Virchows Arch (2006) 449:476–478 DOI 10.1007/s00428-006-0275-5
CASE REPORT
The aortic coarctation and the Etruscan man: morphohistologic diagnosis of an ancient cardiovascular disease Rosalba Ciranni & Gino Fornaciari
Received: 20 April 2006 / Accepted: 12 July 2006 / Published online: 29 August 2006 # Springer-Verlag 2006
Abstract Reports of cardiovascular diseases in ancient time are very rare since the material mainly consists of skeletal remains; therefore, these diagnoses can only be carried out indirectly, through the marks left on bones. Here we show a case of coarctation of the aorta diagnosed on bones. Aortic coarctation is a congenital disorder in which a portion of the aorta is narrowed to various extent. A collateral circulation system is enrolled to allow adequate compensation of the blood flow. Collateral vessels may become enlarged, producing a distinctive notching on the pleural surface of the ribs and on adjacent bones. Excavation of a sixth to fifth century B.C. Etruscan tomb revealed three funerary chambers (celle) housing 14 skeletal remains of adults. The ribs of one of the male skeleton showed Bnail stroke^ indentations. Detailed macroscopic examination enabled us to identify them as notching and led to the diagnosis of postductal aortic coarctation. Histological analysis of bone tissue from the notching areas excluded inflammatory and pathological erosive events, supporting the macroscopic diagnosis. The present paper is the first description of aortic coarctation in paleopathology. Keywords Ribs notching . Hypertension . Collateral circulation
R. Ciranni (*) : G. Fornaciari Section of History of Medicine, First Division of Pathology, Department of Oncology, Transplants and Advanced Technologies in Medicine, Pisa University, 56126 Pisa, Italy e-mail:
[email protected]
Introduction An Etruscan chamber tomb, named Tomba della Selvaccia (Evil Forest Tomb), was discovered near Siena (Tuscany, Italy). Three rectangular celle (archaeological name for funerary Etruscan chambers), organized around a squared vestibule, revealed rich funerary pottery, mainly consisting in ollae, kylikes, and olpai made of the typical Etruscan polished black bucchero ware. The presence of an iron ring, a pair of tweezers, and a very interesting bronze fibula, together with the pottery, allowed us to date the tomb between the end of the sixth century and the early fifth century B.C. The 14 human skeletons remains (nine males and five females) were scattered all over the three chambers. Most of the remains were disconnected ab antiquo. The anthropological data suggested, for all individuals, to belong to the same family group [7]. Fifteen ribs, found mixed with other human bones showed the typical signs of postductal aortic coarctation. Three ribs, still partially connected to the skeleton, made the attribution to an incomplete male skeleton buried there possible. It is well-known that coarctation of the aorta is an obstructive congenital heart disorder consisting of an encroachment of variable severity of the aortic lumen. Clinical signs mainly depend on the degree of narrowing of the aorta. The hypertension upstream of the site of restriction forces the blood into the collateral circulation producing a pathological enlargement of intercostal vessels that assume irregular, sinuous shapes. During growth, ribs are constantly subjected to the pulsating loops of engorged arteries, thus generating the notching, which represents a distinctive sign of the disease. In this paper, we present a detailed macroscopic examination of the pathological ribs and an extensive histological analysis of the bone tissue from the notching area.
Virchows Arch (2006) 449:476–478
Materials and methods Fifteen ribs (Fig. 1a), belonging to an incomplete skeleton of an adult male about 170 cm tall and aged 40–45 years, were examined macroscopically; the ribs were then anatomically positioned and X-rayed (Gilardoni Apparatus, Italy). X-ray films (Du Pont CRONEX 4 Blue Base film, 3040 cm) were developed using Agfa developer and fixative. Ten X-rays per rib were performed. Samples of bone tissue from areas of notching were collected, decalcified, rehydrated according to Sandison [8], and submitted to routine dehydration and paraffin-embedding for histological analysis. Tissue sections were fixed on slides and stained using three different methods: basic staining with hematoxylin–eosin, and differential staining with either periodic acid-Schiff stain or Masson trichrome stain [5, 9]. Slides were examined by light microscopy.
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rib surface. External examination revealed many roundshaped areas of notching, 0.5–1.5 cm in diameter, within the subcostal groove and in proximity to the posterior costal angle (Fig. 1b). Notching was observed through to the lower ribs. X-ray confirmed the macroscopic diagnosis, showing bilateral, symmetrical notching on the pleural surface of the ribs with no signs of inflammatory sclerosis. Few residual vertebras and the only clavicle found were free of notching. Histological analysis on hematoxylin–eosin stained samples revealed well-preserved, thinned, and pierced costal bones with evident haversian systems and with no sign of sclerosis or pathological erosion (Fig. 1d). Differential staining showed an increased thickness of the lamellar tissue within the layer of compact bone (Fig. 1c).
Discussion Results The anthropological data produced by Pardini and Mannucci [7] suggested that the skeleton belonged to a medium-built male. Indices of robustness of the humerus and the femur were average and consistent with each other. A macroscopic diagnosis of postductal aortic coarctation was readily made since notching was clearly evident on the
Fig. 1 Inferior view of the 15 ribs belonging to an adult male skeleton buried in the Etruscan tomb La Selvaccia. Arrows show the distribution of the typical notching due to aortic coarctation (a). Inferior aspect of the third left rib showing some notching (arrows) inside the subcostal groove (2; b). Differential histology of the transversal section of the third left rib shows the excavation (arrows)
Coarctation of the aorta is an obstructive congenital disorder representing about 5% of cardiovascular diseases. Frequency in men is three to four times higher than in women. The defect is thought to result from the incomplete development of the aortic arch during organogenesis, but the etiology is unknown. Two classic forms have been described. The infantile preductal form is usually associated with intracardiac anomalies (ventricular septal defect, subaortic stenosis,
produced by the pathological development of the artery (6). The costal bone is pierced but not sclerotic and with no erosive signs (c). The costal bone is well-preserved, haversian systems are well-evident (arrows), compact bone shows an increased thickness of lamellar tissue at level of the notching (hematoxylin–eosin, 20; d)
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Fig. 2 Diagram showing two of the possible pathways of the collateral circulation between the pre-coarctation arterial branches and the post-coarctation arteries. On the right: blood flows into the internal mammary artery (e) then spreading into intercostal arteries (d), which feed into the aorta descending branch (b). On the left: blood flows into the succlavia artery (f) bypassing coarctation (c) trough the esophageal artery (g) (arrows show the direction of blood flow)
mitral valve defects, etc.) and, from the physiopathologic viewpoint, is classified among the ductus-dependent congenital heart diseases. This form is characterized by high neonatal mortality in the absence of early surgical intervention. The postductal form, mostly asymptomatic during childhood, becomes symptomatic in the adult. Postductal coarctation is characterized by the development of compensatory collateral circulation between pre-coarctation arterial branches and post-coarctation arteries [1, 4]. Figure 2 shows two possible collateral routes. As a consequence of growing blood pressure in the aorta, collateral vessels (i.e., intercostal and internal mammary arteries) may become enlarged producing, in time, notching on the pleural surface of the ribs and onto adjacent bones [1]. Reports of ancient cardiovascular diseases are very rare in paleopathology, and no data are available on aortic coarctation [2]. Atherosclerosis was diagnosed in Egyptian and Peruvian mummies [3]. Recently, Miller et al. [6] have diagnosed a case of myocardial infarction in a mummy. However, paleopathological material mainly consists of skeletal remains; therefore, the diagnosis of cardiovascular diseases can only be carried out indirectly, through the marks left on bones, as in the case of aortic aneurysm [10].
Virchows Arch (2006) 449:476–478
Here we report our findings relative to the skeleton of a medium-built adult man, aged 40–45 years, belonging to a sixth to fifth century B.C. Etruscan population [8]. A macroscopic detailed observation of the ribs revealed distinctive notching that we related to a strong hypertrophy of intercostal arteries as a consequence of aortic coarctation. Histological analysis confirmed the diagnosis and ruled out the possibility of erosive and/or inflammatory disorders. The man was moderately robust; therefore, he must have coped well with the disease. We hypothesize the development of an extended collateral circulation system to allow adequate compensation of blood flow. In fact, notching was very marked, showing a remarkable increase of the costal artery calibers, and it was observed down through to the lower ribs. Furthermore, anthropological data showed that the main limb bones were well-proportioned, yet indicative of good compensation, while aortic coarctation is often accompanied by unbalanced limb development, with the upper limbs becoming more robust. We believe that this report is the first well-documented diagnosis of aortic coarctation based on sole skeletal remains. We see it as a step forward to expand the investigation of ancient cardiovascular disease in paleopathological literature.
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