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On sireniform monsters with a consideration of the causation and the predominance of the male sex among them.

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FOCrR B'IGCKER
r)erelopmental malformatioiis a i d monstrosities remain
sourccs of ncrew-failing interest. R y the ancients they ~ e i * c 1
eyed as products of the supcmiatural; to the naive mind of
yesterday they gave full scope to all sorts of sinistcr speculations, arid t o the biologist of to-day they shed light into the
hidden factors ant1 laws of genesis and growth. That emhryologival freaks contributcd their p a r t in peopling the
realm of mythology with fanciful creatures is evident ; quit(.
as intelligilde that the Western mind of the misnamed Dark
Age, hcmmctl i i i b y the ~ i a r r o whorizoiis of a nascent culture,
should openly or surreptitiously accuse tlie woman who was
iiiifortmiate enough to give hirth to a misshapen child of
Iiariiig lwei1 visited hy an iiicul.)us. When hcr fruit assumed
t lie deformity here pictured (fig. 1),what was i i e a n r than to
iiisiliiiatc shc had llt
too familiar with a merman, or, wheii
gossip a i i t l superst ition hecamc lcss malevolent, to sap that a
triton had friglitened her iii her tlrcams !
('oiisideriiig the impressioii which the sireiiomclian monster
would makc. on the popular mind, it is strange that Aristotlc
( l o w I i o t list it iii liis fairly cxteiisive catalogne of monsti'osities givcii in his treatise " 0 1 i the generation of
animals. ' ' This iii(1icates its relative infrequeiicy. The credulous and \.olul)le I'liny, on tlie other hiiiid, repeats tlie sailors '
?-ar11 that somc~whci~c
iiear 11 lalid of tlie troglodyt cs tliclrv
>
O N SIKEN1FOF;XI
MONSTEHS
367
anatomist w ~ u l t leviiice but a n iota of the poetic license that
permeates the verhose productions of a Ra~tlioliii,he ~vould
staricl discredited for the rest of his life. Rartliolin figures
this monstrosity like tlic traditionally conceived Nereids, possessing wcbhecl haiids and voluptuous breasts, and it differs
only in the abseiice of a forked termination of tlie single
l o ~ v eappendage,
~
the very cliaractcr that was supposcd to
suggest its rcscmblancc~to those imagiiiary beiiigs of the sea.
older to acceiitiiatc tlie similarity, lie pictures it tlispoi-tiiig in tlic waves!
Not uiitil the eigliteeiitli ctwtui-y is e i ~ trace
~ q of
~ adoriiecl
rerbiage swept aim?- by tlie objective a i d factual ciescriptioiis of such meii a s Hottinger (1'706), Superrille (1744),
Sue ( l i 4 6 , 1Tt51), Baster (1'752, 1757) , A1l)reclit voii Haller
(1753), T<aauw LSoei.liaa\~ (1754, 175'7), Hofw (1'758), and
Uigli (liici).Xiiice tlicw tlie sireiiomelian monster lias 1)eeii
tlescribecl a i d discussed in iiumeyous papers, RS e\-idciicctl
13)- tlie appcndecl 1Aliograpliic i*ccoid,which, tliougli exhaus111
tive, hardly makes claim to completeness. Ballaiityne, in 1897,
stated that lie gathered 116 cases reported in the literature,
nntl since then approximately fifty more have been collected
11-: tlie present writer. If the latter adds the report of another
case, it is excused on the grounds that no entirely satisfactory
explanation has a s yet been furnished regarding its causation.
The specimen, here illustrated (fig. l ) ,was born a t the em1
of an apparently normal period of pregnancy, and lived, it
was said, about one 1iour.l Externally, the upper half of the
hod?- is marked by the preserice of a ‘ Turmschiidel ’ (turriceplial)-), incomplete harelip, and a left hand inclined toward
the radial side of the forearm. On the lower half of the
1 ) o t l ~ we
- note the absence of anal and urogenital openings,
a i d the fusioit of the inferior extremities throughout their
entire length in such a way that the soles of the feet face
forward. Comparatively r a r e is the perfect symmetry displayed; each foot being supplied with three toes which
project laterally, tlie terminal part of the coalesced appendage
suggests tlie appearance of a caudal fin o r tlie flakes of a
whale. Posteriorly, in tlie skin of the left gluteal region, is
a little foveola or cicatrix, and anteriorly there is an imperforate genital tubercle, approximately 1 em. long, joined
t o the pnhic region l ~ a7 thiii constricted pedicle.
Esamiiiation of the thoracic viscera revealed n o startling
aiiomalics. But tlic abclomiiial organs, excepting liver and
stom~cli,show almormalities of some sort or other. The
colon, greatly distentled with meconium and suspended by a
long mesentery, loops in a peculiar fashion up and c3own in
tlic left half of the peritoneal cavity. It begins with a malplacwl caecum and appendix and elids blindly in the left iliac
fossa. The head and the body of the pancreas, though united,
still I m r evidence of their early separate origin.
The adrcnals a r c large and flattened. There a r e no
kidneys in the normal locations, but several small and thiii
lohulated masses, which superficially appeared to be composed
T h e specimen was srcuic~tlthrough tlie kindness of Dr. Bertraiii Beers, attriidirig p1r;vsiciaii a t tlic Gr:iiit Hospital, Chicago.
O N SIRENIFORM I1IONSTEl:S
369
onlp of connective tissue, blood spaces, and a few scattered
little Cysts, were found to be situated in the lower p a r t of the
abdominal cavity and to be lying against the centra of the
lumbar vertebrae. Because of the writer's interest in the
iiormal and abiiormal developmciit of the kidiiey, the striking
fcatnrcs wliivli stained sectioiis of tlicse masses exhibit will
Fig. 3 Micropliotogmpli of section through kidney of sirenifarm monster.
cart., cartilaginous nodule ; c., cyst ; t., rcnal tubules ; g, glomerulus.
X 40.
be delineated a little more fully: What at the first glance
stands out most conspicuously in them is their profuse vascularity (fig. 3 ) . Indeed, the maiiy blood spaces and sinuses
create the impression almost of an angioma. Scattered here
and there throughout tlie intervening coiinective tissue are
islands or groups of rerial tubules or tlicir vestiges, but they
may also exist isolated or singly. As evidenced by their appearance and manner of braiicliing, some of the tubules arc
iriicyuestioiiably collecting ducts. But 110 clear case could lie
foinitl of a union 1)ctwecii them aiid the coiled uriiiiferous
tul)nles. 111 one part of the mass there is the segment of a
Inl-ger duct, which is hliritl at both eiids arid is liiietl with
traiisitional epithelium. Whether it represents a part of the
ureter, I am imable to say.
‘I’ht~ conrolnted renal tubules exhibit a teriticticy toward
segregation near the periphery of the mass, arid sometimes
~ v e 1 1proti.ude into the wide capsule that coiifiiies it. Tliey
occur in difierent expressions of retrogression, among wliicli
ivei*e observed the more conspicuous initial cystic forms,
wliich may resemble those discovered by the writer among
t l i t , nriniferous t n h l e s that degenerate during the developrrieiit of the normal
Cysts of much larger size also
exist, as indicatecl in figure 3. hIariy of the glomeruli a r e segmcLiitetl into tlistiiict tufts or lobuli, aiid usually show c k posits of a substaiice that is like hyaline or amyloitl. The
Iirmina of many tubules coiitaiii or a r e slightly distended wit11
i j yc~llowmaterial, waxy or colloidal in appearance, which is
a common attribute of dcfuiict urinifcrons tubules. The illterstitial tissue between the tubules abounds in cells wliicli
look like lymphocytes and leucocytes, aiid wliicli probably arc’
associated in some way or other with the regression of thc~
kifliiey parenchyma.
‘I’lic most astoantling fcatnre ill these abortive k i d i i e p is
t h e preseiice of five tmtilnginous nodules, oiic of which is
pictured in figine 3. ‘Uiese iiotlixles are slightly longer thmi
I)roa(l, are composed iiiimistalial~lyof clioiitlral ccllh, posscss
i i t:-pical
staining matrix, aiid are eiiclosed 117 n periclioudriirm. Cliiii i t he that tlicy HI’C misplaced sclerotornal
t~lcmcwtsof the vet-tehral axis Y
‘I’lici1.e ai’e IIO stnwtnrcs ~vlrieli can lie recognixetl defiriitelja s iwiiia
1)latltler alitl urct1ir.a. it small thick mass, just
“
~
7
S t u t l i r ~ i i ulwi (lie Eiit~icklunRsResc.liiclit? (](,I
RI~iis(l i e i i , iiislwsoiitlci(~: 1j <Jl)er d:is Votlia~ltlcnsclti cSiut,i
s l ) i i I < ~ l l l i : l f t t t i(:eii(’i:itioii \ o i l I € ~ I ~ I ~ ~ ~ I I2 )~ ~
i’licr
~ I ~tl:is
I I ~Scliicksql
II.
~ l c i: i I t ( - i ( i i
iitirlll:il :I11gel(>gt(w H , ~ i i i k : i ~ t ~ ~ l ~3 l) ~ (f i~l i~
<>
i l. tlrii r i s l i i t i i i g t l e i S ~ v i r i i ( ~ ~ r t c ~ i i .
I i i i f t f. tl. g t w r i i i u t c Aii:it., TJ. Aht., W t l . T 3 , 1 W + .
O t t o P‘.
K,liii[)iiieitli : “ \tvitrie
I l l ( i l w i i ( l ( ~ i iS i r i t : I w i i i i
O N SIREXIFORM MOXSTEIS
371
helow the umbilicus oti the iiiiier side of the abdomiiial
parietes, on traiissectiori showed a minute cavity lined with
epithelial cells; probably i t represents a rcmriant of the
uraclius.
Of the two well-formed testes, each with a n epidiclymis, the
left lies near the internal ahdomirial ring, while the right has
already entered the inguinal canal. A section of the external
genital appendage or phallus, slretclied in figure 4, reveals no
signs either of an urethral passage or of the corpora cavey-
Fig. 4 Dmwiiig of :I tl’;riisvcwe scetion tlrroiigli t l i c ~ gciiit:r I appeiidagc of
the sireiiiforrn monster. x 30. I&., 1r:rir folliclc; M U . , snmt glands; se., s e h x r c o ~ ~ s
glands. No indication of uretlir:r, corpom c:ivernos:L :1nt3 sporigios:~.
iiosa, but the cutaneous organs-hair,
sweat, and sebaceous
glaiicls-are numerous arid normally constructed.
A characteristic which, it will be demoristrated snhsc?quently, possesses outstanding significance, is the occur-reme
of oiily om ‘umhilical’ artery. This has a high origin from
tlie aorta, instead of from the exterrial iliac arteries, leaving
it in the region of the pancreas aiid arcliirig across tlie ahclomiiial cavity to thc nml)ilicus iii tlie free margiri of the
meseiitery. Posterior to the source of the umbilical artery,
the aorta is t1onl)le arid coiititiues a s slender vessels to the
amalgamated loiwr extremity.
372
OTTO I?. IiAMPMEIER
A Roentgen pictiire of the skeleton of tlie monster showed
minor defects in the upper cervical vertebrae, a malformed
sacrum, absence of coccyx, approximated ischia, separate and
normal-looking femora though rotated outward, two tibia,
with R single fibula between, and the ossification centers of
tlie metatarsi and plialaiiges of three digits 011 each side.
The musculature, nerves, and vessels of the specimen were
not dissected, because the writer, after fiiiding that detailed
dissections of similar cases had been carried out by previous observers, reserved it for his cmh-yological teaching
c*ollection.
‘I’o-day,the terms ‘ sympoclia’ or ‘ sirenomelia’ a r e geiierally
applied to the group of monstrosities t o mliicli the type just
tlelineated helongs. T. Geoffrey St. Hilaire, in 1836, attempted
to classify the dif‘forent varieties of sircniform monsters
1)y the iiames ‘ symelus,’ ‘uromelus,’ arid ‘sirenomehis,’ and
Fiirster, in 1865, employed tlie terms ‘sympus clipus,’ ‘simpus
moiiopm, ’ am1 ‘sympns apus, ’ ckpeiidiiig on whether the fused
lower extremity termiriated in two fairly distinct feet, one
foot, or none at all. But, like most classifications, these art’
cluite arbitrary. I n truth, 110 systematic arrangement can he
clwised whereby all the different varieties can find their place
iu tlie proper category; for an unbroken series exists from the
snperficially normal condition, in which oiily the sacrum may
I)e defective or the ischia approximated, to the extreme tleformity in which the l o ~ e half
r
of the body is but a coneshaped process, the bones of the pelvis a r e a coalosced mass,
and those of the inferior extremities are represented merely
1)p a single short irregular shaft o r stump. Nor is there a
sliaiy line of division lwtween these more or less symmetrical
teratological types, the Sympodia, arid the spmmetricd
Mouopodia, in mliom one-half of the pelvis and one entire
lower limb are suppressed during developmriit.
The malformations of the lower extremities characteristic
of the Sympodia may he and usually a r e associated with
many other abnormalities, some of which recur with relative
and oiie or two of which a r e met with constantly.
fr*equeii~y,
F l
l h e s e facts arc set forth iti the awornpaii+ig tahle, constructed from data gleaiictl fi.om the papers-ahout
eighty
in number-directly
or indirectly accessible to me. Unfortiiiiately, mail!- reports arc’ too h i c f aiitl fragmeiitai.y to he of
much value ; conscqueritly, {lie f reqneiicj- of rec~nrrriitanomalies caiiiiot he iiitlicatctl 1))- percctitagcs. Oiily the more w i dent aiid strikiiig malformatioiis arc listcd ; deviations in tlie
,
v-ascnlar system a r e oiily tonchetl, in
miis(d;ir, i i e i ~ o i i s ant1
so far as the!- 1i:ii.c. a beariiig 0 1 1 the cxplaiiatioii of the p r o b
1em mid e 1%t 1isc 11 ss i (I11 .
A pccdinrity of tlie sireiiiform moiisteI*, which a t first
strikes oiic’ a s i*c~mai*lial)le
a t i t l ~vliiclirecc4vetl prime comment
in the (lays wlieu the noi*mal c o u i w of the cliffcreiitiatioii of
the liml) h d s w a s not yet entirely utidcrstootl, is tlic i3erei.sd
of tlic sicles of the lon-ei*extremities, so that the soles of thc
feet, the popiiteal 1-egioiis-in sliort their post crior siirfacesfact f o i ~ v a r t l . F’ijrstei. was the first to point out that tlii,i
aiiomaloiis coiitlitioii resulted ~ I i e i ithe normal inward rotatioii of tlie extrclmitics failctl t o t a l x place cluritig their clc\-elopmciit. Tlie f;irts I ~ a i d l yticctl i.c.pc4tio11 here that the
liiiitl limb buds, origiiiall?- est(wlcd with their tihial margins
forward, their pliuitar snifacw metlialwirtl, am1 their f i l ~ d a r
mai*giiis cnntlalivartl, ant1 separated from each other only by
tlie iiiteryeiiiiig c~louc.ul0 1 ’ perincia1 regioti and tail bntl, woulcl
1)c brought to#cthcr-c.~1rlier at the fibular mnrgins-l,y n tlefkieiicy iii tlie mass of tliat region. If tlie hiatus occui-rcd
near tlic lwgiiiiiiiig of geiiesis, the ten.itoi*iesof the limh hutls
~ w i i l t iii
l cwiiseqiieiiw 1~ iii direct coiitiiinity and \vonltl tliffercntinte as morc 0 1 . less fused strnctures n-hicl~cwnld ~ i o te s emtc tlic tiorma1 rotatioils 011 t l i ~ i rasc’s.
Wlieii
iip1)roiwli the question of the causes uiicierlj-iiig
the sii*eiiomeliaii monstrosity, W P meet wit11 many tliflferent
Iiypothescs. The earlier ones naturally a r e vagne, siiice too
little w a s l a i o i v i i of the normal geiietic c o ~ ~ r s erl’lills,
.
IIcckel.
iii 182(i,esplaiiiecl it niitl other monstrosities as products of
ii ‘regelwi(1rig wirliciideii 1)iltlc~iitlcn
‘I’iitigkeit.’ Fiirster ( ’60)
bcliovctl that i t MYIS caiisetl l y 21 defective formatioii ant1
394
OTTO P. IiBJIPRTEIEl:
375
O N SIIlENIPORRI MONSTERS
CONDIflONS AND
YAXTS CONSIJlEI1F.D
CY O F CON1,ITIOSS A N D CK 4RAOTBK3
1
I
~~
~
I
Aldoinilial viseera
~~-~~
Atlren:rls abnorinall~h r g e (111 1)oth scxrs) : .? ; :lhsrnt : 7 ;
one absent: 2 .
Ahnormnl coiling : i i ~ l mesrnteric relations of intestines in
a11 cases esa~niiiell. Appendix :Llxxxnt : 2 ; colon ends
iiliii(1ly: 33: continued :is tlireatl t o :1n:11 region or caudal
a],pc~ll'l:lge: 9.
I<ithicjs a l ~ s e n t : 18; :itiophic or c?stic :ind malplaced: 2 2 ;
normal: 3 ; one :tl)sent : 6.
T'rrters :rl)scnt : 23 ; when piesent, defective.
Urinary
bladder absent : 2 5 . Urethra, wlicii mentioned, :il)seiit.
Primitive c1oac:il conditions : 13.
Testis and epididymis tisu:illy wrll formed, b u t in different
localities : abdominal, pehic, inguinal.
I Cteius double (separatr nidllerian ducts) or bifnicated: 4.
rni1)ilicaI (oin~~li:ilomrsriitrric?)arteiy single, and arising
higli from aorta (laiely from conmion iliac arterj-) in all
'
cases in wliicli this vessel is mentioned. Vestige of trae
ninbilic.:d a r t w > : 1; 1iypog:rstric artery ;ibsc~lt: 6 ; ren:iI
:tl,srnt : 4.
'
~
'
I
\
~ y ~ p ecxtiemities
i
'
Tlioi:ieie viscein
,
El e;l d
AI~nornii(llobation of lungs : 4 ; trachea joins ocsop1i:igus : 1.
Oiie or I K I ~ I Ialiseiit: 4 ; forearm drfcctiie: 2 ; hands Iwnt:
6 ; clnb 1i:inil: 2 ; supernurneiaiy fingers: 2 ; fingers rclduccd: 3 ; s?ndactyly: 3.
II~diocrplialy: 3 ; meningocoelr: 1 ; turiiceplialj : 1.
Izulbi oculi :il)sent : 2 ; defectil e : 1.
E:ris al)seiit or tlrformed: 5; nosc flnttmed: 3.
Month rlrfectil r (Ii:ii clip, c l r f t p:il:rtc) : 10.
Keck :il)srnt: 2 .
ahnormal twisting of the tail elid of the embryo-a vie\!expressetl earlier 11y BisclioE, and modified later 1)Alilfeld ( '79).
Labougle ailti Regiiier ( '89), finding 110 eviclciice of ail
allniitois 01- uraclius in tlie specimen which they examiiietl,
suggested that the polk sac took on the functions of tlic alla11tois, and that the malformations resulted from this change.
Similar conclusioiis n.erci 1-caclicci iriclepeiideiitly hy Bnllaiityiic and Kuliga later.
fetils. This, lioivever, does not necessarily imply that it was
waiiting a t an ear1~-geiietic period Those wlio claim that
the fetal liitliieys contribnte tlie bulk of the amniotic fluid
~vonltlsee the explaiiation of tlie tleficiciicy in the suppression
of the urinary system in n-hole o r in part, which is evident in
the vast majority of cases of siiwi formation. But it should
also lie stated that the opposite condition, that of liydramiiios,
has been observed to accompaiiy it.
Boll;, iii 1899, tle\-clopiiig his idea of ‘ segmental anatomy,’
employs it iii his elucidation of the sireuomelian monster.
The greater the 11iiml)er of somites that fail to be laid tlowi~
at tlie candal ciitl of the hocly, the more estcnsive Ilic mallformations. Theoretically, tliis statement should not arouse
ohjcctioii, hut actual oliservatioiis show tliat the self-same tlefccts appear in specimeiis possessing. a iiormal number of
x-crtchral segments, or eyeii snperiinmcrary segments, as indicated in the table.
Wlicii tlie presciit xi ritei- examined tlic specimen here
tlesci.il)etl, aiitI fouiitl only oiie ‘iimbilical’ ai*tei*yarising. high
from the aorta, and the i ~ l a t i v e l ysleiitler coiitinuatioii of
llie cloi~blcaortae beyond that point, tile tliought occurrcd to
liim that the fault? development of the caudal half of thc
liotlj- might be the effect of nutritional want. On readiiig
otlier papers iii search for fnrther evidence in this direction,
the writer discoverccl with rather mixed feelings that essentially the same theory had beeii expressed as early as 18T8
by C’. TTeigert lwfore the Physiological Society in Leipzig.
Yet f ew subsequent writers liave taken cogiiiznnce of his view,
ant1 those that did rejected it, so t h a t his opiiiioii stands aloiic.
The character ~vliicliwas founcl to recur constantly in the
reports 011 sireiiiform moiisters, that had bceri thorouglilp
esamiiietl iii tei-iially, is the presence of a single umbilical
artery comiiig off tlirectly from the aorta, or rarely from an
arteria iliaca commuuis. Already Weigert maintained that
tliis umbilical vessel in reality is a persistent ritelline or
omplialomeseiiteric artery. Uiifortiuiately, most observers
have paid little, if any, attention to the arteries of the abtlo-
rneii :iii(l pelvis beyoiid tlic point of emergence of the ‘urn1)ilical’ vessel, hiit tlie fen- that did so emphasize tlie smallness
of the aorta aiitl the iliac artei*ics aiicl the absciice or insigiiificaiice of tlic liypogastric. Iiideetl, it may he argued that
tlie latter iwriaiiietl small 1)ecaanse the ter1itoi-y which tho\.
snpplied, lwiiig defective from the start, demaiitled lit tlc from
tlie 1)lootl stream. R u t the coiistalic-y of the high origin of
tlie ‘nrrihilical ’ ai-tery, which appears like a direct proloiigatioii of the aorta, impels me to accept it a s the prime factor,
ah f a r a s the emlti-\.oiiic hod)- itself is coiiceriied, iii tlic procIiit+oii of tlie (list nrlmices cliaracteristic of the s i i w
rrioiistiwsity. That the gimvtli a i d cspansion of the omplialomeseiitei.ic artcry, to tlie exclixsioii of the allnntoic or poteiit ittlly ti-iie um1)ilical arteries, is an aiiomaly which bcgaii vcr;v
clarly in tlic. caareei*of tlie e m l n y ) , is attestetl not oiily 1))- the
t’;icxt of the coalesced Iiind-limb bucls, but also l)y that of tlie
1Jci.sisteiice of primitive coiitlitioiis iii tlic i*egioii of tlie cloaca
a i i t l iirog:.c,iiital orgaiis, such a s tlie jniictioii of the ureters
;iiitl \rolfiaii ducts with the posterior eiitl of the iiitestiiial
t i x c t , aiitl the occ~asioiialprcseiice of two sepai.ate miilleriaii
(Illcts.
111 spite of aiid yet iii li~irmoiiy-paradoxical a s it may
st~rn-witli the dicta of Ronx, formnlatctl in his ti-eati
‘ k;iitli ic~lilniigsmecliaiiik,’oii tlie fiuictioiial adaptation of tlir
oi.gaiiism to alteretl situatioiis, the writer is prone to ltelievc.
t h a t ill cases of deficieiit nutritive supply becanse of faulty
implantatioii a i i t l plat.c.iitatioii, that region of the cml)ryoiii(*
oi.g:;iiiism \vliich is most favoi.al~lyplaced ill tlie fetal ciiuilat ioii, iinmely, the aiitciior half of the body, will coiisequeiitly
siift’ei. less t l i a ~ it h e ~ m t e i i o rl i a l f , tlie uiifavoraI,lc positioii
of wliicli is aggravatctl still moi-c 1))- the aiiomalicis iii thc
riml)ilicwl highway.
‘I’lic i*catlci. slioiild not Ite lctl t o siippose, however, that ;I
siiiglc am1)ilical a r t e r y is found oiilj- in the sirenomeliaii
~noiistci*s
; on tlir. contrary, it has heeii reported again a i i t l
agaiii iii otlier~visenormal fetuses a t hirtli. 1’t.t tlie writci.
is riiiwl)le t o compreliciitl l i o ~ vAlilfeld, l\’olff, i l i i t l otlitArs (’a11
ox sIl1ERIFOBM MONSTERS
379
utilize this fact as prime evidence against the view which
lioltls the single high ‘nmbilical’ artery to be the most farreaching feature of these moiisters. Nutritiorial disturbances,
like all other occ~i~-reiices,
a r e matters of relative degree; they
may raiige from a condition nearly normal to one that is extreme. Even with the existence of siicli a derangement early,
the adaptation of the growing organism may in some cases become nearly perfect and overcome its effects, especially wlieii
the initial deficiency of the nntritional supply was not excessive, or the iiiitial faulty implantation coiild he circumvented
hy further differentiation and growth of tlie extrafetal membranes or the nteriiie mncosa.
But by designating tlie ahseiicc of the true umhilical arteries
and the persistence of a siiigle omplialomeseiiterie artery as
the primary defects, arid the other deformities as secondary,
we 1iaTTe not determined the ‘final’ cause-we have riot cxplaiiietl the umbilical anomalies. It is c l ~ a rthat the causes
of teratogeiietic coiiditioiis a r e either intrinsic or extrinsicthat is, either germinal or environmental. Even though we
are not ahle to marshal1 decisive evidence against the ‘germilia1 thcory, ,it is, nevertheless, significant horn often tlie statcments recur in the clinical history of the cases of siren
formation that tlie mother’s children horii previously a r e
normal a i d healthy a i d tliat there is no history of malformations in the family.
Yet, traits cvei‘ exist that m a r the face of aii otherwise
nicely coiistructed theory. The disturbing features here a r e
the frequent association of deformities in the anterior half‘
of the hody with thosc: of the posterior, as indicated in the
table. Hydrocephaly, defective bulhi oculi, harelip, radial
defects, syndactyly, dinpliragmatic hernia, etc., appear several times in tlie series of cases and apparently lend snbstance to the view of their germinal basis. But I feel that
even these discrepant attributes will ultimately, after deeper
search is made into the vascnlar conditions, find their elucidation as iiutritioiial tleficieiicies.
1908, AIall,+ iii his cwmpi*eliciisivc treatisv o i i the oiigiii
moiisters, tleclarctl : “It is rny purpose to (lemons t r a t e that all monsters ai-e produced by external iiiflucrices
i i 1 ) o t i ~iormalo v a tvliich affect t h iintrition
~
of tlie eml)ryos
~ 1 1 1 ~t1o faiilty implaiitatioii of thcl ovum.
That the powc~rt o
Iwcomr H rnoiistei. is fiilly dcmoiistrntetl Iiy experiments upon
;I variety of vClrtcl)riitc!s, as ~ ~ ( 1 1a1s by all of my piitliological
ot.21. cy)ctcially tliose o1)taiiiecl from t ubal pregiiaiicies. ’ ’
\ Y l i c ~ i i T V C ~searcli for cvicleiice of imperfect implantation a i d
1)Iawiitat ion iii the series of sireniform monsters reportcd,
ii1ifoi.t unatclly we fintl that iii most cases the observer failed
to s a ~ aiiytltiiig
a h n t tlie character of the placenta ant1 other
c~sti.;ifettilmeml)raiies. Gciici.all~-,tlic fault is not his, siiice
I I ( ~was iiot presciit at tlie birth, aiicl tlie atteiidiiig pliysiciaii
failcvl to save tlic afl er-hirtli for a tliorougli esaminatioii.
H i i t i t is significant that, among tlic seveitt;\- o r r n o i a c . paperh
to n-liiclr t l i r writer Iiacl ;i(wss, tlierc. were reportcd five cases
o f twiiis, tlirech of placeiita praevia, eight placentae ahnormal
iii otlici. respects, six vases of tlt+icieiit amniotic fluid, o ~ i c01’
Iiydramiiios, aiitl t h r w cases of margiiial iiisri*tioii of tlie
( ~ ) i x l ,most of 11 liicali contlitioiis a r c eitlicl. f o l l o n - c d hy a l l iiormal iintritional s l ates or are tlic r c s d t ot’ vascnlar
( 1i s t ti 1-1) a I 1
s.
V7e m a y ask, does tlic pc~i*sistciiceof an omphalomeseriteri~
artvi*y a i i t l the repiwsion of the allantoic or poteiitial um1)ilical arteries meaii that, early iii tlie emhryoiiic life of these
i’s, the yolk sac, tliougli entirely inadequate iii mail as
:I i i u t ritioiial resei-voir, is trmisiciitly pressed into service as
sircli ; oi., as seems m o w prol~al>le,did tlie yolk sac i ~ i i t lits
vessels play ;i predomiiiaiit rBle iii determining the locality
of the placciita mi t l i c cliorioii m c l in excludiiig the n o ~ m a l
siipi-cmacy of tlie body stalk aiitl tlic alluiitoic \-esscls iii this
pi*occ1ss! Already Ballaiityiie ( ’97) advanced the idea that
tlic sireniform monsters possilily arc associated with a Titelh i e placciita. Going back to tlic theory of Darestc, that
cornpi-essioii 1)y tlie arrested caudal fold of the amnion is re111
of
liiimnii
(a (1
k’. 1’. 3I:ill: .Jonriial of Morphology,
I01.
l!), 1908.
spoiisible for the deformity of tlie posterior region of the
lwdy, Ballaiityiie believed that tliis compression must necessarily influelice also the body stalk, wliile the vitelliiie vesicle
remains ontside the pressure sphere. Similarly, Knliga ( ’03)
maiiitaiiietl that n tfcfective development of tlie body stalk,
cspecially thc failurci of the allantois aiitl its vessels to clcvcl 01)’ ai it1 t 11e coils cq u eiit part icip a t ioii of the vi t elline ves sels
iii the placental c i i ~ n l a t i o nis
, sufficieiit to effect the anomalies
herc considcrctl. H e refers to the findings of Kollmann and
Kiioop’ who have described cleat1 aborted embryos that
possessed no clioi-ionic stalk. Rut these cases were quite diffeiwit in appearance. W e cannot agree with Tculiga that tlie
allantois is always absent iii the sirmi formation. Tlic
writer’s observations point to tlie existence of this structnre
i n his specimt~ri,and the same i m s stated to have heen found
hy other iiivestigators (compare tahle).
Until there is evidence to tlic eontrai~y,I think we are permittccl to assume tliat tlic persistelice of the omplialomesenteric artery indicates a participation of tlie yolk sac in the
cstahlislimerit of the placenta. This possibility should not
seem a strange one, for it is stated that, among the mammalia, the rodents, insectivores, and chiroptera possess a11
allaiitoic. placeiita wliicli is preceded by R temporary vitelline
placenta, and in the marsupials, which d o riot possess a true
placenta, the yolk sac takcs C R T C of the transfer of iiiitrimeiit
from mother to fetus before the latter is placed into the marsnpinm. If we descend still lower in tlic scale of vertebrate
animals, we firid a similar arrangement iri the elasmobraiich
fishes, iii which tlierc is a placenta-like connection between tlie
yolk sac arid the wail of tlie uterus. I n man the allaiitoic or
umbilical vessels iiormally are established hefore the vitelline,
hut it is coiiceivable that, nrider certain conditions of ahnormal implaritatioii arid abnormal growtli of tile cliorionic
’Knoop : j h e r ciiic fiiilizeitige :~mniotische Misshildung iielmt Bernclkungen
Uber d:is Waclrstunr (lei Eililute n : i ( ~ h dem Fruclrttode, uiid die, Bildurig dcr
sogcw;~nnteiifreien AI1:intois. Beitrape ziir Gc4)urtsli. u. Gyn., Bd. 7. 1903.
1Zollmaiin : Die Korpei f o r m meiisrllliclier noiiiialer untl p:itliologiscliw Eirilmj onen. Arch. f . An:rt. u. Plrys., 1889.
382
OTTO F. liAM1’1LIElER
tlic reverse older of vascularizwtioii call take place. Or,
is it possihie that hccause of such conditions tlie early cstahlislicitl allaiitoic Vessels atrophied, after tiie viteliiiie circulatioii was laitl down and its arteries became tlie chief highway
of I)lootl from the embryo to the placeiita? Initial or early
stages in tiie formation of this monster no doubt occiir from
tirnv t o time among tlie great iiixmber of abortions, aiitl a
clcfinite ansmor to our surmises will he fomid as sooii a s more
of thc. e m h r y n i c material so oljtaiiied is systematicdly settioiitd ~ i i t lexamined.
with striking frequency, if
.4nother feature ~ l i i ( ~ 1recurs
1
tiot cwiistaiitly, in tlic sirciiomelian monsters, hut wliicli is
vlinixcteristic also of many cases of spiiia bificla, ectopia viswt’1im, ctc.., is the recnrred coccyx ant1 sacrum, which fiiids
ii s chstremcl rxpressioii in the preseiice of a tail-lilrc appeiidiigo. A cloi-sai cwiicavity of the 1uml)osacral region of the
\*vi*tdwalaxis, c~mphasizingtiie proniiiieiicc of tlic protnhera i i t yolk sac*, o(*c~irs
often amoiig tlic earliest human emlxyos
f i g u i ~ t li n the literatiire-most
familiarly in the series pict i i i w l 1)) Wilhelm His--antl perhaps is still regarded 11y some
cirril)i.voio~istsa s a iiormal tliougli ti*aiisieiit character. I belit.vc. 1Iall was correct iii judging such embryos abiiormal.
‘ h e supposition is justified that if they had iiot heeii aborted
o I’ t*ouioved,hiit could havc coiitiiiucd on their tlevelopmcntal
couixe, they \voiild 11aw fiiiislied as a monster o r deformity
of’ some ltintl.
B’iiidly, the ivritc>t*\vislics to tiired attciitioii to the giwit
Imxlominaiice of males among tiie sireniform monsters. ()f
a wries of fifty-two accessible 1-eports of cases, ill which the
SPX Eiacl I)ec>ii detrrmiiietl, thirty-eight were males aiitl fourteeii
fcm;tles. Trne, a much larger series is iiecessary hefore a
tfcfiiiite percwitage of relative frequency can bci establislietl,
hut I believe tiie inimbers given already reveal some relatioii>hip I)ctwcwi sex ant1 llie moiistiwsity liere considered. I t is
o f ititerest to quote Hyrtl’s observations oil fourtecii newl)orn, who possessed h i t one umbilical a r t e r y mid most of
wliom w c ~ e;ippareiitly normal otherwise. A411w ( w males.
siic’,
O N SlRENlFOILM MONSTERS
383
Such fiiidiiigs a r e difficult to explain, aiid we can a t present
attempt 110 more than to suggest an hypothesis. If we accept
the mendelian scheme of inheritance, consider sex itself a
meiideliaii clitiracter, and the individual to hegiii with hisexual aillagen, one of which is dominant and the other recessive, a r e we permitted to assume that early nntritioiial
factors-here nutritioiial deficiency-favor tlic ascendancy of
the male attributes in spite of the fact that they may h a w
heeii recessiye in the fertilized ovum?
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THm ANATOMICAL RECORD, VOL. 34, NO. 5
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