CONGENITAL A Case M.

BEN-BASSAT,
From Beilinson the

MACRODACTYLY
with a Three-year and Follow-up
PETAH-TIKVA,
Endocrinology, Medical School

Report

J. CASPER,

I. KAPLAN
Plastic Israel,

Z. LARON,
and Tel

ISRAEL

Departmetzts (etitre,

ofPathologv, Petah Tik ia,

Surgery a,zd the

Paediatric

Medical

.4 iiv University

The Laurenzi theory Debeyre but,

have not

literature 1962) relates 1961).

been

pertaining and the a clear condition the

to macrodactyly concept to cases studies neurofibromatosis reported in bone

is sparse

(F#{232}vrend a does (Moore not 1942,

Bricage yet exist. Inglis (Moore

1936, The 1950, 1942, of the

Clifford most Inglis Mouly affected

1959, popular and 1950) finger

of its pathogenesis some maturation were

Among
reported.

in children and development

so far

as is known,

CASE

REPORT

lii April was the

mental

1960, only,

after was

a normal Jewish

first

pregnancy parents. No other years was

and

labour,

a boy involving was Body

weighing the

33 middle

kilograms finger of and the

born left

to young hand
development

Yemenite

Macrodactyly, abnormality normal.

present at birth. for the first three

found. The physical length increased along

tenth

percentile and weight along the twenty-fifth percentile. The affected finger, as shown in Figure 1, showed great uniform enlargement, of soft tissues, nail and bones with limitation of movement in the interphalangeal had ulnar deviation. skeletal age (Greulich hand. with the exception Radiographs and Pyle of the of the right hand 1950) was compatible enlarged finger, at the age of six months with the chronological the same degree

thickening joints, which showed age. that The the left The

showed

of maturation.

TABLE
COMPARATIVE SIZE AND BONE MATURATION

I
AND ABNORMAL MIDDLE FINGERS

OF NORMAL

Length

of middle
(centimetres)

finger

Bone
(years)

age

Chronological
age (years)
Normal

Abnormal

Normal

Abnormal

48

68

II

3 3.1

enlarged finger were compatible

epiphysial centres

showed, with
of

apart from gigantism, epiphysial a bone age of approximately

ossification were

growth centres two years. In

radiologically.

in the phalanges which the normal fingers no

The difference in bone

demonstrable

maturation twenty the

VOL.

between months (Fig. fingers

NO.

the 2). and

1966

macrodactyle Table their I shows bone

and ages

the until

normal the age

finger between of three

is seen the years.

even length

better of the

at the normal

age

of and

a comparison

abnormal 48 B,

2,

MAY

359

360
Because disturbances of were

M.

BEN-BASSAT,

J. CASPER,

I. KAPLAN

AND

Z.

LARON

the was 3).

loss three

of

function years old.

caused amputation

by

the at the

gigantism before

and operation

because showed

psychological joint
DO

already

manifest,

metacarpo-phalangeal

was

done

when

the

child (Fig.

Angiography

vascular

abnormality

FIG.

I
hands at the age of 6 months.

The clinical

appearance

of the

FI

2
Note finger. the centres of ossification in the

Radiographs

of both

hands

at 20 months. affected

PATHOLOGICAL Macroscopic through two-thirds the examination-The distal one-third ofthe digit showed finger (Figs. that
measured nine

FINDINGS by four centimetres. section both soft

JOURNAL OF

A transVerse through tissue and

BONE ANI)

Sectioll

4 and 5) and the hypertrophy

a longitudinal involved
1HE

the proximal bone. In the

JOINT SURGERY

CONGENITAL

MACRODACTYLY

361

#{182}

FIG. Microphotograph of

5 4. (Haematoxylin and eosin,

the

distal

phalanx,

as in Figure 65.)

VOL.

48 B,

NO.

2, MAY

1966

362
distal

M. BEN-BASSAT,

J. CASPER,

I. KAPLAN

AND

Z. LARON

third the enlargement was mostly caused by soft tissue. The distal phalanx was in appearance, one centimetre in diameter and with a thin wall and relatively wide It was surrounded by a broad ring of fatty tissue one and a half centimetres in width. In the proximal two-thirds of the finger the enlargement were These was caused by both of bone and soft normal phalanges tissue. The two proximal phalanges ofa child ofcomparable age (Fig. 6). approximately twice were also surrounded

tubular lumen.

hypertrophy the size of by adipose

as

tissue but to a lesser extent. Microscopic examination-Histological in the bone. In the abundant

fatty

abnormalities were found in the soft tissues as well tissue small nerve fibres were detected with proliferation

FIG.

6
the proximal eosin, phalanx. Inset-

A longitudinal
A normal

section

through

phal3nx

of the same

age at the
and

same
65.)

magnification.

(Haematoxylin

of perineural in the blood

consisting periosteum of

as well vessels.
fusiform

as endoneural The phalanges

cells

connective showed
with

tissue. No an unusual
collagen

pathological abnormality
fibres which

changes in the
was found

were observed form of a band

between the

interspersed

towards showed

and the end Howships

the cortical bone (Fig. 7). This band of the phalanges (Fig. 8). The fusiform Above the cortical containing bone a layer osteoclasts of osteoid (Fig. lacunae

was of variable thickness cells appeared to be either tissue No was cartilage present cells and were 9).

widening fibroblasts the seen. surface The

or osteoblasts.

areas of endochondral ossification and the centres of ossification were marrow of the phalanges was adipose with a hollow and abnormally
THE JOURNAL

undisturbed. wide marrow

OF BONE AND

The bone cavity.

JOINT SURGERY

CONGENITAL

MACRODACTYLY

363

FIG.

7
bone. with collagen (Haematoxylin fibres, can be and eosin, x28.) seen

The band
between

of

proliferating

fusiform

cells,

interspersed

the

periosteum

and

the

cortical

::

..

__-;

I.

.._....,

v,$c.._
_ . #{149};#{149}:-

:
#{149},,
#{149}.

, . . .,I.;. ..

,1
--. ., . ..

&_.:

...c,-

---

.-..;

;..-

,
i./ p .

i...-,-

, ,
. .,.

.,
.-. .) . - 1..

. .

#{149} . .

A.;

(;

,i,.&v,__
.

;
:f

0 .4.1k..

, -

FIG.

FIG.

9 are and

Figure

8-The

periosteal

segment

shown

(Haematoxylin Howships

and eosin, Iacunae containing

proliferating

>: 96.) Figure osteoclasts periosteal cells.

Figure 9-Osteoid on the border (Haematoxylin

in

7 at a higher magnification. tissue is shown and there between the cortical bone and eosin, >. 96.)

VOL.

48B,

NO.

2,

MAY

1966

364

M. BEN-BASSAT,

J. CASPER,

1. KAPLAN

AND

Z. LARON

DISCUSSION
in

Proliferation previously 1942,

of the adipose tissue and fibrosis in the peripheral nerves have reported cases of macrodactyly. On the basis of these findings Inglis 1950,
and

been described most authors is related

Inglis

(Moore
neurogenic

Mouly
that

and

Debeyre

1961)
a component

believe
of

that

macrodactyly

to a
(1950)

disorder

it constitutes

neurofibromatosis.

suggested the neural

cases of

that the increased growth of the intrinsic factor of neurofibromatosis

macrodactyly in

enlarged acting changes of

digits in macrodactyly is influenced locally. Moore (1942) described in the peripheral others, there were nerves were the clinical signs

by five found; of the

all of which

pathological and, in three lipomata in contact

in

one

there

was

a neurofibroma

neurofibromatosis-caf#{233}-au-lait appearance of dense connective cells. He

associated so that

spots, tissue

and with state, was as yet

naevi. Inglis (I 950) the phalanx containing or osteochondromata, in the finger The in described

described cartilaginous which

interpreted this as an early tumorous with neurofibromatosis. In the case reported here no tumorous growth we are not finding justified in this in regarding case, which has

is also

demonstrable to neurofibromatosis. not been

or elsewhere, outstanding cases of the on of other

it as related

pathological

macrodactyly, was the proliferation periosteum which was responsible the other for destruction of bone, the phalangeal bone.

of the fibroblastic tissue on the one hand for laying with the resulting thickening

between the cortex and down of osteoid tissue and of the cortex and gigantism

SUMMARY 1. A patient with macrodactyly of the middle finger of the grew left hand was rate followed than up from birth until the finger was amputated at the age 2. The affected finger, besides showing gigantism of three. at birth,

at a faster

the normal

fingers. The degree of bone maturation (as judged from the appearance and size of the phalangeal ossification centres) proceeded at a faster rate than the normal fingers. No vascular abnormality which could account for the gigantism was detected either radiologically or microscopically. The affected finger showed histological abnormalities of both bone and soft tissues.
REFERENCES
CLIFFORD,

R. H. (1 959)
245.

: The

Treatment

of Macrodactylism

: A Case

Report.

Plastic

and doigts.

Reconstructire Annales of the Ha,zd

Surgery, dAzatomie and Wrist.

23,

FIEVRE, M.,
GREULICH,

Pathologique,

R. (1936): Hypertrophie 13, 337. W. W., and PYLE, S. I. (1950): Radiographic

and
BRICAGE,

congdnitale
Atlas

irr#{233}guli#{232}re des of Skeletal

Developnieizt

Stanford : Stanford INGLIS, K. (1950): Local and to Acromegaly

Body
LAURENZ!,

University

Press.

Gigantism
Illustrating
American

(A Manifestation
the
Journal

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its Relation
in Disease when

to General

Gigantism

Influence
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Development

of

the

is Abnormal.

26,

V. (1962):
B. H.

Macrodactilia

del medio.
and

Giornale

di Medicina

I 12, 401.
Journal A propos of Boize cas. and Joint

Moore,

(1942) : Macrodactyly Surgery, 24, 617. MOULY, R., and DEBEYRE, J. (1961): de Chirurgie Plastique, 6, 186.

Associated

Peripheral
digital. Etiologie

Nerve

Changes.

Le

gigantisme

et traitement.

dun

Annales

THE

JOURNAL

OF

BONE

AND

JOINT

SURGERY