0:00

So this is, uh, a patient of a physician, um,

0:05

son of a physician.

0:07

They wanted a simple quick test to diagnose.

0:12

They palpated something in the neck on the left side of this patient.

0:18

And, uh, they wanted a quick, quick answer. Can you see? Anyway,

0:23

so there was a palpable, uh, mass, mass like thing,

0:28

uh, in the left neck.

0:33

I know this is a soft tissue window, but let's see.

0:39

So they wanted to do mri. I was reluctant to do mri. Now, um,

0:44

for the purposes of information,

0:48

it's very tricky to get these patients under sedation.

0:51

There is a paper published in 2018,

0:54

which said that even minor sedation may have long-term effects. Uh, for example,

0:59

if a patient has an IQ of one 30 under sedation after

1:04

sedation, they can actually lose their IQ points by two to four.

1:08

So if you think about that in every bit, every sedation,

1:12

if a kid has a potential to lose one to two points, that is a lot of problem.

1:17

And especially the bright kits, this is extremely bright kit otherwise.

1:22

So I thought, okay, how about we do a low dose ct?

1:25

We will put some contrast to rule out any enhancing lesion, um, because it,

1:30

it is a hard, hard swelling.

1:33

We can get some answer if there is any problem with the bone.

1:38

And guess what we found?

1:43

This is an abnormality. This is the abnormal area.

1:46

You can see there is an abnormal shape to the chest and this is the right side,

1:51

which is a normal side.

1:55

And I have some recons here. That's the left side

2:03

for comparison. Same location on the right side.

2:07

This is the right side, which is normal.

2:10

Now we are rotating towards the right and this is the left side.

2:14

So can you please share the questions?

2:18

So here we are dealing with something, uh, which is potentially bony in nature.

2:23

So what would be the best possible diagnosis in this case?

2:32

So rib. So skeletal dysplasia is usually less.

2:37

Um, common if there is only like presentation of isolated, uh, rib anomaly,

2:42

skeletal dysplasia will usually present with multiple boney anomalies and you

2:47

have to actually image, uh, multiple bones.

2:51

So rib anomaly,

2:52

here it is rib synostosis or it's a partial fusion of the ribs.

2:57

Two ribs are abnormal

2:58

And broad, and uh,

3:00

those two ribs are actually partially bridged.

3:04

There's a partial fusion of those ribs. This is an extremely rare, uh,

3:08

anomaly seen in less than 0.5% of the patients.

3:13

Bi ribs are actually more common,

3:15

but actual ribs synostosis is relatively rare. Uh,

3:19

they're usually found on the right side. And in females,

3:22

our patient was a male and this happened on the left side, extremely uncommon.

3:27

Uh, they're usually found incidentally.

3:28

You can see when you're reading like hundreds of um, chest films,

3:31

you can see couple of, uh, um,

3:34

patients with incidental findings of rib anomaly. There may be hypoplastic ribs,

3:39

they can be accessory ribs or uh, there can be some partially fused ribs.

3:45

Um, there are three types of rib anomalies.

3:48

If there are aberrations in the number, they're like 11 and 13. Um,

3:53

or hypoplastic or cervical ribs, hypoplastic 12 ribs.

3:57

All these come under type one.

3:59

Type two rib anomalies are associated with segmentation errors including cost to

4:04

fusion and bridging like our patient and type three result in resegmentation.

4:08

Once the segmentation is done,

4:10

it more segmentation happens and that results in the bi ribs and

4:15

cervical ribs are the cause of usually, um, thoracic outlet syndrome.

4:19

In patients who are, um, young adults,

4:22

I've not seen a single patient with cervical rib, uh, with, uh,

4:27

thoracic outlet syndrome in less than 12 years of age. So this is, uh,

4:32

rib synostosis. They got the good news that there is no mass.

4:35

They were thinking about having sarcoma, they were thinking about neuroblastoma.

4:39

The, so all those things we are ruled out. And this is a rib anomaly.

4:44

The patient can live with this rib anomaly forever,

4:46

without any implications because it, this is first and second rib, it's,

4:51

and it is,

4:51

the synostosis is away from the brachial plexus or neurovascular bundle.

4:56

So they will never present with any kind of neurological or vascular problems.

5:01

Um, isolated rib anomaly, extremely rare,

5:04

particularly because it is on the left side and is less suspected. Also,

5:08

the patient was male, so this was rib synostosis.