Marfan syndrome is the correct diagnosis - Emergency surgery
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Dr. Yash Patel
Marfan syndrome is the correct diagnosis - Emergency surgery followed many missed chances to diagnose illness
A CT scan showed that Songer had suffered an extensive acute aortic dissection. Dissections can be caused by uncontrolled hypertension, a genetic predisposition or an inherited disorder such as Marfan syndrome. Treatment usually involves emergency surgery to repair or replace the aorta; the mortality rate within the first 48 hours is 50 percent. After meeting Songer and reviewing his CT scan, doctors were fairly certain that the dissection was the result of Marfan syndrome: Songer’s aortic root had the characteristic pear-shaped appearance seen in such patients, and he had the requisite body type: tall with spider like fingers and a narrow face. Marfan patients are given medicines to drive down their blood pressure; Songer monitors his several times a day.

The question was posted earlier -
As a child, Kevin Songer was unusually tall and skinny, with severe buck teeth and weak wrists and ankles that were susceptible to repeated sprains. At age 5 he underwent surgery for a double inguinal hernia. For many people, hernia repair is a one-time fix; in Songer’s case, it would recur repeatedly as he got older. As he got older, doctors never offered an explanation for his frequently sprained wrists and ankles or painful lower back. In 1995, when he was 38, Songer underwent the second of six hernia surgeries after his abdominal tissue inexplicably tore. Twelve years later, during his third hernia operation, the surgeon noted his lack of abdominal muscle. He chided Songer about his diet and advised him to eat more protein, which builds muscle and to lay off carbohydrates. After his sixth hernia surgery in 2009, his doctor told him that losing weight would prevent future hernias. At 6-4 and 228 pounds, he was overweight but not obese. That same year, Songer’s blood pressure hit 140/90, the threshold for hypertension. His doctor put him on medicine to lower it. Sometimes when he was working strenuously — he experienced a tearing sensation in his neck that would last a few seconds, followed by a brief, intense headache that also disappeared quickly. Songer, then 54, was alone in his home, after a long day at work when he suddenly felt a searing pain, like something had hit him on the back of the head with a sledgehammer — that radiated through his jaw, down his back and into his legs. After a few minutes, he felt worse. If he didn’t get off his bed and to the nearest hospital, his wife would probably find him dead. Can you tell what was the cause?

https://www.washingtonpost.com/national/health-science/emergency-surgery-followed-many-missed-chances-to-diagnose-illness/2013/08/12/9d39f74c-f4a7-11e2-9434-60440856fadf_story.html?utm_term=.9f8f4b1c20af
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Dr. S●●●●m PK
Dr. S●●●●m PK Psychiatry
Interesting case description.informative
Jun 13, 2017Like