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267 Journal of Natural Science, Biology and Medicine | January 2015 | Vol 6 | Issue 1

Case Reports

Infra-orbital nerve schwannoma: Report and review

Abstract

Extra-cranial schwannomas although common in head and neck region are very rarely seen originating from the infra-orbital nerve. We report a case of schwannoma arising from infra-orbital nerve in a 40-year-old male patient. The case presented as an isolated, asymptomatic, slow growing sub-cutaneous nodular swelling over left side of mid-face. On ultrasonography, a localized lesion within the sub-cutaneous tissue of cheek was observed, without involvement of orbital, maxillary sinus or underlying bone. Aspiration biopsy of the lesion showed spindle shaped cells predominantly arranged in Antoni A pattern around verocay bodies, with less organized Antoni B tissue in few places. Diagnosis of schwannoma, probably arising from terminal branch of infra-orbital nerve was established. The tumor was approached through skin incision. At the time of exploration, the lesion was found to emanate from the nerve trunk of peripheral branch of infra-orbital nerve, which was dissected and preserved. We correlate our experience with previously reported cases of infra-orbital nerve schwannoma.

Key words: Infra-orbital nerve, mid-face, schwannoma, swelling

Kumar Nilesh

Department of Oral and Maxillofacial Surgery, School of Dental Sciences, KIMSDU, Karad, Satara, Maharashtra, India

Address for correspondence:Dr. Kumar Nilesh, Department of Oral and Maxillofacial Surgery, School of Dental Sciences, Krishna Hospital, Karad, Satara - 415 110, Maharashtra, India. E-mail: drkumarnilesh@yahoo.com

INTRODUCTION

Schwannoma is a benign tumor ar is ing from neuroectodermal schwann cell of cranial, intraspinal, peripheral and autonomic nerve sheaths.[1] Lesions in head and neck region account for 25-45% of extra-cranial schwannomas.[2] However, it rarely involves infra-orbital nerve and are uncommon in the maxillary and buccal space.[3] These tumors usually present as slow growing nodular mass and can mimic any benign growth in the head and neck. The treatment of extra-cranial head and neck schwannomas is surgical and the approach depends on the location and extent of the tumor and the nerve involved.

This paper describes a case of schwannoma arising from infra-orbital nerve, presenting as slow growing nodular swelling in the buccal space. The lesion was approached through skin incision and completely resected. At the time of exploration, the lesion was observed to emanate from the nerve trunk of peripheral branch of infra-orbital nerve, which was dissected and preserved. Only nine cases of infra-orbital nerve schwannoma are previously reported, which we discuss in relation to our experience.

CASE REPORT

A 40-year-old male patient presented with a chief complaint of painless swelling over left side of face.

The patient fi rst noticed the swelling about a year back, which had grown gradually to its present size. Patient also complained of mid paresthesia over left cheek since past few months. On examination, a localized, nontender, fi rm, round swelling of about 4 cm was evident over left side of mid-face, extending from below the infra-orbital margin to upper lip [Figure 1]. The overlying skin appeared stretched and tense. The skin was pinch-able over the lesion, except at the center of the lesion. The naso-labial fold was obliterated. Intra-oral examination showed no swelling over anterior maxilla. The maxillary anterior teeth were fi rm and nontender on percussion. There was no proptosis and vision was normal. The cervical

Figure 1: Lesion presenting as large sub-cutaneous nodular swelling

268Journal of Natural Science, Biology and Medicine | January 2015 | Vol 6 | Issue 1

Case Reports

lymph nodes were not palpable. No signifi cant medical history or history of previous trauma was reported. The overall clinical presentation was suggestive of a benign sub-cutaneous lesion and differential diagnosis included lipoma, fi broma, dermoid cyst, epidermoid cyst and neurogenic tumor.

Ultrasonography was advised to study nature, size and extent of the lesion. The ultrasonogram showed well-defi ned, heterogeneous oval mass of 45 mm × 30 mm × 23 mm within the buccal sub-cutaneous tissue with no orbital, maxillary sinus or underlying bone involvement [Figure 2]. Aspiration biopsy of the lesion showed spindle shaped cells predominantly arranged in Antoni A pattern around verocay bodies, with less organized Antoni B tissue in few places [Figure 3]. Diagnosis of schwannoma, probably arising from terminal branch of infra-orbital nerve was established.

Surgical excision of the lesion was planned and executed under local anesthesia. After attaining

adequate anesthesia, linear incision of about 3 cm was placed over skin along the natural skin crease of naso-labial fold. The lesion was well-encapsulated, and freed from surrounding tissue by blunt dissection [Figure 4a]. Portion of skin overlying the center of the lesion appeared thinned and was included in the excised nodule. At the time of exploration, the lesion was found to emanate from the nerve trunk of peripheral branch of infra-orbital nerve, which was dissected and preserved [Figure 4b]. Histopathological examination of the excised lesion confirmed diagnosis of schwanomma [Figure 5].

DISCUSSION

Schwannoma (neurinoma, peripheral glioma, perineural fi briblastoma and neurilemmoma) is ectodermal benign neoplasm, which originates from schwann cell of cranial,

Figure 2: Ultrasonogram showing a heterogeneous oval mass within the sub-cutaneous tissue

Figure 3: H and E stained section of aspirate showing verocay bodies (arrow head) surrounded by spindle shaped cells arranged in orderly parallel fashion (arrows)

Figure 5: H and E stained section (×10) of excised specimen showing spindle shaped cells predominantly arranged in Antoni A pattern around verocay bodies (black arrow), with less organized Antoni B tissue in few places (red arrow). Vessel in schwannoma having wide lumen, fi brotic wall and adjacent hyalinization (blue arrow)

Figure 4: (a) Exposure of the lesion through skin incision; (b) dissected tumor attached to the nerve trunk of peripleral branch of infra-orbital nerve (block arrow)

a b

269 Journal of Natural Science, Biology and Medicine | January 2015 | Vol 6 | Issue 1

Case Reports

Tab

le 1

: R

evie

w o

f p

revi

ou

sly

rep

ort

ed c

ases

of

sch

wan

no

ma

invo

lvin

g IO

N*

Aut

hor a

nd

year

of

publ

icat

ion

Spec

ialty

of

jour

nal

Age

Sex

Clin

ical

feat

ures

Are

a in

volv

edSi

ze o

f tum

orIO

N in

volv

emen

tSu

rgic

al

appr

oach

Rec

urre

nce

Ha

et a

l.[10]

2013

Cra

niof

acia

l su

rger

y20

Fem

ale

Pain

less

sw

ellin

g of

righ

t ch

eek

Eye

signs

: Non

e

Infra

-orb

ital s

ub-

cuta

neou

s sw

ellin

g13

mm

×

10 m

m ×

5 m

mN

o pr

eope

rativ

e pa

rest

hesia

po

stop

erat

ive h

ypoe

sthe

sia

of n

asal

sep

tum

and

ve

stib

ule

(intra

nasa

l bra

nch

of IO

N in

volve

d)

Intra

-ora

l app

roac

hN

one

Kin

ger e

t al.[1

4]

2013

Oth

er30

Fem

ale

Pain

less

sw

ellin

g of

righ

t ch

eek

(sin

ce 1

yea

r)Ey

e sig

ns: N

one

Rig

ht in

ferio

r orb

it,

pter

ygop

alat

ine

foss

a an

d po

ster

ior p

art o

f m

axilla

ry s

inus

5.2

cm ×

3.1

cm

No

preo

pera

tive

pare

sthe

sia. I

ON

id

entifi

ed

and

pres

erve

d in

traop

erat

ively

Intra

-ora

l app

roac

hN

one

(1 y

ear

follo

w-u

p)

Kok

et a

l.[11]

2013

Plas

tic s

urge

ry12

Mal

eN

aso-

labi

al s

ub-c

utan

eous

lu

mp

(3 m

onth

s du

ratio

n)E

ye s

igns

: Non

e

Infra

-orb

ital s

ub-

cuta

neou

s sw

ellin

g2.

5 cm

ION

iden

tifi e

d an

d pr

eser

ved

intra

oper

ative

lyIn

tra-o

ral a

ppro

ach

Non

e (2

yea

rs

follo

w-u

p)

Rav

iraj[1

2] 2

011

Hea

d an

d ne

ck

surg

ery

8M

ale

Rec

urre

nce

of s

wel

ling

on le

ft ch

eek

belo

w o

rbit

(sin

ce 4

yea

rs)

Eye

sig

ns: N

one

Infra

-orb

ital s

ub-

cuta

neou

s sw

ellin

gN

A*N

o pr

eope

rativ

e pa

rest

hesia

Web

er-F

urgu

sson

in

cisio

n w

ith

Def

fenb

ach

exte

nsio

n

Non

e (6

mon

ths

follo

w-u

p)

Cho

i et a

l.[3]

2009

Ora

l and

m

axillo

facia

l su

rger

y

54Fe

mal

eP

ainl

ess

swel

ling

on le

ft m

id-fa

ce re

gion

(2 m

onth

s du

ratio

n)E

ye s

igns

: Non

e

Max

illary

sin

us7.

5 cm

×

4.3

cm ×

2.5

cm

ION

atta

ched

to tu

mor

m

ass

iden

tifi e

d an

d th

e pa

rt at

tach

ed to

the

lesio

n w

as

sacr

ifi ced

Cal

dwel

l-Luc

ap

proa

ch c

ombi

ned

with

ext

ra-o

ral

subc

illiar

y in

cisio

n

Non

e (1

yea

r fo

llow

-up)

Cla

renç

on e

t al

.[7] 2

009

Neu

rora

diol

ogy

45Fe

mal

eP

ainl

ess

infra

-orb

ital m

ass

(dev

elop

ed re

cent

ly)

Eye

sig

ns: D

ispl

acem

ent

of g

lobe

upw

ards

Left

low

er o

rbit

NA*

No

preo

pera

tive

pare

sthe

sia. I

ON

sac

rifi c

ed

intra

oper

ative

ly

Extra

oral

skin

in

cisio

nN

A*

Gar

g et

al.[8

] 20

08O

ptha

lmol

ogy

35Fe

mal

eP

ainl

ess

slow

gro

win

g m

ass

in ri

ght i

nfer

ior o

rbit

Eye

sig

ns: N

one

Rig

ht lo

wer

orb

it20

mm

× 1

5 m

mN

A*Ey

elid

cre

ase

incis

ion,

righ

t or

bito

tom

y

NA*

Kar

kas

et a

l.[13]

2008

Hea

d an

d ne

ck

surg

ery

14M

ale

Hea

dach

e an

d rig

ht n

asal

ob

stru

ctio

nE

ye s

igns

: Rig

ht e

ye

exop

htha

lmos

, scl

eral

sh

ow

Rig

ht m

axilla

ry s

inus

, in

frate

mpo

ral f

ossa

, in

ferio

r par

t of r

ight

or

bit,

pter

ygop

alat

ine

foss

a, p

art n

asal

cav

ity

59.5

mm

× 4

3 m

mN

o pr

eope

rativ

e pa

rest

hesia

. IO

N s

acrifi

ced

in

traop

erat

ively

Web

er-F

ergu

sson

in

cisio

n, o

steo

plas

tic

max

illect

omy

Non

e (7

mon

ths

follo

w-u

p)

Teze

r et a

l.[9]

2006

Oto

lary

ngol

ogy

16Fe

mal

ePa

in a

nd d

ispla

cem

ent o

f lef

t ey

ebal

lLe

ft lo

wer

orb

itN

A*N

A*Su

bcilli

ary

incis

ion

NA*

ION

: Inf

ra-o

rbita

l ner

ve, *

NA

: Dat

a no

r ava

ilabl

e/de

scrib

ed

270Journal of Natural Science, Biology and Medicine | January 2015 | Vol 6 | Issue 1

Case Reports

intraspinal, peripheral and autonomic nerve sheaths. They are common in head and neck region, accounting for 25-45% of extra-cranial schwannomas and 1-8% of all head and neck tumors.[4] It can involve any of the 12 cranial nerves, except the olfactory and optic nerves since they lack schwann cells in their sheaths.[5,6] Schwannoma arising from infra-orbital nerve (branch of maxillary division of fi fth cranial nerve) is extremely rare. To best of our knowledge, only nine cases of infra-orbital schwannoma has been previously described in English literature.[3,7-14]

Schwannoma generally presents as slow growing painless sub-cutaneous or submucosal swelling. Depending on the site of nerve trunk or branch from which it arises, it may be located within the lower part of orbit,[7-9] maxillary sinus[3] or present as sub-cutaneous mass in infra-orbital region[10-12] [Table 1]. Intraorbital schwannoma generally develop from supraorbital or supratrochlear nerves, and less frequently from infra-orbital nerves.[9] Lesions arising from infra-orbital nerve grow to involve surrounding structures such as maxillary sinus, nasal cavity, infratemporal fossa and pterygopalatine fossa.[13,14] However, it is rare to fi nd schwannoma in the paranasal sinus, especially in the maxillary sinus.[15] Swelling in the cheek, nasal atresia, downward transversion of the palate and pain, rarely accompanying exophthalmosis are common symptoms. However in our case, a well-localized painless infra-orbital sub-cutaneous swelling was observed without any ocular lesions or involvement of underlying osseous skeleton.

Rarely schwannomas may present with numbness in the distribution of involved nerve or with pain.[16] Our patient reported paresthesia localized over area of distribution of superior labial branch of infra-orbital nerve, which was probably due to pressure or direct involvement of the peripheral branch of the nerve. However during the surgical dissection, lesion was found to emanate from the nerve trunk of peripheral branch of infra-orbital nerve, which was dissected and preserved. None of the previous cases of infra-orbital schwannoma reported preoperative paresthesia [Table 1].

Fine-needle aspiration cytology (FNAC), ultrasonography, magnetic resonance imaging and computed tomography image are diagnostic tools for schwannoma. Diagnosis using FNAC is characterized by the presence of spindle cells and has only 17.6% accuracy.[17] Aspiration of our lesion yielded blood tinged aspirate, which showed spindle shaped cells predominantly arranged in Antoni A pattern around verocay bodies, with less organized Antoni B tissue in few places confi rming the diagnosis of schwanomma.

The treatment of schwannomas is exclusively surgical and the appropriate approach is dictated by the extent and location of the tumor. In our case, the tumor originated from the peripheral branch of infra-orbital nerve and presented as nodular swelling immediately underlying the skin. Incision placed in the natural skin crease of naso-labial fold was used to approach and completely remove the lesion. Other approaches used include intra-oral vestibular incision for smaller lesions localized in the buccal space,[10,11,14] Caldwell-Luc approach for tumor within the maxillary sinus,[3] Subcilliary or eyelid crease incision for intraorbital schwannoma.[8,9] Larger lesion involving greater areas of mid-face are treated by extra-oral Weber-Furgusson incision combined with ostectomy[13] [Table 1]. Although malignant transformation and recurrence of schwannoma is very low, a case of recurrent infra-orbital schwannoma[12] attributed to incomplete removal of the lesion through a limited skin incision was reported.

CONCLUSION

Schwannoma arising from the infra-orbital nerve is rare, making the clinical diagnosis often diffi cult. Although uncommon, schwannoma involving the peripheral branch of infra-orbital nerve may present as localized mid-face swelling and should be included in differential diagnosis of benign sub-cutaneous swelling in infra-orbital region.

REFERENCES

1. Marx RE, Stern D. Oral and Maxillofacial Pathology: A Rationale for Diagnosis and Treatment. 2nd ed. United States Quintessence Publishing Co. Inc.; 2003.

2. Samet A, Podoshin L, Fradis M, Simon J, Lazarov N, Boss H. Unusual sites of schwannoma in the head and neck. J Laryngol Otol 1985;99:523-8.

3. Choi BH, Park SW, Son JH, Cho YC, Sung IY, Byun KJ, et al. Schwannoma in the maxillary sinus and buccal space: Case report. J Korean Assoc Oral Maxillofac Surg 2009;35:494-8.

4. Yoon E, Rhee SC. Solitary trigeminal schwannoma of paranasal region. Int J Pediatric Otorhinolaryngol Extra 01/2007;2:120-4.

5. Leu YS, Chang KC. Extracranial head and neck schwannomas: A review of 8 years experience. Acta Otolaryngol 2002;122:435-7.

6. Katz AD, Passy V, Kaplan L. Neurogenous neoplasms of major nerves of face and neck. Arch Surg 1971;103:51-6.

7. Clarençon F, Jafari A, Lefevre M, Périé S, Angelard B, Marsault C, et al. Infraorbital nerve schwannoma. J Neuroradiol 2009;36:301-3.

8. Garg R, Dhawan A, Gupta N, D’souza P. A rare case of benign isolated schwannoma in the inferior orbit. Indian J Ophthalmol 2008;56:514-5.

9. Tezer MS, Ozcan M, Han O, Unal A, Ozlugedik S. Schwannoma originating from the infraorbital nerve: A case report. Auris Nasus Larynx 2006;33:343-5.

10. Ha W, Lee JW, Choi J, Yang SW, Kim SY. Schwannoma originating from infraorbital nerve. Arch Craniofac Surg 2013;14:61-4.

11. Kok YO, Yeo MS, Nallathamby V, Lee SJ. Infraorbital nerve schwannoma presenting as an upper lip mass in an adolescent boy. Ann Plast Surg 2013;71:196-7.

271 Journal of Natural Science, Biology and Medicine | January 2015 | Vol 6 | Issue 1

Case Reports

12. Raviraj GA, Thomas R, Dhanraj GA, Rao US. Pediatric infraorbital nerve schwannoma: A rare clinical entity. Int J Head Neck Surg 2011;2:73-5.

13. Karkas AA, Schmerber SA, Be ega GV, Reyt EP, Righini CA. Osteoplastic maxillotomy approach for infraorbital nerve schwannoma, a case report. Head Neck 2008;30:401-4.

14. Kinger A, Kawatra M, Chaudhary TS, Chaudhary A. Plexiform schwannoma of infraorbital nerve – A rare case report. Int J Bioassays 2013;02:534-6.

15. Sarioglu S, Ozkal S, Güneri A, Ada E, Sis B, Erdag TK, et al. Cystic schwannoma of the maxillary sinus. Auris Nasus Larynx 2002;29:297-300.

16. Dervin JE, Beaconsfi eld M, Wright JE, Moseley IF. CT fi ndings in orbital tumours of nerve sheath origin. Clin Radiol 1989;40:475-9.

17. Salemis NS, Karameris A, Gourgiotis S, Stavrinou P, Nazos K, Vlastarakos P, et al. Large intraparotid facial nerve schwannoma: Case report and review of the literature. Int J Oral Maxillofac Surg 2008;37:679-81.

How to cite this article: Nilesh K. Infra-orbital nerve schwannoma: Report and review. J Nat Sc Biol Med 2015;6:267-71.

Source of Support: Nil. Confl ict of Interest: None declared.

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DOI:10.4103/0976-9668.149229

A rare case of thyrotoxic periodic paralysis precipitated by hydrocortisone

Abstract

Thyrotoxic periodic paralysis (TPP) is a rare, but serious condition characterized by acute paralytic attacks and hypokalemia in association with thyrotoxicosis. Although carbohydrate rich meals, strenuous exercise, alcohol, emotional stress are known precipitants of TPP, steroid treatment has rarely been reported to induce TPP. We report a case in which a patient with previously untreated Grave’s disease developed TPP following administration of Intravenous hydrocortisone for control of severe anaphylaxis, which to best of our knowledge is very rare.

Key words: Anaphylaxis, hydrocortisone, hypokalemia, thyrotoxic periodic paralysis

Subrata Chakrabarti

Department of General Medicine, IPGMER, Kolkata, West Bengal, India

Address for correspondence:Dr. Subrata Chakrabarti, Doctor’s Hostel, IPGMER, A. J. C Bose Road, Kolkata - 700 020, West Bengal, India. E-mail: subratachakrabarti2011@gmail.com

INTRODUCTION

Thyrotoxic periodic paralysis (TPP) is mainly reported in young Asian males in their 3rd-4th decade of life,[1,2] classically evident as acute paralytic attack and hypokalemia in the background of thyrotoxicosis.[3] TPP should be considered as a cause of acute weakness to avoid missing a treatable and curable serious condition when established (heavy meal, exercise) or probable (beta 2 agonists, insulin, steroids) clinical history is evident.

CASE REPORT

A 32-year-old female not on any medication was rushed to the emergency unit with severe dyspnea along with intense

wheezing, pruritus, and swelling of face for ½ h following multiple bee-stings. She did not complain of any myalgia or muscle weakness of any part of the body or passage of dark colored urine. Examination revealed the presence of generalized urticarial rash, angioedema, nasal congestion, tachypnea, rapid thready pulse, and hypotension (blood pressure [BP]: 96/66 mmHg at admission). A clinical diagnosis of anaphylactic shock was made and patient was administered high fl ow oxygen, nebulization with levosalbutamol and intravenous fl uids (crystalloids). As her respiratory distress was not being alleviated following 1 h of this treatment and epinephrine injection not being available locally, it was decided to administer corticosteroids to diminish her dyspnea. Hydrocortisone (100 mg) was injected intravenously in short push. Her urticaria and