W, Schwechendiek: Further Findings Concerning the Occurrence of Clefts
References
Battersby, T. G.: Cavernous angioma of the mandible.
Report of a case. Brit. dent. j. 19 (1957) 347
Erich, ]. B.: Central hemangioma of the mandible.
Report of a case. Oral Surg. 33 (1947) 611
Kaburda, M., R, G. Taylor: Hemangioma of the eden-
tulous Mandible. Canad. dent. Ass. J. 33 (1967) 65
Landing, B. H., S. Farber : Tumors of the cardiovascular
system. In: Atlas of tumor pathology, Sect. III,
Fast. 7. Armed Fore. Inst. of Path., Washington D.C.
1956
Lucas, R. B.: Pathology of tumors of the oral tissues.
Livingstone, Edinburgh 1972
Marie, M. M. R., J. Bardty: Les h6mangiomes du
maxillaire inf6rieur. Rev. Stomat. (Paris) 54 (1953)
940
Obwegeser, H. L.: The surgical correction of mandibular
prognathism and retrognathia with consideration of
genioplasty. Oral Surg., Oral Med., Oral Path. 10
(1.957) 677
Obwegeser, H. L.: Die KinnvergrSgerung. Ust. Z.
Stomat. 55 (1958) 535
Obwegeser, H. L.: Zur Korrektur der Dysostosis oto-
mandibularis. Schweiz. Z. Zahnheilk. 80 (1970) 331
Pizer, M. E.: Cavernous hemangioma occurring in the
Further Findings Concerning the Occurrence of Clefts in Twins
Wolfram Schweckendiek
Dr. Schweckendiek's Clinic, 355 Marburg/Lahn, W. Germany (Head: W. Schweckendiek, M.D.)
Summary
This paper presents a report on 22 pairs of twins with
cleft formation, 6 of which were monozygotes. In two
cases out of these, both of a pair were involved. The
problem of concordance in twin is discussed.
Key-Words: Cleft formation in twins.
Introduction
In investigating the causes for formation of clefts
in the face, a h e r e d i t a r y factor seems to be the most
certain. In addition, recent experimental inves~
tigations have brought to light that various exo-
genous factors also contribute to the formation of
clefts. The combined action of exogenous and
genetic factors is referred to as a multifactorial
occurrence. Clefts of the palate which could also
occur as a m a l f o r m a t i o n with arrest of develop-
109
body of the mandible. Oral Surg., Oral Med., Oral
Path. 7 (1954) 1201
Pusey, R. F.: Mandibular central hemangioma. Brit. J.
Oral Surg. 5 (1967/68) 25
Rittersma, J., L. P. ten Kate, P. Westerink: Neuro-
fibromatosis with mandibular deformities. Oral Surg.
33 (1972) 718
Thoma, K. H., H. M. Goldman: Oral Pathology.
Mosby, St. Louis 1960, p. 1309
Villa, V. G., F. Limchayseng: Central hemangioma of
the mandible (Central angioma). Report of a case.
Oral Surg., Oral Med., Oral Path. 8 (1955) 1254
IdYeinstein, 1, H. Yamanaha, H. Fuchihata: Resection
and reconstruction of the mandible for removal of
a central hemangioma. Oral Surg., Oral Med., Oral
Path. 16 (1963) 2
Williams, I/V. ]., R. K. Gilbert: Hemangioma of the
mandible. Brit. dent. J. 21 (1955) 445
Hermann F. Sailer, M.D., D.M.D.
Kieferchlrurgische Abteilung,
Plattenstr. 11, Postfach
CH-8028 Zi~rich, Switzerland
J. max.-fac. Surg. 1 (1973) 109-112
@ Georg Thleme Verlag, Stuttgart
ment, are less frequently inherited as clefts of lip,
alveolus and palate.
In early investigations, the monozygotic pro m`
perties of a pair of twins were generally deter-
mined by means of the p o l y - s y m p t o m a t i c similar-
ity diagnosis worked out by Siemens (1924). This
method is today still v e r y important. But it must
be complemented by a thorough analysis of blood
factors. T h e total nurnber of monozygotic twins
with clefts reported in the literature is, at present,
about 130. It can therefore be justified to continue
the inclusion of twins in order to supplement
research in heredity with a large number. The
p a r t i a l l y incomplete findings in earlier publica-
tions should now be supplemented through an
exact genetic analysis.
110 W. Schweckendiek

Table 1 Cleft Formation in Twins. In 3 cases, it could not be determined whether the
n Concord- °/o twins were mono- or dizygotic. These were twins
ance of the same sex in which one of each pair died
either ante- or perinatally, so that it was no longer
Monozygotic Twins 6 2 33
Dizygotic Twins 13 0 0 possible to carry out any genetic investigations on
Twins of same sex but 3 0 0 them. The 13 twins considered to be dizygotic,
of unknown zygosis consisted of homo- as well as heterosexual twins,
Total 22 2 - who, on reasons of similarity and blood analysis,
had to be reckoned, without doubt, as dizygotes.
A survey of the type of cleft found in our twins is
Table 2 Classification of our Clinical. Cases of Twins
according to Cleft Formation. given in Table 2.
A m o n g the monozygotic twins who could be
Primipara Bipara reported upon, we found clefts only in two pairs.
t. Monozygotic Twins In each case, it was a unilateral cleft of lip and
1. d bilateral cleft lip, ~ cleft lip, alveolus palate in one pair, and a bilateral total cleft lip,
alveolus and palate (CLA) right alveolus and palate in the other. Of the remaining
(CLAP) 4 cases, only one of eada pair of twins was involved,
2. c~ bilateral CLAP c~ CLA right
although monozygosis was proved with certainty.
3. (3 ~ c~ CLAP right
4. d CLAP left ~ Figs. 1 and 2 show the monozygotic twins referred
5. 9 (7) 9 CLAP left to, in which one partner had cleft of lip and palate
6. d ~b d~ CLAP left
in each case, and the other born with bilateral cleft
If. Dizygotic Twins of lip, alveolus and palate. After surgical repair,
1. 9 C P d@ similarity was very striking, although the facial
2. ~ ~ 9 CL right, CP cleft took different courses of development. The
3. ~ CLAP left ~ 0
4. r i c e ~(~ polysymptomatic similarity diagnosis, as well as
5.6 ~ 2cp blood findings speak for monozygotic twins.
6. d ~b ~ CLAP bilateral Fig. 3 shows female twins at the age of three, in
7. d CLA right ~ q) whom monozygosis could also be established. One
8. d CLAP bilateral c~
of these children had cleft lip, alveolus and palate
9. C~ @ d} CLAP bilateral
10. ~ (l) 9 CLAP right of the left side; the other was free of any mal-
11. d CLAP bilateral d (l) formation. The photographs - both children are
12. _~ CLAP left ~ (~ crying - reveal a remarkable physiognomic simi-
13. 5~ eL left d (~ larity in the children.
III. Unknown
1. d CLAP left d~ Discussion
Partner without In investigating heredity in hmnans, studies of
malformation, died
antenatally twins, and in the first place, in monozygotic twins,
2. d CLAP right rightly attracts special interest. Systematic pre-
d~
Partner without sentations of investigations of cleft formation in
malformation, died twins were made by Birkenfel d (1927), SchrSder
perinatally (1931, 1935), Idelberger and Idelberger (1940),
3. 9 CLAP left 9¢ and in more recent times by ]Srgensen (1967,
Partner without 1969, 1972), and ]6rgensen and Gabka (1971), as
malformation, died
perinatally well as by Hartmann (1972).
Although on basis of many investigations of other
authors and of our own clinical material of over
Clinical Material 1500 cases, inheritance in cleft formation must be
Table 1 shows the pairs of twins in our case looked upon as a very essential factor, many kinds
material who were investigated. The collective of environmental mischance are obviously decisive
embraced a total of 22 pairs of twins, out of which in the development which leads to malformation,
6 were proved to be monozygotic and 13 dizygotic. so that clefts are manifest in a few cases, but show
 Further Findings Concerning the Occurrence of Clefts in Twins 1i ]

Fig. 1 Monozygotic pair of

twins: Ralph and Bernd G. atthe
age of 6 months.
a) Ralph: Cleft Lip and Palate of
the right side.
b) Bernd: Bilateral Cleft Lip,
Alveolus and Palate.

Fig. 2 Monozygofic pair of

twins: Michael and Thomas S.
a) Michael: formerly unilateral
Cleft Lip and Palate of right side.
b) Thomas: formerly bilateral
Cleft of Lip, Alveolus and Patate.

Fig. 3 Monozygoticpair of
twins: Anja and Simone K.,
3-years-old.
a) Simone: No Cleft formation.
b) Anja: formerly Cleft of Lip,
Alveolus and Palate of left side.
112 W. Schweckendiek: Further Findings Concerning to Occurrence of Clefts in Twins
no penetrance in others. The cases in which the type
of cleft is similar, but with different grade of
development, prove that the manifestation of cleft
formation in individual cases could be diverse, in
spite of a surely equivalent hereditary predisposi-
tion.
The number of monozygotic twins with cleft for-
mation is naturally small, so that I had to revert to
the results of other investigators. Stellmach and
Frenkel (1970) reported of siamese twins in whom
a completely similar and symmetric unilateral
total cleft of lip, alveolus and palate was present.
A similar anomaly (bieipital Child with cleft
formation) was described by Schorr (1909), who
also found a symmetric cleft defect in a double
malformation. Birkenfeld (1927) has also called
attention to it that the cleft formation in mono-
zygotic twins could be symmetrical. On the other
hand, Idelberger and Idelberger (1940) found a
pair of monozygotic twins in which cleft formation
on the same side in each was involved. But Idel-
berger and Idelberger's number of actual con-
cordance is strikingly small.
Conclusion
This paper seeks to establish that complete con-
cordance in relation to cleft formation in mono-
zygotic twins is rare. The greater number of the
cases are discordant, that is, cleft formation is
present only in one of a pair in twins. The cases
References
Birkenfeld, W.: Vererbungspathologische Untersuchun-
gen an Zwillingen mit Lippen-Kiefer-Gaumen-
spalten. Bruns' Beitr. klin. Chir. 141 (1927) 265
Gabka, H.-]., G. ]6rgensen: Erfahrungen in der Pro-
phylaxe von Lippen-Kiefer-Gaumenspalten unter
besonderer Berficksichtigung genetischer Gesichts-
punkte. Dtsch. Ges. Kiefer- u. Gesichtschir., Ham-
burg 1970
Hartmann, D.: Spaltbildungen des Gesichtes bei Zwil-
lingen. Diss., Marburg 1972
Idelberger, A., K. Idelberger: Serienmfit~ige Zwillings-
untersuchungen zur Erbpathologle der Lippen-Kie-
fer-Gaumenspalten. Z. Vererbnugsl, 24 (1940)
417
]6rgensen, G.: Genetische Analyse yon Krankheiten
mit multifaktorieller (polygener) Basis. Landarzt 43
(1967) 852
]6rgensen, G.: Zur Atiologie der Lippen-Kiefer-Gau-
menspalte. Med. heute 10 (1969) 293
]6rgensen, G., H.-]. Gabka: Zwillingsuntersuchungen
bei Lippen-Kiefer-Gaumenspalten. Med. Mschr. 25
(1971) 447
in which both twins are affected by cleft defect
often show varied grades of manifestation, so that
one is prone to refer to it as conditional con-
cordance. Only in a few cases is there a complete
conformity in cleft formation, whereby, however,
the affected side m a y be different (Table 3). It
will be the duty of this our brave future era to
further enlighten us on the nature of the causative
factors that might be active here.
Table 3 Possibility of Cleft Formation in Monozygotic
Twins.
1. Cleft Formation only in a partner.
2. Similar type of Cleft formation in various degrees.
3.a) Equivalent degree of cleft formation: homolateral
side.
3.b) Equivalent degree of cleft formation: mirror image
(different sides).
It is remarkable that the extent of cleft defect in
Stellmach's siamese twins and the double mal-
formation mentioned by Schorr should conform,
even though a symmetrical and not an equilateral
deformity was present. W e consider it possible
that the moment of separation of both twins is of
importance. If separation takes place at a very
early stage - perhaps shortly after fertilisation -
both of the pair will seldom be involved. But if
separation occurs at a later stage, or is completely
absent, both of the twins will show malformation.
]6rgensen, G.: Miflbildungen im Bereich der Hals-
Nasen-Ohrenheilkunde. Arch. Hals-, Nas.- u. Ohren-
heilk. 202 (1972) 1
Schorr, G.: Qber Wolfsrachen vom Standpunkt der
Embryologie und der pathologischen Anatomie.
Virchows Arch. path. Anat 197 (1909) 16
Schr6der, C. H.: Die Vererbung der Hasenscharte und
Gamnenspalte. Arch. Rassenbiol. 25 (1931) 370
Schr6der, C. H.: Untersuchungen fiber die Vererbung
der Hasenscharte und Gaumenspalte unter besonde-
rer Berficksichtigung des Erbganges. Arch. klin. Chir.
182 (1935) 299
Siemens, H. W.: Die Zwillingspathologie. Ihre Bedeu-
tung, ihre Methodik, ihre bisherigen Ergebnisse.
Springer, Berlin 1924
Stellraach, R., G. Frenkel: Ober das ¥orkommen yon
spiegelbildlich konkordanten vollst/indigen einseiti-
gen Lippen-Kiefer-Gaumenspalten bei einem siamesi-
schen Zwillingspaar. Dtsch. zahn/irztl. Z. 25 (1970) 28
Wolfram Schweckendi&,M.D.,
D-355Marburga. d. Lahn,
Blitzweg 1