Congenital Pseudoarthrosis of Tibia

 Assalamualaikum,


I just wanna share the story of 17 years old Malay boy who has the condition called Congenital Pseudoarthrosis of the Tibia (CPT).  He is currently in my ward after had a surgery done on his right leg.


He was diagnosed to have this rare condition since childhood. The problem of this condition is that a segment of the tibia (shin bone) is abnormal. The segment composed of fibrous tissue and not the normal bone that we all have, in other words, the segment is weak, fragile and easily broken.


The bone was broken many times before and he was managed in many hospitals and undergone multiple surgeries. Unfortunately, none of the surgeries were successful. The most probable explanation for the failures is because the abnormal segment was not completely excised intraoperatively.



When he presented to us, he has multiple cafe-au-lait spots, normal spine, no ligamentous laxity and normal vision. His IQ was low (mentally challenged). His affected leg was crooked and bent and we can easily feel the edge of the affected bony segment. It was painless though. He has 20 cm shortening and he walks by using specially modified shoes (by his father). His father glued a 20cm rubber-construct over the sole of his right shoe to compensate for the shortening...very clever. I should have taken a picture of his shoe...&#^*&

Multiple Cafe-au-lait spots

So, after discussing with the patient and his parents regarding the pro and cons of the surgery, they agreed to have the surgery. We have excised the affected the whole abnormal segment (remove the abnormal part), plate the tibia (meaning bring the bone ends together), put a bone graft and to a certain degree, correct the alignment. It looks much better after the surgery...but the leg is still 10cm short, haha. Oh no, what should we do now?

The view from the side

Top view before the surgery

Don't worry, we are planning for the second stage surgery later, to lengthen the leg. Hopefully everything goes well for him, insyaAllah.

After the surgery, much better looking now, right?

 

 

 

That's all for now...I will share more cases as they pop-up.Wassalam.