forming an angle of about 90°; b) antero-posterior projection of the column

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Cardinal Carlo (1595-1666) (71 years old) The spine of Carlo showed a number of lesions and anomalies. Atlanto-occipital fusion was observed. The articular facets, pillars and posterior bodies of the 1st-5th cervical vertebrae were also fused, with narrow disc spaces, forming a C1-C5 block, but with absence of bone remodeling. The axis was fixed in an abnormal oblique-right directed position (fig.8a, b, c).

A second block, involving the vertebral bodies with wedge-shaped collapse, fusion, and formation of an angular kyphosis, was at the level of C6-C7 (fig.8b, black arrow). CT revealed massive bone remodeling of the vertebral bodies (fig.8d).

Several other fusions, involving the posterior arches as well, were present in the thoracic

segment, between T2 and T3, T7 and T8, T9 and T10, T11 and T12, and in the lumbar segment, where L5 was fused with a supernumerary L6. The lumbar column was composed of six true vertebrae without lumbarization, as the sacrum had the five normal elements. A bony bridge between T9 and T10 vertebral bodies is the result of the anterior-right vertebral ligament ossification. The remaining free vertebrae articulated normally with the adjacent elements. Discussion

As far as congenital diseases are concerned, the fusion of the cervical spine observed in Cardinal Carlo clearly showed the absence of bone remodeling at the level of the C1-C5 block; this feature, as well as the fusion between the atlas and the occipital bone, give evidence in favour of a congenital, rather than acquired disease. The spinal column of Carlo showed a block involving the arches and posterior bodies between C1 and C5; other anomalies, such as fusions in the thoracic and lumbar spine and a variation in the number of lumbar vertebrae (6), were also observed. These features suggest the diagnosis of Klippel-Feil’s Syndrome (KFS). The congenital fusion of two or more cervical vertebrae was first described in 1912 by Maurice

Fig. 8 Klippel-Feil’s syndrome and Pott’s disease in Cardinal Carlo: a) ankylosis of the cervical column in frontal view; b) lateral view; c) X-ray latero-lateral projection of the cervical column; d) Computed Tomography showing osteolytic lesions in C6. A first block from C1 to C5, referable to Klippel-Feil syndrome, and a second fusion merging C6 and C7 (black arrow), referable to Pott’s disease, are visible

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Klippel and André Feil, who examined a clinical case characterized by a massive fusion of cervical elements (Klippel and Feil, 1912). In particular, the case of Carlo belongs to type III of Feil’s classification, where fusion of the cervical spine is associated with the fusion of other elements in the thoracic and lumbar segments. Furthermore, occipito-atlantal fusion, marked facial asymmetry and temporo-mandibular dysplasia, producing a deviation of the face on the right, anomalies frequently observed in KFS, were present (Giuffra et al., 2009).

Two cases of severe scoliosis were documented in the Medici family. Giovanna, who appears in numerous portraits, was considered a rather unattractive woman; indeed, some contemporary reports even describe her as "hunchbacked". Giovanna died in 1578 after a difficult delivery at the age of 31. This was the seventh pregnancy for the Grand Duchess and presented several problems. The foetus was badly positioned in shoulder presentation. The midwife had tried to reduce the arm, but in the meantime the uterus ruptured and the placenta was expelled. The description of the autopsy confirms that the spinal column of the Grand Duchess was deformed: “the day after [the death], that was a Friday, she was opened and it was found the foetus was outside the uterus, and the cervix was torn, a fact that surprised the physicians, and explained the reason for expulsion of the placenta. In the rest of the body she was badly conformed, because she had the lower portion of the column twisted in the shape of a capital S…” (Pieraccini, 1986). As recognised by contemporary physicians, the spinal deformity well explains the difficult deliveries and death of Giovanna as a result of the ruptured uterus.

With regard to Cristina from Lorraine, her severe scoliosis probably did not cause particular problems to her deliveries, considering that she had four sons and three daughters, and also some miscarriages. She died at the age of 72 and, in the written sources, no reference is made to this spinal deformity, although it could be hypothesized that it caused back pain and movement problems to the Grand Duchess. The absence of body fractures and osteoporosis strongly supports a static or juvenile etiology of this severe scoliosis.

Another congenital defect documented in the Medici sample is the spina bifida occulta, consisting in the failure of vertebral arch development involving one or more sacral segments. In the living the defect, which is covered by the soft tissue, does not imply the protrusion of spinal cord and nerves and is therefore asymptomatic. In Ferdinand I and Eleonora from Toledo this congenital anomaly is partial, involving only the last three sacral vertebrae. Therefore, this condition was undoubtedly asymptomatic.

Finally, sacralization and angulation of the coccyx are not rare findings. Reed (1967) does not consider sacralization as an anomaly. In Ferdinand the malformation is incomplete, as demonstrated by the absence of extra sacral foramina.

Degenerative arthritis seems to have particularly affected the male members of the family, and this could be related to the life style and activity patterns that characterized the male representatives of the high social classes of the Renaissance age. The Grand Dukes and their male relatives constantly practiced jousting, horse back riding, and hunting. In addition, they wore heavy armour which caused mechanical stress. Their wives and daughters led a more sedentary life style. Vigorous physical activity and advanced age were probably responsible for the diffused spinal arthritis of Cosimo I (55 years), Ferdinando I (60 years) and Francesco I (46 years).

Schmorl’s nodes are the result of vertical disk hernias. The pressure of the intervertebral disk on the upper and lower surface of the vertebral bodies produced a defect in the trabecular bone of the vertebral plate, generally up to 5 mm in diameter and 1-1.5 cm in

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depth (Aufderheide and Rodriguez-Martin, 1998). The etiology of this defect was related to mechanical stress produced by load axial weight; for this reason Schmorl’s nodes are found in the thoracic and lumbar spine and they tend to increase with age. Their presence revealed that, during adolescence, Cosimo and Cardinal Giovanni used to carry very heavy loads on the thorax, probably the heavy armour of that period. A similar pattern was observed in a Naples contemporary series of Aragonese princes (Fornaciari et al., 1988).

As for the metabolic diseases, the changes observed in the column of Cosimo I and Ferdinando I support a diagnosis of diffuse idiopathic skeletal hyperostosis (DISH) (Fornaciari et al., 2009; Rogers and Waldron, 2001). DISH is a common systemic disorder, first identified in 1950 by Forestier and Rotes-Querol, whose main characteristic is the ossification of the anterior longitudinal spinal ligament, with large osteophytes that flow down the spine, producing a typical candle-wax appearance. The thoracic spine is the most commonly involved segment, but any of the spinal segments may be involved. The presence of the descending aorta on the left side of the column prevents ossification to the left side of the thoracic spine. For the diagnosis of DISH the fusion of at least four contiguous vertebrae is necessary, but some authors estimate three vertebrae as sufficient diagnostic criteria (Rogers and Waldron, 2001; Utsinger, 1985). Ossification leads to ankylosis of the involved vertebrae, but the intervertebral disc spaces and apophyseal joints are preserved (Cammisa et al., 1998). The aetiology of DISH, even if still uncertain, has been related to metabolic disorders, in particular obesity and type II diabetes mellitus (Denko and Malemud, 2006; Kiss et al., 2002; Sarzi-Puttini and Atzeni, 2004). Recent paleopathological investigations have linked the incidence of DISH to social status and life style, in particular with nutritional patterns (Jankauskas, 2003; Verlaan et al., 2007; Waldron, 1985). The archival documents and the pictorial sources demonstrate that Cosimo was a well-built individual, who became corpulent in the last decades of life, and that Ferdinando was rather fat during all his life, becoming obese after 40 years of age. On the other hand, a paleonutritional study performed recently on the Medici Grand Dukes and their families, based on the analysis of carbon and nitrogen stable isotopes, revealed a high consumption of meat and intake of marine proteins at 14-30% (Fornaciari, 2008). This evidence seems to confirm the association between DISH and high social status.

Finally, as concerns the infectious diseases of the spine, a case of tuberculosis was identified in the remains of Cardinal Carlo. The fusion of C6-C7 is not to be related with the KFS previously mentioned. In fact, massive bone remodeling observed at X-ray examination and the edge-shaped deformation of the body of C6, which produced collapse of the vertebral body and angular kyphosis, is to be considered the result of cervical spine tuberculosis (Pott’s disease), characterized by neck fistula and gibbus (Ortner, 2003). This has been well-described in the archival records, when Carlo was 8 years of age (Pieraccini, 1986). In order to correct the spine deformation Ferdinando I, Carlo’s father, called one of the most famous physicians of that period, Girolamo di Fabrizio d’Acquapendente, professor of anatomy and surgery at the University of Padua and pioneer in orthopedics, who applied to Carlo a sort of corset, that seems to have been partially efficacious, preventing a more marked forward flexion of the spine, which did not appear to be severely deformed (Marinozzi and Aruta, 2007; Pieraccini, 1986). Conclusion

The paleopathological investigation carried out on the skeletal remains of some members of the Medici family revealed a set of vertebral pathologies, including congenital

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lesions, degenerative changes, metabolic disorders and infectious disease, as summarized in Table 1, and furnished a complete picture of spinal pathology affecting an aristocratic class of the Italian Renaissance.

Table 1

Acknowledgments We thank Laura Cignoni for language review. This study is an integral part of the “Medici Project”, performed with the permission of the Superintendent for Florentine Museums. References Aufderheide C, Rodriguez-Martin C. 1998. The Cambridge Encyclopedia of Human

Paleopathology. Cambridge: Cambridge University Press. Cammisa M, De Serio A, Guglielmi G. 1998. Diffuse Idiopathic Skeletal Hyperostosis. Eur J

Radiol 27:S7-S11. Denko CW, Malemud CJ. 2006. Body Mass Index and Blood Glucose: Correlations with Serum

Insulin, Growth Hormone, and Insulin-Like Growth Factor-1 Levels in Patients with Diffuse Idiopathic Skeletal Hyperostosis (DISH). Rheumatol Int 26:292–97.

Fornaciari G, Brier B, Fornaciari A. 2005. Secrets of the Medici. Archaeology 58 (4):36-41. Fornaciari G, Bruno J, Amadei A, Tornaboni D, Tognetti A. 1989. Pathologie Rachidienne sur un Echatillon d’une Classe Socialement Elevée de la Renaissance Italienne: la sèrie de momies de la basilique de S. Domenico Maggiore à Naples (XVe-XVIe siècles). Journal of Paleopathology 1:59-64. Fornaciari G, Giuffra V, Giusiani S, Villari N, Vitiello A. 2009. The “Gout” of the Medici, Grand Dukes of Florence: a Palaeopathological Study. Rheumatology 48:375-77.

Spine pathology category Disease Affected individuals

Congenital Klippel-Feil Sindrome Cardinal Carlo Scoliosis Giovanna from Austria

Cristina from Lorraine Partial spina bifida occulta Ferdinand I

Eleonora from Toledo Coccyx sacralization

Coccyx malformation Ferdinand I Francesco I

Degenerative Osteoarthritis Cosimo I Francesco I Ferdinand I

Schmorl’s nodes Cosimo I Cardinal Giovanni

Metabolic DISH Cosimo I Ferdinand I

Infectious Pott’s disease Cardinal Carlo

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