The registrar's face was taking on a testy look. So enduring was the silence our furtive glances had developed a nystagmic quality. “Galactosaemia” came her peremptory reply. Right on queue the disjointed chorus of ahs and head nods did little to hide our mental whiteboard of differentials being wiped clean. At the time conjugated bilirubinaemia in children only meant one thing: biliary atresia. A fair assumption; we were sitting in one of three specialist centres in the country equipped to treat these patients. Ironically the condition has become the unwieldy yardstick I now measure the incidence of paediatric disease.
Biliary atresia is the most common surgical cause of neonatal jaundice with a reported incidence of 1 in 14-16ooo live births in the West. It is described as a progressive inflammatory obliteration of the extrahapatic bile duct. And Dr Charles West, the founder of Great Ormond Street Hospital, offers an eloquent description of the presenting triad of prolonged jaundice, pale acholic stools and dark yellow urine:
‘Case 18...It was born at full term, though small, apparently healthy. At 3 days however, it began to get yellow and at the end of 3 weeks was very yellow. Her motions at no time after the second day appeared natural on examination, but were white, like cream, and her urine was very high coloured.’
1855 was the year of Dr West's hospital note. An almost universally fatal diagnosis and it would remain so for the next 100 years. The time's primordial classification of biliary atresia afforded children with the 'noncorrectable' type, a complete absence of patent extrahepatic bile duct, an unfortunate label; they were beyond saving. Having discovered the extent of disease at laparatomy, the surgeons would normally close the wound. The venerable Harvardian surgeon, Robert E. Gross saved an enigmatic observation: “In most instances death followed a downhill course…”
K-A-S-A-I read the ward’s board. It was scrawled under half the children's names. I dismissed it as just another devilishly hard acronym to forget. The thought of an eponymous procedure had escaped me and in biliary atresia circles, it's the name everyone should know: Dr Morio Kasai.
Originating from Aomori prefecture, Honshu, Japan, Dr Kasai graduated from the National Tohoku University School of Medicine in 1947. His ascension was rapid, having joined the 2nd department of Surgery as a general surgeon, he would assume the role of Assistant Professor in 1953. The department, under the tenure of Professor Shigetsugu Katsura, shared a healthy interest in research.
1955 was the landmark year. Katsura and Kasai operated on their first case: a 72 day old infant. Due to bleeding at the incised porta hepatis, Katsura is said to have 'placed' the duodenum over the site in order to staunch the flow. She made a spectacular postoperative recovery, the jaundice had faded and there was bile pigment in her stool. During the second case, Katsura elected to join the unopened duodenum to the porta hepatis. Sadly the patient's jaundice did not recover, but the post-mortem conducted by Kasai confirmed the development of a spontaneous internal biliary fistula connecting the internal hepatic ducts to the duodenum. Histological inspection of removed extrahepatic duct showed the existence of microscopic biliary channels, hundreds of microns in diameter. Kasai made a pivotal assertion: the transection of the fibrous cord of the obliterated duct must contain these channels before anastomosis with the jejunal limb Roux-en-Y loop. This would ensure communication between the porta hepatis and the intrahepatic biliary system. The operation, entitled hepatic portoenterostomy, was first performed as a planned procedure for the third case at Tohoku. Bile flow was restored and Kasai published the details of the new technique in the Japanese journal Shujutsu in 1959. However, news of this development did not dawn on the West until 1968 in the Journal of Pediatric Surgery. The success of the operation and its refined iterations were eventually recognized and adopted in the 1970s. The operation was and is not without its dangers. Cholangitis, portal hypertension, malnutrition and hepatopulmonary syndrome are the cardinal complications. While diagnosing and operating early (<8 weeks) are essential to the outcome, antibiotic prophylaxis and nutritional support are invaluable prognostic factors.
Post operatively, the early clearance of jaundice (within 3 months) and absence of liver cirrhosis on biopsy, are promising signs. At UK centres the survival after a successful procedure is 80%. The concurrent development of liver transplantation boosts this percentage to 90%. Among children, biliary atresia is the commonest indication for transplantation; by five years post-Kasai, 45% will have undergone the procedure.
On the 6th December 2008, Dr Kasai passed away. He was 86 years old and had been battling the complications of a stroke he suffered in 1999. His contemporaries and disciples paint a humble and colourful character. A keen skier and mountaineer, Dr Kasai lead the Tohoku University mountain-climbing team to the top of the Nyainquntanglha Mountains, the highest peaks of the Tibetan highlands. It was the first successful expedition of its kind in the world. He carried through this pioneering spirit into his professional life. Paediatric surgery was not a recognized specialty in Japan. By founding and chairing multiple associations including the Japanese Society of Pediatric Surgeons, Dr Kasai gave his specialty and biliary atresia, the attention it deserved.
Despite numerous accolades of international acclaim for his contributions to paediatric surgery, Dr Kasai insisted his department refer to his operation as the hepatic portoenterostomy; the rest of the world paid its originator the respect of calling it the ‘Kasia’. Upon completion of their training, he would give each of his surgeons a hand-written form of the word ‘Soshin’ [simple mind], as he believed a modest surgeon was a good one.
At 5 foot 2, Kasai cut a more diminutive figure one might expect for an Emeritus Professor and Hospital Director of a university hospital. During the course of his lifetime he had developed the procedure and lived to see its fruition. The Kasia remains the gold standard treatment for biliary atresia; it has been the shinning light for what Willis J. Potts called the darkest chapter in paediatric surgery. It earned Dr Kasai an affectionate but apt name among his peers, the small giant.
Miyano T. Morio Kasai, MD, 1922–2008. Pediatr Surg Int. 2009;25(4):307–308.
Garcia A V, Cowles RA, Kato T, Hardy MA. Morio Kasai: a remarkable impact beyond the Kasai procedure. J Pediatr Surg. 2012;47(5):1023–1027.
Mowat AP. Biliary atresia into the 21st century: A historical perspective. Hepatology. 1996;23(6):1693–1695.
Ohi R. A history of the Kasai operation: Hepatic portoenterostomy for biliary atresia. World J Surg. 1988;12(6):871–874.
Ohi R. Morio Kasai, MD 1922-2008. J Pediatr Surg. 2009;44(3):481–482.
Lewis N, Millar A. Biliary atresia. Surg. 2007;25(7):291–294.
This blog post is a reproduction of an article published in the Medical Student Newspaper, April 2014 issue.
I have recently spent a few days following around registrars on military ward rounds. It has been a fantastic experience for learning about trauma care and rehab, but more importantly it has shown me just how vital team spirit is to modern health care! The military ward round is done once a week. It starts with a huge MDT of almost 40 people, including nurses, physios, registrars and consultants from all of the specialities involved in trauma and rehab. The main trauma ward round team then go to speak to all of the patients in the hospital. The team normally consists of at least one T+O consultant, one plastics, two physios, two nurses, 3 registrars and a few others. This ward round team is huge, unweildly and probably very costly, but those military patients receive a phenomenal level of care that is very quick and efficient.
Having then compared this level of care with what I have experience on my 4th year speciality medicine placement, I now feel the NHS has a lot to learn about team work.
I am sure that everyone working in healthcare can relate to situations where patients have been admitted under the care of one team, who don’t really know what to do with the patient but struggle on bravely until they are really lost and then look around to see who they can beg for help. The patient then gets ping-ponged around for a few days while management plans are made separately. All of the junior doctors are stressed because they keep having to contact multiple teams to ask what should be done next. The patient is left feeling that their care wasn’t handled very well and is probably less than happy with the delay to their definite treatment. The patient, thankfully, normally ends up getting the correct treatment eventually, but there is often a massive prolongation of their stay in hospital. These prolonged stays are not good for the patient due to increasing risks of complications, side effects, hospital acquired infections etc. They are not good for the health care staff, who get stressed that their patients aren’t receiving the optimum care. The delays are very bad for the NHS managers, who might miss targerts, lose funding and have to juggle beds even more than normal. Finally, it is not good for the NHS as a hole, which has to stump up the very expensive fees these delays cause (approximately £500 a night).
There is a simple solution to this which would save a huge amount of time, energy and money. TEAM WORK!
Every upper-GI ward round should be done with the consultant surgeon team and a gastroenterologist (even a trainee would probably do) and vice versa, every Gastroenterology ward round should have a surgeon attached.
Every orthopaedic ward round should be done with an elderly care physician, physio/rehab specialist and a social worker.
Every diabetic foot clinic should have a diabetologist, podiatrist, vascular surgeon and/or orthopaedic surgeon (even trainees).
Etc. etc. etc.
A more multi-disciplinary team approach will make patient care quicker, more appropriate and less stressful for everyone involved. It would benefit the patients, the staff and the NHS. To begin with it might not seem like an easy situation to arrange. Everyone is over worked, no one has free time, no one has much of a spare budget and everyone has an ego. But...
Team work will be essential to improving the NHS. Many MDTs already exist as meetings. MDTs already exist as ED trauma teams, ED resus teams and Military trauma teams. There is no reason why lessons can’t be learnt from these examples and applied to every other field of medicine.
I know that as medical students (and probably every other health care student) the theory of how MDTs should work is rammed down our throats time after time, but I personally still think the NHS has a long way to go to live up to the whole team work ethos and that we as the younger, idealist generation of future healthcare professionals should make this one of our key aims for our future careers. When we finally become senior health care professionals we should try our best to make all clinical encounters an MDT approach.
In January 2012 I wrote about a girl who had created a Facebook page because she urgently needed a liver.
In August 2004 I had a car accident in Germany, where damaged my limbs and some of my internal organs. That's why I need a liver URGENTLY!
Over 26.000 people (family members, doctors, nurses, her friends and students from all parts of the country) followed and liked her page in 3 months. Finally she'd found a suitable liver, and she is fine now.
I believe that our generation of health care professionals should be prepared for this and should provide meaningful help, because in the future we can not avoid patients who are interested in social media. E-patients will increasingly use web 2.0 tools as part of their health management and we must respond to that.
Can you imagine being able to search for locations in the human body in the same way you can on Google Maps? This thinking lead Programmer Rich Stoner to create this amazing video of a 21st Century Map of the Brain which was our most popular tweet last week.
A 21st Century Map of the Brain http://t.co/tkojzJBW55 via @brianglanz #openscience— Meducation (@Meducation) October 18, 2013
Rich writes in his blog - “Now we can quickly search Google Maps for a location, ask what is nearby, and even see what it looks like using StreetView. Now, imagine if something like that existed for the human brain: an interactive environment to search, visualize, and explore layers upon layers of neuroanatomy. This is the dream of cortical cartographers (also known as neuroanatomists). 10 years ago, one of the largest brain mapping initiatives was founded by Paul Allen with a single goal: to build a 21st Century Map of the Brain.” Click here to read more.
The mapping of the brain is a working progress and therefore not 100% accurate. Even so the video gives us an insight into the innovate ways we will be able to interact with science in the future.
You can follow Meducation on twitter to see more tweets like this at twitter.com/meducation.