The antiquity of hydrocephalus: the first full palaeo-neuropathological description.

The Pathology Museum of the University of Florence houses a rich collection of anatomical specimens and over a hundred waxworks portraying pathological conditions occurring in the nineteenth century, when the museum was established. Clinical and autopsy findings of these cases can still be retrieved from the original museum catalogue, offering a rare opportunity for retrospective palaeo-pathological diagnostics. We present a historical case of severe hydrocephalus backed by modern-day anthropological, radiological and molecular analyses conducted on the skeleton of an 18-month-old male infant deceased in 1831. Luigi Calamai (1796-1851), a wax craftsman of La Specola workshop in Florence, was commissioned to create a life-sized wax model of the child's head, neck and upper thorax. This artwork allows us to appreciate the cranial and facial alterations determined by 30 lb of cerebrospinal fluid (CSF) accumulated within the cerebral ventricular system. Based on the autopsy report, gross malformations of the neural tube, tumours and haemorrhage could be excluded. A molecular approach proved helpful in confirming sex. We present this case as the so-far most compelling case of hydrocephalus in palaeo-pathological research.

[Brain X-radiation for Childhood Epilepsy, Hydrocephalus or Mental Retardation? Research at Tuebingen University, 1940-1946]. / Röntgenbestrahlung des Gehirns zur Behandlung von kindlicher Epilepsie, Hydrocephalus oder Schwachsinn? Forschungen an der Tübinger Universitätsklinik von 1940-1946.

Brain X-radiation for Childhood Epilepsy, Hydrocephalus or Mental Retardation? Research at Tuebingen University, 1940-1946 We reconstructed 65 cases out of a series of "experimental" X-ray-therapy by chart review and reanalysis of publications from a contextual historical perspective. The research procedures in the context of NS-pressure for effectiveness soon dismissed structured scientific procedures and surrendered own standards, whereas radiation impact did not transgress the contemporary guidelines.

Skull deformations in craniosynostosis and endocrine disorders: morphological and tomographic analysis of the skull from the crypt of the Silesian Piasts in Brzeg (16th-17th century), Poland.

RESULTS: of morphological and tomographic (CT) studies of the skull that was found in the crypt of the Silesian Piasts in the St. Jadwiga church in Brzeg (Silesia, Poland) are presented and discussed here. The established date of burial of probably a 20-30 years old male was 16th-17th century. The analyzed skull showed premature obliteration of the major skull sutures. It resulted in the braincase deformation, similar to the forms found in oxycephaly and microcephaly. Tomographic analysis revealed gross pathology. Signs of increased intracranial pressure, basilar invagination and hypoplasia of the occipital bone were observed. Those results suggested the occurrence of the very rare Arnold-Chiari syndrome. Lesions found in the sella turcica indicated the development of pituitary macroadenoma, which resulted in the occurrence of discreet features of acromegaly in the facial bones. The studied skull was characterized by a significantly smaller size of the neurocranium (horizontal circumference 471 mm, cranial capacity ∼ 1080 ml) and strongly expressed brachycephaly (cranial index=86.3), while its height remained within the range for non-deformed skulls. A narrow face, high eye-sockets and prognathism were also observed. Signs of alveolar process hypertrophy with rotation and displacement of the teeth were noted. The skull showed significant morphological differences compared to both normal and other pathological skulls such as those with pituitary gigantism, scaphocephaly and microcephaly.

William Watson Cheyne (1852-1932): a life in medicine and his innovative surgical treatment of congenital hydrocephalus.

William Watson Cheyne lived and trained during a period of great advances in medical knowledge and surgical techniques. Despite his various contributions to the fields of bacteriology and surgery, little is known about his career or his life apart from his affiliations with Joseph Lister. This article aims to identify Cheyne as a pioneer in the treatment of congenital hydrocephalus and sheds light on the man who existed in Lister's shadow for most of his life. Cheyne's technique for surgical intervention of hydrocephalus was a great turning point and contributes to the current treatment strategy utilized today for hydrocephalus.

Choroid plexus coagulation for hydrocephalus not due to CSF overproduction: a review.

OBJECTIVE: This study aims to review the role of choroid plexus coagulation (CPC) for hydrocephalus not due to CSF overproduction. METHODS: The literatures covering CPC/cauterization/extirpation and ablation searched through PubMed were reviewed. RESULTS: The history of CPC goes back to early 1900s by open surgery. It has evolved to mainly an endoscopic surgery since 1930s. With the development of other treatment methods and the understanding of CSF dynamics, the application of CPC dramatically decreased by 1970s. In late 2000, there was a resurgence of CPC in combination with endoscopic third ventriculostomy (ETV) performed in Africa. CONCLUSIONS: CPC remains one of the options for the treatment of hydrocephalus in selected cases. CPC might provide a temporary reduction in CSF production to allow the further development of CSF absorption in infant. Adding CPC to ETV for infants with communicating hydrocephalus may increase the shunt independent rate thus avoiding the consequence of late complication related to the shunt device. This is important for patients who are difficult to be followed up, due to geographical and/or socioeconomic constrains. Adding CPC to ETV for obstructive hydrocephalus in infant may also increase the successful rate. Furthermore, CPC may be an option for cases with high chance of shunt complication such as hydranencephaly. In addition, CPC may act as an adjunct therapeutic measure for complex cases such as multiloculated hydrocephalus. In comparison with the traditional treatment of CSF shunting, the role of CPC needs to be further evaluated in particular concerning the neurocognitive development.

Harvey Cushing and early spinal dysraphism repair at Johns Hopkins Hospital.

As neurological surgery began developing into a surgical subspecialty in the US at the turn of the 20th century, with Harvey Cushing at the forefront, the operative treatment of spinal dysraphism was refined with attempts to minimize complications. Following institutional approval, and through the courtesy of the Alan Mason Chesney Archives, the authors reviewed the Johns Hopkins Hospital surgical files from 1896 to 1912. Patients presenting with spinal dysraphism who underwent surgical intervention by Dr. Harvey Cushing were selected for further analysis. Ten patients presented for surgical intervention for spinal dysraphism, and 7 of these had concurrent hydrocephalus. The mean age of these patients was 5.8 months (range 1-14 months). The mean length of stay was 20.4 days. There were 6 inpatient deaths. At the time of last follow-up, 2 patients were well, 1 patient remained unimproved, and 1 patient (for whom no discharge outcome was available) had died. The cases described in detail offer insight into the breadth of Cushing's practice and the varied approaches he employed. The use of Faradic stimulation to assess nerve root function, the use of complex multilayered closures, and the creation of operative tables for combined treatment of hydrocephalus and spinal dysraphism illustrate Cushing's contributions to developing the field of pediatric neurosurgery.

Pediatric neurosurgical techniques in the 15th century.

The renowned Turkish surgeon Serefeddin Sabuncuoglu (1385--1468 AD), delineates management of various pediatric neurosurgical diseases such as head trauma, spinal trauma, and intrauterine and infantile hydrocephalus, as well as basic surgical principles in his textbook Cerrahiyyetü'l Haniyye, which consists of three chapters dealing with a variety of surgical specialities. The sections on neurosurgery are still relevant to modern medicine. The book combines previous knowledge of Greek, Roman, Arabic, and Turkish surgery. Today, Sabuncuoglu is recognized as a pioneer of pediatric neurosurgery.

Concept and treatment of hydrocephalus in the Greco-Roman and early Arabic medicine.

In the ancient medical literature hydrocephalus was not often described although its existence and symptomatology were well known. Most detailed descriptions of hydrocephalus including the surgical treatment are extant in the encyclopaedic works on medicine of the physicians Oreibasios and Aetios from Amida from the 4th and 6th centuries AD, respectively. Because of their broad scientific interests, this type of physicians, typical for the late Roman empire, were known as philosophy-physicians (iota alpha tau rho o sigma o phi iota sigma tau alpha iota). They defined hydrocephalus in contrast to our present understanding as a fluid collection excluding abscesses visible as a bulging tumour localised either outside or inside the skull of an infant. They classified the hydrocephalus similar as stated first by Galen in the 2nd century AD in four types corresponding to the assumed anatomic localisation of the fluid collection: 1st Type between the skin and the pericranium corresponding to the subgaleal haematoma or caput succedaneum of the newborn in our terminology, 2nd Type between the pericranium and the skull corresponding to the cephal haematoma after delivery, 3rd Type between skull and the meninges with increased head circumference, bone sutures being increasingly driven apart corresponding most likely to the hydrocephalus in our understanding, and 4th Type between the menings and the brain characterised by severe neurological deficit with lethal prognosis corresponding probably to all pathologies which were accompanied by an excessive increase of the intracranial pressure with a bulging fontanel. Due to the lack of autopsies in ancient times, the hydrocephalus was never linked to the pathology of the ventricles. All forms of hydrocephalus were believed to be caused by improper handling of the head by the midwife during delivery. Only the extracranial fluid collections, but not hydrocephalus in our sense, were considered to be suitable for surgical treatment. The surgery consisted in one or more incisions and evacuation of the fluid. The wound was not closed but let open for three days. Thereafter plasters or sutures closed the incisions. The surgical technique goes back probably to Antyllos a surgeon from the 3rd century AD whose considerations were cited in the work of Oreibasios. The early Arabic physicians took over the surgical indications, the operative technique and modified the Greek concept of hydrocephalus. Avicenna separated the traumatic haematomas outside the skull from the term hydrocephalus. However Avicenna, as all previous authors, had not linked hydrocephalus with the ventricular system. The autopsy of a child with an exorbitant hydrocephalus performed by the anatomist Vesalius in the 16th century revealed as a single pathology an extremely dilative ventricular system filled with water-like fluid which made it necessary to change completely the ancient concept of hydrocephalus.

Vault reduction cranioplasty for extreme hydrocephalic macrocephaly.

Due to early diagnosis and treatment of hydrocephalus, neurosurgeons rarely are called upon to treat patients with extreme hydrocephalic macrocephaly. Macrocephaly can limit mobility and hygiene. The critical evaluation and surgical correction of the morphological problem of macrocephaly secondary to hydrocephalus is complex. Various techniques such as quadrantal, picket fence, crossbar, and modified rr techniques have been used to reduce the size of the cranial vault to decrease cranial volume while achieving good cosmesis. Limitations of vault reduction cranioplasty include the inability to alter the anteroposterior and lateral diameters of the skull base, the inability to shorten the superior sagittal sinus, and the need to avoid infolding of the brain due to the risk of venous infarcts. Reduction cranioplasty is indicated in the occasional patient whose large head size represents a mechanical or cosmetic problem of sufficient magnitude to seriously interfere with motor development and functioning, with resultant development of pressure sores and difficulties with nursing care. Reduction cranioplasty should be avoided in patients under the age of 3 years.

In utero surgery for hydrocephalus.

INTRODUCTION: Neonatal hydrocephalus is one of the most common congenital anomalies affecting the nervous system. DISCUSSION: Currently, ultrasonography allows for early detection of fetal ventriculomegaly and presents the family with several treatment options: termination of pregnancy, early delivery and neonatal shunting, and delivery at term followed by shunting. Despite ventricular decompression after birth, the cognitive outcome is variable as prolonged in utero hydrocephalus has a detrimental effect. In the early 1980s, fetal intervention was explored with the intention of improving outcome. However, patient selection was poor. Fetal ventriculomegaly from other conditions was not adequately distinguished from fetal hydrocephalus. In addition, fetal surgical techniques were not advanced. Consequently, the results were poor and a de facto moratorium on fetal shunting was imposed. However, recent improvements in fetal imaging, such as magnetic resonance imaging, and advances in fetal surgical techniques offer the possibility that properly selected fetuses with hydrocephalus can benefit from an in utero intervention.

A case of pituitary adenoma: Thomas Willis revisited.

Thomas Willis (1621-1675) was the founder of modern clinical neuroscience. His habit of taking detailed histories with acute clinical observation often accompanied by post mortem studies has left a body of work which still provokes thought and debate. While we were considering a case of pituitary tumour described by Willis, a similar case presented coincidentally at our hospital. The two cases are compared and contrasted.

The history of spina bifida, hydrocephalus, paraplegia, and incontinence.

This paper covers the history since ancient times of spina bifida (SB) and its main associated conditions, viz., hydrocephalus, paraplegia and incontinence. Particular stress has been placed on the ancient authors who recognised these conditions. The article concludes with the history of some general aspects of SB and the dilemmas in its treatment.

History of hydrocephalus and its treatments.

Hydrocephalus has amazed and challenged clinicians throughout the history of medicine. In reviewing the treatment of hydrocephalus, the integral relationship between basic science and therapy is reaffirmed. As we embark into a new millennium, it is appropriate to reflect on the past studies of this disorder, review various attempted and currently used, and finally speculate on possible future directions in its treatment.

[Arne Thorkildsen and Thorkildsen's surgery]. / Arne Torkildsen og Torkildsens operasjon.

Arne Torkildsen (1899-1968) studied neurosurgery for one year at Queen Square and for four years (1930-34) with Wilder Penfield in Montreal. He returned to Oslo, Norway, in 1935 and took up responsibility for the neurosurgical section of the Department of Neurology of the National Hospital of Norway. In 1939, he introduced a new technique for operative treatment of hydrocephalus (ventriculocisternostomy, Torkildsen's shunt). His technique was the first successful procedure for shunting of cerebrospinal fluid and soon became internationally known and accepted as the standard operation for obstruction of the aqueduct or posterior third ventricle. In 1950, he defended his doctoral thesis at the University of Copenhagen, describing the technique and the results of the operation. He was professor of neurosurgery at the University of Cairo, Egypt 1951-1954.

The scientific history of hydrocephalus and its treatment.

Hydrocephalus cases were regularly described by Hippocrates, Galen, and early and medieval Arabian physicians, who believed that this disease was caused by an extracerebral accumulation of water. Operative procedures used in ancient times are neither proven by skull findings today nor clearly reported in the literature. Evacuation of superficial intracranial fluid in hydrocephalic children was first described in detail in the tenth century by Abulkassim Al Zahrawi. In 1744, LeCat published findings on a ventricular puncture. Effective therapy required aseptic surgery as well as pathophysiological knowledge--both unavailable before the late nineteenth century. In 1881, a few years after the landmark study of Key and Retzius, Wernicke inaugurated sterile ventricular puncture and external CSF drainage. These were followed in 1891 by serial lumbar punctures (Quincke) and, in 1893, by the first permanent ventriculo-subarachnoid-subgaleal shunt (Mikulicz), which was simultaneously a ventriculostomy and a drainage into an extrathecal low pressure compartment. Between 1898 and 1925, lumboperitoneal, and ventriculoperitoneal, -venous, -pleural, and -ureteral shunts were invented, but these had a high failure rate due to insufficient implant materials in most cases. Ventriculostomy without implants (Anton 1908), with implants, and plexus coagulation initially had a very high operative mortality and were seldom successful in the long term, but gradually improved over the next decades. In 1949, Nulsen and Spitz implanted a shunt successfully into the caval vein with a ball valve. Between 1955 and 1960, four independent groups invented distal slit, proximal slit, and diaphragm valves almost simultaneously. Around 1960, the combined invention of artificial valves and silicone led to a worldwide therapeutic breakthrough. After the first generation of simple differential pressure valves, which are unable to drain physiologically in all body positions, a second generation of adjustable, autoregulating, antisiphon, and gravitational valves was developed, but their use is limited due to economical restrictions and still unsolved technical problems. At the moment, at least 127 different designs are available, with historical models and prototypes bringing the number to 190 valves, but most of these are only clones. In the 1990s, there has been a renaissance of endoscopic ventriculostomy, which is widely accepted as the method of first choice in adult patients with aquired or late-onset, occlusive hydrocephalus; in other cases the preference remains controversial. Both new methods, the second generation of valves as well as ventriculostomy, show massive deficits in evaluation. There is only one randomized study and no long-term evaluation.

Hidrocefalia. Conceito, aspectos históricos, fisiopatologia, classificaçäo e evoluçäo do tratamento / Hydrocephalus - Concept, historical aspects, phisiopathology, classification and evolution of the treatment

Os autores fazem uma revisão sobre o conceito de hidrocefalia e abordam o interesse histórico que essa patologia tem ocasionado desde os escritos de Hipócrates até a realidade atual. A sua fisiopatologia e classificação são revisadas bem como a evolução do tratamento. As dificuldades que as derivações valvulares ainda apresentam nos dias atuais são analisadas e por fim elaboram os fundamentos e tecem considerações históricas no que concerne à utilização da neuroendoscopia em geral e em particular o seu emprego no tratamento das hidrocefalias