April 1st 1856
On 1st April in 1856 many people flocked to The Tower of London to witness the lions being washed. Yes, I said “lions”. Tickets were issued with the seal of the Tower’s Senior Warden: “entry by The White Gate”. Only neither lions nor white gate existed – it was an April Fool’s joke.
Elsewhere in London, on the same day, at a meeting of The Pathological Society of London, Dr John Syer Bristowe, physician at St Thomas’s Hospital, was exhibiting a pathology specimen entitled
“Cystic Disease of the Liver associated with similar Disease of the Kidneys”. He described it as “a rare form of affection” and commented
“I am inclined to think that the co-existence of the same form of disease in the liver and kidneys was merely a coincidence.”
Two weeks later, a Dr Wilks, inspired by Bristowe’s Liver, presented a similar specimen that had been “on the shelves of the museum at Guys” for some time. The cysts were “crowded together in immense numbers” from walnut-size to egg-size (everything was described in relation to food – Bristowe himself used “Spanish chestnut” and “pigeon’s egg”; today we use centimetres!)
Bristowe came from a medical family, qualified after just 3 years at medical school and walked away with numerous prizes. By 1856 he was lecturing in Botany (I am so glad botany isn’t on the medical curriculum today), Anatomy and Pathology. He also wrote poetry, some of which was published – which doesn’t make it actually readable, but if you want a challenge they have been made available by the Hathitrust.
By the age of 33 he was a full physician and had a retinue of eager medical students following him daily. Some years later he wrote “The Theory and Practice of Medicine“, one of the most comprehensive medical text books of the time. It is rather sad, however, that he never had his name attached to any particular pathology or disease. Perhaps we should posthumously attribute polycystic liver to him : Bristowe’s Cystic Disease of the Liver.
John Syer Bristowe died in 1895, leaving 10 children, at least one of whom followed him into medicine. His daughter, Beatrice, painted a portrait of him to hang in St Thomas’s Hospital, London.
Now, back to this cystic liver.
It seems strange that liver cysts associated with kidney cysts had not previously been described in detail. As far back as 1654 a text was published “Anatomia Hepatis “ by Glisson, a physician, the result of 10 years of study of pathological specimens of the liver. Of course Glisson did not have the benefit of a microscope (1590s) and histology had not been discovered, but liver cysts that people with ADPKD have are certainly large enough to be seen by the naked eye. While I haven’t read this 1654 text, it is likely Bristowe did and so if such liver cysts had been previously described then Bristowe would not have been so interested in the patient from 1856. This patient was a 53 year old shoemaker with a short 10 week history of right upper quadrant pain associated with blood in the urine – probably recognisable by ADPKD patients as a burst renal cyst with subsequent haematuria. It is not at all clear why he died or whether it related in any way to the cysts in his kidneys or liver. Bristowe’s examination of his body did not reveal any other significant abnormalities.
Bristowe described that the liver cysts consisted of smaller ones near the centre of the organ and larger ones that coalesced together towards the surface of the liver. The liver was enlarged but the liver tissue itself appeared normal, just distended by the presence of the cysts. The kidneys, on the other hand, seemed to be replaced by the cysts and little recognisable renal tissue remained. This is exactly what is seen today in PLD associate with PKD. So while the kidney cysts lead to loss of normal function, liver cysts tend not to affect the liver’s performance.
It isn’t easy to track the knowledge base for polycystic livers from 1856 onwards.
In 1906 a paper by Moschowitz claimed there were just 85 cases of combined liver and kidney cyst reported in the medical literature.
Then in 1929 Oppenheimer published a case report of polycystic liver with polycystic kidneys.
He described symptoms attributable to declining renal function but with retention of normal liver function. This was probably ADPKD with PLD.
In 1932 a paper was published describing a familial PKD associated with liver cysts in children. This is likely to have been the autosomal recessive PKD (ARPKD) that presents in children and where liver abnormalities are common and where liver function may be compromised.
Jumping forward to 1985, CT scans had become a mainstream diagnostic tool and Levine published a retrospective analysis of CT scans on ADPKD patients, reporting some 57% patients having liver cysts (31% had large cysts, 25% had multiple small ones). However this study begins with the statement that liver cysts in ADPKD are of little clinical significance.
Thirty years further on and the picture has changed again – while the incidence of liver cysts with ADPKD probably remains unaltered, better detection methods make it appear more common. it is also now acknowledged that the clinical impact of liver cysts can be for some patients as prominent as the impact of renal cysts.
Also, we no longer have an Annual Washing of the Lions at The Tower of London as an April Fool’s prank.