Hydrocephaly at Medici Court of Florence (16th century)
M. Castagna, S. Fattori, A. Vitiello, D. Caramella°, N. Villari*, G. Fornaciari**
° Division of Diagnostic and Interventional Radiology, Dep. Of Oncology, Transplants and Advanced Technologies in Medicine, University of Pisa, Italy
*Department of Clinical Physiopathology, Section of Clinical Radiology, University of Florence
**Department of Oncology, Transplants and Advanced Technologies in Medicine, Division of Paleopathology, University of Pisa
Abstract
Introduction. Hydrocephalus is a congenital or acquired pathology of CNS caused by a misbalance
between the production of cerebrospinal fluid and its absorption.
Within the framework of the “Medici” Project, the ancient rulers of Renaissance Florence, a paleopathological team of experts from the University of Pisa, the University of Florence and the Superintendence for Florentine Museums, is carrying out a study on 49 tombs of some of the Medici
family members (16th-18th centuries) housed in the so-called Medici Chapels of the Basilica of San
Lorenzo in Florence. The most recent biomedical technologies have been employed to obtain as much information as possible about the genetic make-up, eating habits, life styles and diseases of these important rulers of Renaissance Florence.
Methods. The skeletal remains of a child, recovered inside a small crypt of the Basilica of S. Lorenzo
revealed a little boy of about 5 years, with a stature of 1.15 m. This individual has been identified whit Don Filippino (1577-1582), young son of the Grand Duke Francesco I (1541-1587). The abnormally enlarged skull of this subject was submitted to macroscopic and radiological study (standard X-ray and CAT).
Results and discussion. The paleopathological study, cranial dimensions and calculation of cranial
capacity show evident hydrocephaly. The curvature of the left tibia and fibula demonstrates light rickets. A portrait of don Filippino, painted in the same year of death (1582), shows a picture of non-severe hydrocephaly, with low implant of the orbits and auricles and evident expansion of the cranial vault.
Conclusion. This case of hydrocephaly is important not only from paleopathological point of view, but
also because it is the first time that a diagnosis of this disease is supported also by an artistic representation.
Abstract
Hydrocephalus is a congenital or acquired pathology of the central nervous system distinguished by a misbalance between the production of cerebrospinal fluid and its absorption.
A review of archaeological materials reveals that diagnostic criteria which fully delineate the condiction have not been established.
Remains of 5 years old child was recovered within the archaeological excavation inside the crypt of S. Lorenzo’s church in Florence (Italy). This skeleton has been identified whit Don Filippino (1577-1582), young son of the Granduca Francesco I of the Medici’s family.
The skull of this child manifest some outstanding skeletal abnormalities that indicate hydrocephalus. Cranial capacity and measures of cranial dimensions was calculated and applied to this individual.
Background
Hydrocephalus is a congenital or acquired pathology of the central nervous system, consisting in an intracranical cerebrospinal fluid (CSF) increase and accumulation due to different situations.
The classification of this pathology include a large number of subtypes, due to different causes. Inordinate quantities of fluid may collect over the sites of deficiencies in cerebral substance caused by cerebral atrophy or hypoplasia or by brain surgical excision. In these cases there is no increase in pressure, the cranial cavity is normal, the calvarium is thick, the sutures and the fontanels close early. This disorder is called hydrocephalus ex vacuo (Dandy, 1944).
Excessive fluid might accumulate either when the secretion of fluid from the choroids plexuses is excessive or when its absorption through the arachnoid villi is impaired or when there is a ventricular system obstruction (malformations, inflammations, tumors). In these circumstances the results are the increased intracranial pressure and the distention of the fluid channels in the near side of the
obstruction.[Squartini].
The most common forms of hydrocephalus occur in young children (Tillier et al., 2001) and it is the congenital type (today about 1 ‰) (Rizzuto and Cavallaro, 2007), due to a malformation obstruction for errors in development in which the channel is stenosed or atresic, particularly in the aqueduct or in the foramens of Luschka and Magendie.
Because the condition results from a differential pressure gradient, cranial elements respond differently depending on the state of craniofacial development. In infants, until two years, hydrocephalus results in cranial enlargement and may include signs of increased intracranial pressure, while in children and in adults, who lack the expandability of the infantile cranium, it results in increased intracranial pressure only (Vaughan and MacKay, 1975 in the Archaeology of disease; Milhorat, 1972 ). In the latter case, ventricular expansion generally results in:
an increased depth of the sulcal and gyral impressions; erosion of the dorsum sellae and posterior clinoids;
enlargement of the emissary venous channels (New and Weiner, 1971; Milhorat, 1972)
A variable degree of sutural separation may occur in children up to the age of 10-15 years (Milhorat, 1972).
Obstructive hydrocephalus has been subdivided into three major types according to the site of accumulation of the cerebrospinal fluid: internal, communicating and external hydrocephalus.
When the fluid damming back inside the ventricular system occur the internal hydrocephalus, while in the communicating hydrocephalus the basal cisterns as well as the ventricular spaces are distended with fluid (Dandy, 1944).
The external hydrocephalus is a rare type of disease, in which fluid accumulates outside the ventricular system in the subarachnoid and subdural spaces (Rakate, 1999). The ventricles are collapsed, the brain is small and is compressed onto the base of the skull.
collection of variable amounts; 2) dilatation of subarachnoid spaces without cortical atrophy; 3) mild to moderate ventriculomegaly without periventricular lucency and without clinicoradiological features of raised intracranial pressure; 4) increased head circumference with opened but non-tense fontanelles (Kumar, 2006).
This type of hydrocephalus is probably always secondary to the internal or communicating ones and follows rupture of the walls of the ventricles or the delicate arachnoid membrane (Dandy, 1944). The usual cause of congenital communicating hydrocephalus is the absence of the subarachnoid space due to failure of splitting of the leptomeninx into pial and arachnoid layers.
Common causes of obstructive acquired hydrocephalus are inflammatory exudates and hemorrhagic residues or neoplasm and subdural hematomas. In particular the possibly causes of external
hydrocephalus include: trauma, meningitis, haematoma and intraventricular surgery.
The condition is benign and self-limiting in the majority of cases. Usually in fact it is called benign infantile hydrocephalus (BEH).
External hydrocephalus may simulate a condition known as benign familial megalencephaly, but it is usually seen when multiple family members have large heads; or macrocephaly, but it usually resolves spontaneously by the age of two years (Rakate, 1999).
A recent hypothesis propose the benign external hydrocephalus as a first step of a two hit disease: idiopathic normal pressure hydrocephalus, consisting of BEH in infancy, followed by deep white matter ischemia in late adulthood (Bradley 2006).
Other causes of hydrocephalus are summed up in table 1.
In past the condictions was different, for example the parasitic infection of toxoplasmosis had more importance than today. The infection is associated with eating undercooked meat and this may have been a common practice in earlier people. The possibility of a slowly growing tumor within the skull causing hydrocephalus must also be remembered, but these causes are of changes in soft tissues and must therefore remain hypothetical in palaeopathology (Roberts C. and Manchester K., 1995). In these group of tumors there are: pilocytic astrocytoma; ependymoma; choroid plexus papilloma; craniopharyngioma.
The diagnosis of hydrocephalus from archaeological contexts rests upon the recognition of skull enlargement and upon the anatomical configuration of the facial and cranial skeleton (Richards and Anton, 1991). In particular there are several features important in archaeological specimens to consider in order to diagnose the condition (el-Najjar and McWilliams, 1978 in The Cambridge
encyclopedia…):
enlargement of the head; thinning of the skull bones; bulging fontanelles;
widely separated sutures often showing wormian bones; atrophy of the supraorbital ridges;
flattening of the cranial base.
In palaeopathological records hydrocephalus is rare (Brothwell and Sandinson, 1967; Ferembach, 1968; Ortner and Putschar, 1985; Zammit and Crubezy, 1986; Richard and Anton, 1991; Mari and Campillo, 1995) and only 6 of the 30 cases reported in literature are true hydrocephalic subjects.
Reading the autopsy report of the times we can think that this case was an external hydrocephalus, with the liquor increase only in the sub-arachnoid space.
Materials and methods
The discovery of the skeleton occured in an archeological excavation inside the crypt of S. Lorenzo’s church in Florence (Italy), where the Medici family is buried.
This skeleton has been identified with Don Filippino (1577-1582), young son of the Granduca Francesco I. His tomb was together with other seven infant burials, very damage by the Arno river flood in 1966, but appeared well maintained.
The skeleton was considered to belong to a child of 4-5 years old, based on patterns of dentition and long-bone length (Ubelaker, 1987).
The cranial bones of this individual manifest some outstanding features that may indicate that the child suffered from hydrocephalus.
The exocranial and endocranial surfaces show no evidence of an osteolytic inflammatory reaction or intravital skull fracture.
The endocranial surface is unusually heavily marked by sulcal and gyral impressions.
Cranial size, abnormal skull traits and hypodevelopment of the postcranial skeleton (??) support the deduction of hydrocephalus.
Results and discussion
The skull possibility of enlargement due to a pressure increase it’s great, specially during the first years of life. So it’s very helpful the cranial capacity to make a diagnosis of hydrocephalus. Cranial capacity of the study individual was calculated using the Bergeroff method (Lischi and Manichini, 1959). Its measure is 1553 cc, higher respect the medium normal values of a 4 and 5 years old male (respectively 1400 cc e 1420 cc).
An enlarged or swollen cranial vault accompanied by a normal facial skeleton has usually been considered sufficient evidence for a diagnosis of hydrocephalus.
It is the case: we have found increased measures of cranial dimensions (max. cranial breadth, max. frontal breadth, min. frontal breadth, biasterionic breadth, nasion-bregma chord, lambda-opisthion chord, frontal arc and occipital arc) and reduced or normal facial dimensions (nasal breadth, orbital breadth, palatal breadth, bigonial breadth and symphysial height), compared with medium normal values of 5 years old male (Richards and Anton, 1991) (table 2).
The lack of evidence of skull fracture, together with the smooth surface of the edges of suture diastasis, indicate that the separation occurred when the child was alive and that he survived, or it can
demonstrate a no fusion of the sutures, in accord to a congenital hydrocephalus. While it is normal for a 3 year old child to have open cranial sutures, their presence at this age is indicative of a developmental anomaly.
The margins of the fontanelle are characterized by long indentations.
The left side of the skull is larger than the right side. This asymmetry seems to be the result of a differential development of the cerebral hemispheres. An hypothesis is described by a slow-growing left-sided process (e.g. tumour) in this child, or asymmetric enlargement of the ventricular system (Weber et al. 2003).
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