Dr Child's Casebook: Gut Feelings
The fourth instalment of Dr Child’s Casebook sees our Medical Director encounter an unusual ramification of Marfan syndrome: serious, long-standing irritable bowel syndrome which cannot be controlled with diet and medication.
Q: My 21-year-old son has been offered bowel surgery to remove his large bowel. Do other Marfan patients have this problem and how do they handle it?
A: So sorry to hear of your son's problems, but he is in the very best hospital to deal with them. Although irritable bowel syndrome is more frequent in Marfan (constipation predominant) I have never heard of a patient yet who needed a colostomy. I understand you have tried diet and medication to no avail and this condition has left him thin and weak. He is 6 foot 5, and used to weigh 9 stone at age 15 but now weighs 44 kg (6.9 stone), and is being fed by nasogastric tube. He wants his life back. He should weigh in the 25th percentile for young men of his height.
Regarding surgery, I would suggest:
1) careful tying/cautery of all bleeding points, to avoid reoperation
2) leaving the stitches in longer than for ordinary patients because of possible delay in wound healing
3) cover with antibiotics as long as stitches are in place to prevent wound Infection
4) feeding throughout because he has no reserves
5) that the services of an experienced anaesthetist are required because neck extension is limited due to high-level scoliosis involving the neck, and because high arched palate and narrow trachea make intubation difficult. Also, they must be ready for cardiac arrhythmia during intubation and surgery - cardiac anaesthetist if available please.
I have asked my secretary to send you our chapter on GI symptoms in MFS. Offered in all humility since you are with experts. Ehlers Danlos and MFS are very similar.
For more information please refer to our comprehensive leaflet on Bowels and Marfan Syndrome.