Anyone ever dealt with a "Nasal Dermoid"?

[Vegas_Dave]

My 9 month old son has had a small bump on his nose since birth. The original pediatrition for the first 7 months of his life was not at all concerned about it and said "we will watch it". Well, I got fed up with that pediatrition and went to another doctor for a second opinion.

The new doctors were Very concerned with it. It has gotten bigger as he has grown. They sent us to a plastic surgeon for an evaluation.

This particular PS deals alot with children as he volunteers his services at the local Special Needs clinic for various birth defects. He also comes VERY highly recommended by 5 out of 5 doctors that we talked to.

He walked in and within about 3 seconds called it a "Nasal Dermoid". It has something to do with some sort of tissue that in developement in the womb is supposed to receed back into the skull. There are 2 possibilities:

1. It is just a left over remenant. This is removed with about a 2 hour surgery and 1 day in the hospital.

2. In 20% of the cases, this material is actually still connected to the brain and would require Neuro-Surgery (on my baby) consisting of about a 5-6 hour surgery and at least 5 days in the hospital.

We have to do an MRI to find out which one it is.

My wife and I are both taking this pretty hard and cannot find anywhere near as much information as we would like to about it. So if anyone has any experience with this, I would appreciate anything you can tell me.

[Braincase]

(Prayers)

I don't know much, but I'm offering up the best I can.

[Simplex3]

My wife and I were told all kinds of crap about what *might* be wrong with our first kid because of the results of that craptacular "triple test". I finally convinced my wife to look at it this way:

You've done what you can. You got the right doctors. Now, if it's nothing, then stressing out won't help any of you. If something is wrong, you will take care of it. Once again, stressing out won't help either of you and it most certainly won't help the kid. The last thing your kid needs is to see you spazing out over something, they will pick up on that.

As for info on something this rare, call the plastic surgeon's office. Ask them if they have any literature or know where you can get any. Sounds like you guys are mostly worried about potential side effects/mortality rates, I'm sure he's got that stuff. As far as I'm concerned that's part of what I'm paying them for.

Most of all, keep your chin up. It will be just fine.

[Phobia]

I don't have any experience with that, but I do have experience with a wife who got unbelievably stressed due to worst case scenarios involving a couple issues with our kid (she was a premie and wore a helmet to correct a skull flattening for about 6 months). You can't stress over those things until the tests are run. If it comes back as Neuro-surgery required, stress all you want to but the odds are significantly in your favor. Don't sweat it. Pediatrics know their stuff these days. Plus, if anybody makes a mistake, you have the added benefit of knowing Gil Grisham will be there to find the damn culprits.

[Baby Lee]

Have they explained the urgency if there's no cerebral involvement? Or couldn't this be postponed until he has developed a little more?

[Vegas_Dave]

Well, we go in for the MRI tomorrow morning and then a consulation with the Plastic Surgeon on Thursday with the operation possibly the following Thursday or Friday...

[Braincase]

Well, we go in for the MRI tomorrow morning and then a consulation with the Plastic Surgeon on Thursday with the operation possibly the following Thursday or Friday...

(Prayers)
Hope everything goes OK, Dave.

[DanT]

Hey Vegas_Dave,
Here's the abstract and some other excerpts from a 30-year review of nasal dermoid cases at the Children's Hospital in Boston. It looks like it's a rare condition; you need to do imaging studies (e.g. CT scans, MRI) to help decide the best surgical approach, and the only treatment involves surgery. Once it is dealt with, it's unlikely to pose any new problems.:

The Presentation and Management of Nasal Dermoid
A 30-Year Experience

Reza Rahbar, DMD, MD; Prerak Shah, MD; John B. Mulliken, MD; Caroline D. Robson, MD; Antonio R. Perez-Atayde, MD; Mark R. Proctor, MD; Margaret A. Kenna, MD; Michael R. Scott, MD; Trevor J. McGill, MD; Gerald B. Healy, MD


Arch Otolaryngol Head Neck Surg. 2003;129:464-471.


ABSTRACT

Objective: To review the presentation of nasal dermoid in children and present guidelines for its management.

Design: Retrospective study (January 1, 1970, through December 31, 2000).

Setting: Tertiary-care pediatric medical center.

Patients: Number of patients: 42 (28 boys and 14 girls).

Intervention: Extensive review of the initial presentation, significant family and medical history, workup, surgical approach, complication, and rate of recurrence.

Results: Mean age of presentation was 32 months. The most common presentation was a nasoglabellar mass, in 13 patients (31%). Five patients presented with an associated craniofacial abnormality. Thirty-nine patients (93%) underwent a preoperative imaging workup. Thirty-one (74%) did not show any clinical and/or radiographic indication of intracranial extension. Thirty-four (81%) underwent extracranial excision, and 8 (19%) underwent combined intracranial-extracranial excision. Five patients (12%) presented with recurrence, extracranially in 4 and intracranially in 1. No other complication was noted, with a mean follow-up of 7 years.

Conclusions: Nasal dermoid is a rare congenital anomaly. Preoperative evaluation is essential to rule out intracranial extension. Surgical strategy depends on the location and extent of the lesion, ranging from local excision to a combined intracranial-extracranial approach. Recurrence is uncommon and often easily managed.



INTRODUCTION


NASAL DERMOID is a rare developmental anomaly. Unlike other craniofacial dermoids, the nasal lesions can present as a cyst, a sinus, or a fistula and may have an intracranial extension.(1) The incidence is estimated at 1:20 000 to 1:40 000 births.(2-3) Pathogenesis involves the incomplete obliteration of neuroectoderm in the developing frontonasal region.(2, 4) Progressive enlargement of a nasal dermoid can cause soft tissue and skeletal deformity, local infection, meningitis, and brain abscess. Timely diagnosis is essential, and surgical excision is the only therapeutic modality.

The purpose of this study is to review a 30-year experience in the management of nasal dermoids at The Children's Hospital, Boston with emphasis on the presentation, role of preoperative imaging, surgical approach, and rate of recurrence. On the basis of our findings, we present a treatment algorithm for the management of nasal dermoid.

...

[Excerpt from Section on Surgery]

Our surgical experience at The Children's Hospital, Boston has been large and diverse. Thirty-one patients underwent extracranial excision with no clinical and/or radiographic evidence of intracranial extension. None of these patients showed any evidence of intracranial extension intraoperatively. Three patients (18, 23, and 29) presented with preoperative imaging findings suggestive of intracranial extension of the sinus tract without a definite intracranial component. All 3 patients underwent an extracranial excision only because there was no intraoperative indication of intracranial extension, and biopsy of the tract suggested a fibrous tissue without any dermal component. Eight patients presented with nasal dermoid and intracranial extension, based on preoperative CT and/or MRI findings. They underwent combined intracranial-extracranial excision, which confirmed an intracranial-extradural dermoid in all patients. We have had a total of 5 recurrences (12%) with a mean follow-up of 7.5 years (range, 1-15 years). Four patients (25, 28, 31, and 36) presented with recurrence extracranially, and 1 patient (16), intracranially. The mean time of recurrence was 3.6 years (range, 1-6 years).

Failure to diagnose and resect nasal dermoid properly can result in progressive enlargement, skeletal distortion, infection, meningitis, and intracranial abscess. We advocate a complete evaluation and neurosurgical consultation if any clinical or radiographic indication of intracranial involvement is found. We recommend fine-cut CT with axial and coronal planes through the nose, skull base, and cranium using bone and soft tissue algorithms to determine the extension and possible intracranial involvement. Because of the limitations of CT assessment of soft tissue at the skull base and the reported false-positive findings of scans in the past, complementary multiplanar, high-resolution MRI is strongly recommended (Figure 6).




The timing of resection for an isolated nasal dermoid without intracranial complication remains controversial. We advocate early intervention to prevent the potential risk for infection and the possible need for a more extensive procedure. We agree with Pollack (29) that the surgical approach should fulfill the following 4 criteria: (1) provide excellent access to a midline cyst; (2) allow access to the base of skull; (3) provide adequate exposure for reconstruction of the nasal dorsum; and (4) result in an acceptable scar. An external rhinoplasty approach has a well-concealed scar with an excellent cosmetic outcome. It provides a wide exposure for nasal osteotomy and allows easy access to follow the sinus tract to the skull base. We have used this approach in 3 of our patients without any difficulty. For a lesion in the nasal-glabellar area without a sinus opening that might not be accessible via an external rhinoplasty approach, we prefer a paracanthal incision halfway between the inner canthus and the bridge of the nose or a bicoronal approach. When there is a dorsal ostium, a vertical elliptical incision is essential for removal. However, we agree with Bradley (18) and Denoyelle et al(10) that a poor aesthetic result and widening of the scar can occur after medial vertical excision specifically in the bony dorsal region. We also agree with Sessions,(1) Pensler et al,(6) and Bartlett et al(17) that craniotomy can be avoided if there is a fibrous tract at the cranial base without any evidence, based on intraoperative biopsy findings, of a dermal component. However, it is important to underscore that at present, no study can confirm that the epidermal and adnexal structures extend along the entire sinus tract or are discontinuous as the tract crosses the cranial base. When there is radiographic evidence of intracranial extension, we recommend direct excision by means of a coronal approach, with additional nasal incision if a sinus ostium is present. First, the external cyst or sinus is excised, and then the stalk is followed to the cranial base, followed by a formal craniotomy and removal of the intracranial component (Figure 6).

The recurrence rate of nasal dermoid is low, but may occur several years after the initial surgery. Therefore, the long-term follow-up of all patients with a history of nasal dermoid is essential.


CONCLUSIONS


Nasal dermoid is a rare congenital lesion, which often poses diagnostic and surgical dilemmas. Preoperative evaluation is essential to rule out intracranial extension. Workup should include fine-cut CT, and complementary MRI should be considered if there is concern for intracranial extension. Surgical management is dependent on the location and extent of the lesion, ranging from local excision to a combined intracranial-extracranial approach.


http://archotol.ama-assn.org/cgi/content/full/129/4/464 (you'll probably need to be at a University or Hospital computer in order to access this URL)

[DanT]

Sounds like you found a damn good Doctor, there, Vegas Dave. :thumb:

I'll hold your family in my prayers.

[NJ Chief Fan]

thats just awful...i hope your child doesnt have any major problems resulting from this and i hope that his treatment goes well

my prayers go out to your family and hold in there things will be alright

[R&GHomer]

I'm sure things will be fine. I'll say a prayer for your child and family. Keep the faith

[6 Iron]

Dave,

You have gotten past the first big hurdle with this problem, and that is finding doctors that recognize the significance of the lesion, and the possibility of extension into the skull. I hope that the MRI gives them all the information they need, and that the surgery goes very well. Good luck and my prayers are with your family.

[Vegas_Dave]

Thanks guys. They will be giving me the results of the MRI while I am in the office (doctor doesnt trust the local courier services).

I do believe that we have found an excellent doctor. My wife is a treatment coordinator for a local orthodontist. He does a lot of free work on children at the "special needs" clinic where this plastic surgeon does as well. This surgeon came highly recommended by 4 of 4 doctors, all in different fields, that I talked to. In fact, they all brought up his name before I had a chance to. So I know that we are in good hands.

We are hoping and praying that everything is by the book and does not have the intracranial extension. Personally, I cant stand the thought of my 10 month old little angel going in for neurosurgery... but if its what is needed, then we must do it.

I will keep you all posted and we appreciate the support and prayers.

[Vegas_Dave]

So we had the MRI on Wed and the appointment with the surgeon on Thurs.

Luckily, the dermoid is NOT attached to the brain .

This means that it will be the "easier" surgery that will last about 2 hours and take 1 night in the hospital.

However, now I get to deal with the stress of the surgery on my mind. At least before this, I only had the stress of finding out what was going on. Now that I know, the surgery is up on deck.

Dont yet know when the surgery will be but will be before the month is out.