Proptosis with orbital soft tissue and bone changes and unilateral

papilloedema: unusual presentation of POEMS syndrome

Dheeraj Gandhi, Sushma Vashisht*, Amit Mahajan, Anu Kapoor, Manorama Berry

Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India

Received 20 April 2000; accepted 22 August 2000

Abstract

POEMS syndrome is a rare manifestation comprising of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and

skin changes. Orbital involvement in this syndrome is rare and manifestation with proptosis, even rarer. The imaging features in Poems

syndrome and rarity of various features in our case are being highlighted here. D 2001 Elsevier Science Inc. All rights reserved.

Keywords: POEMS, Orbit, Proptosis, Imaging, Papilloedema

1. Introduction

POEMS syndrome is an unusual complex multisystem

disorder comprising of polyneuropathy (P), organomegaly

(O), endocrinopathy (E), monoclonal gammopathy (M)

and skin changes (S). Orbital involvement in the form

of papilledema and infiltrative orbitopathy has been

described previously in this syndrome. We report an

unusual case of POEMS syndrome with unilateral prop-

tosis due to extensive orbital soft tissue and bony

changes. Our patient also had unilateral papilledema and

infiltrative orbitopathy.

2. Case report

A 40-year-old man presented with generalised weak-

ness, weight loss, breathlessness and swelling over the feet

for 2 years. He also noticed blackening of the skin over the

trunk and impotence for the past 6 months. One month

before the presentation, the patient noticed progressively

increasing redness and protrusion of the left eye.

General physical examination revealed a man of

average build with gynecomastia and blackening of the

skin over the trunk. He had left-sided proptosis and

moderate conjunctival hyperaemia. Peripheral lymphade-

nopathy was present in the form of enlarged neck and

axillary nodes. Abdominal palpation revealed hepatosple-

nomegaly. Neurological examination was suggestive of

peripheral sensori-motor neuropathy with bilateral foot

drop, diminished knee and ankle jerks and wasting of

small muscles of the hand. Bilateral ulnar nerves were

thickened and left-sided papilledema was present on

fundus examination.

Complete blood counts were within normal limits

except for a mildly elevated ESR (32 mm, 1st hour,

Wintrobe). Bone marrow aspiration showed predominance

* Corresponding author. Tel.: +91-11-6864851.

E-mail address: gdheeraj@yahoo.com (S. Vashisht).

Fig. 1. PA view of orbits revealing expansile mixed sclerotic and lytic

lesions of roof and lateral wall of left orbit.

Journal of Clinical Imaging 24 (2000) 193± 196

0899-7071/01/$ ± see front matter D 2001 Elsevier Science Inc. All rights reserved.

PII: S0 8 9 9 - 7 0 7 1 ( 0 0 ) 0 0 2 09 - 6

of plasma cells. Peripheral lymph node biopsy revealed

follicular hyperplasia. There was evidence of hypergam-

maglobulinemia and an `M' protein spike was present on

serum electrophoresis. Urine examination was negative for

Bence±Jones proteins. Motor-nerve conduction velocity

was reduced in all the limbs but lower limbs were more

severely involved.

Multitudes of interesting findings were present on radio-

graphic skeletal survey. Mixed sclerotic and lytic lesions

with predominant sclerotic component were involving the

roof and lateral walls of the left orbit. The involved bones

were also expanded (Fig. 1). Multiple ill-defined lytic

lesions were also seen involving the axial skeleton includ-

ing ribs and vertebrae. Sclerotic and lytic lesions were seen

in the pelvis. A fluffy spiculated bony proliferation was

seen along the vertebral appendages (Fig. 2) especially

transverse processes, costovertebral and facet joints, obtura-

tor rings and iliac crests. Pleural effusions were present on

chest X-ray bilaterally.

An ultrasound scan of the abdomen confirmed the

presence of hepatosplenomegaly. Contrast enhanced CT

of orbits was performed in axial and coronal planes.

Extensive sclerotic and lytic lesions with bone expansion

and irregularity were seen involving the left orbital roof,

lateral wall and the greater wing of sphenoid. Associated

soft tissue mass was present along the bony lesion with

orbital component as well as a component in the middle

cranial fossa and left temporal fossa (Fig. 3). Diffuse

thickening of extraocular muscles and left-sided optic

nerve was also present. The margins of involved muscles

were irregular, suggestive of infiltrative orbitopathy (Fig.

4). Left-sided proptosis was present consequent to the

thickening of orbital contents.

3. Discussion

The acronym POEMS syndrome was first coined by

Bardwick et al. [1] in 1980. It was named as Crow±

Fukase syndrome by Nakanishi et al. [2]. There is a

debate as to how many components are necessary to

justify a diagnosis of POEMS syndrome. Osteosclerotic

Fig. 2. Fluffy new bone formation is seen along transverse processes of L4

and L5 vertebrae (arrow) and left obturator foramen along with lytic cum

sclerotic lesions in left iliac bone on PA view of pelvis.

Fig. 3. Sclerotic expansile lesion of left orbital roof with soft tissue mass in

left temporal fossa and middle cranial fossa (arrow) seen on axial contrast

enhanced CT of orbit.

Fig. 4. Axial contrast enhanced CTof orbits. Note irregular thickening of left-

sided medial and lateral recti and optic nerve, a manifestation of infiltrative

orbitopathy. An ill-defined soft tissue mass is also present laterally.

D. Gandhi et al. / Journal of Clinical Imaging 24 (2000) 193±196194

bone lesions and peripheral neuropathy are considered

essential [3].

This disorder usually affects the patient at a relatively

young age, more commonly males [M:F = 2:1] [4]. In this

unique syndrome, abnormalities occur in the central and

peripheral nervous system, the integument, the endocrine

glands, the skeleton and the reticulo-endothelia and

immunohematopoetic systems [1]. The widespread

abnormalities are possibly related to associated plasma

cell dyscrasias [1].

A variety of multisystem findings including peripheral

neuropathies, hepatosplenomegaly, diabetes, hypothyroid-

ism, gynecomastia and impotence in males and oligomenor-

rhoea or ammenorrhoea in females, skin pigmentation and

hypertrichosis can be present as features of this rare dis-

order. Other reported findings include lymphadenopathy,

pleural effusions and ascites, monoclonal gammopathy and

a host of skin lesions [4].

The abnormal M protein is usually the lambda subtype as

opposed to kappa subtype in myeloma patients [2,4].

Despite several theories on the pathogenesis of this syn-

drome, there is still a lack of unifying hypothesis. However,

plasma cell dyscrasia appears to be linked closely to the

clinical manifestations of this syndrome.

A variety of Roentgen features have been described in this

syndrome. Spiculated bone proliferation is thought to be a

pathognomonic feature of this disorder [5]. Osseous prolif-

eration is seen at the apophyseal joints, laminae, transverse

processes, discovertebral and costovertebral articulations in

the axial skeleton. Similar changes are observed in the areas

of tendinous and ligamentous attachments, such as the

obturator rings, iliac crests and interosseous membranes.

Because of the distinctive nature of this bony proliferation,

POEMS syndrome can easily be differentiated from other

enthesopathic entities like diffuse idiopathic skeletal hyper-

ostosis, seronegative spondyloarthropathies, fluorosis and

hypoparathyroidism [5].

Osteosclerotic skeletal lesions with or without lytic areas

are seen in 85% of patients with POEMS syndrome. The

lesions are seen as either a uniformly dense focus or lytic

lesion surrounded by sclerosis. Sclerotic foci need to be

differentiated from other causes, such as osteosclerotic

metastases, bone islands, mastocytosis, etc. If the clinical

features are not helpful, biopsy may be required for accurate

diagnosis [6]. Purely lytic lesions are uncommonly seen in

POEMS syndrome.

In our patient, almost all classical findings of POEMS

syndrome were seen on skeletal survey. Extensive spicu-

lated bone proliferation was seen along entheses and

apophyseal joints. Multiple lytic lesions were present in

ribs and spine and mixed sclerotic± lytic lesions were

present in the pelvis.

Orbital involvement described in POEMS syndrome

includes papilledema and infiltrative orbitopathy. Bardwick

et al. [1] in 1980 found that 68% of patients described in

published reports had papilledema. In a review of 102 cases

by Nakanishi et al. [2], 63% of patients were found to have

papilledema. Bolling and Brazis [7] reported 11 cases of

POEMS syndrome, 8 of which had papilledema. In all of

these eight cases, papilledema was present at the time of

referral to their centre suggesting that this can be an early

sign of POEMS syndrome. The patients had a clinical

course similar to papilledema associated with increased

intracranial pressure. These patients had transient obscura-

tion of vision, good visual acuity, enlarged blind spots and

progressive constriction of visual fields.

The cause of papilledema in POEMS syndrome is

unclear. It may occur in the presence or absence of increased

intracranial pressure or increased levels of CSF protein [8].

Diffuse swelling of posterior orbital contents bilaterally,

consistent with infiltrative orbitopathy recognised on CT

has been reported by Bourdette and Rosenberg [9]. Changes

of infiltrative orbitopathy may have been responsible for the

papilledema in their patient.

Several previously unreported findings in the orbit in

our patient prompted us to report this case. This report adds

one more cause of proptosis in an already extensive list of

causes of proptosis. In none of the previously reported

patients of POEMS syndrome, infiltrative orbitopathy was

so extensive as to cause proptosis. Also unusual is the

presence of unilateral papilledema along with characteristic

radiological changes in the orbit. Presence of unilaterality

suggests a local rather than intracranial cause of papille-

dema in the present case. Although sclerotic lesions occur

in 86±88% of cases of POEMS syndrome, such marked

osseous changes in the bones of the orbit have not been

observed earlier. Differential diagnosis of sclerotic bony

lesions in the orbit includes fibrous dysplasia and osteo-

blastic metastasis. Fibrous dysplasia is easily excluded, as

soft tissue masses do not accompany this disorder.

Although the radiological picture in the present case merits

a strong consideration for metastatic disease, other changes

in the skeleton and the clinical presentation easily excluded

this possibility.

Similarly, differential diagnosis of infiltrative orbitopathy

includes inflammatory pseudotumor, Grave's disease and

lymphoma. In this case, however, the presence of sclerotic

expansile bony lesions with associated extraconal orbital

and intracranial mass along with infiltrative orbitopathy

make it a characteristic radiological appearance of POEMS

syndrome. Unilateral papilledema and proptosis were pos-

sibly caused by plasma cell infiltration of optic nerve or

nerve sheath and extraocular muscles.

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