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Hallermann-Streiff syndrome: Case report and literature review

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Hallermann-Streiff syndrome is a rare genetic disorder characterized primarily by head and face abnormalities. Patients show birdlike faces; hypotrichosis; various ophthalmic disorders; and dental abnormalities including absence of teeth, natal and neonatal teeth, enamel hypoplasia, and supernumerary teeth. In addition, delayed eruption of existing teeth and severe agenesis of permanent teeth are frequent findings. Dental and hereditary disorders can be associated with disturbances during tooth development and cause shortened roots. Short roots are a rare developmental anomaly in the permanent dentition, and the etiology is not well established. The generalized form is extremely rare. Generalized diminished root formation can lead to early loss of teeth. This article provides a case report of a 9-year-old boy with Hallermann-Streiff syndrome. Extraoral examination revealed a brachycephalic head, proportionate short stature, sparse hair, and atrophic skin. His face was characterized by a thin beak-shaped nose and retrognathia, resulting in a characteristic birdlike appearance. Radiographically, all teeth of the permanent dentition showed severely underdeveloped roots and partially underdeveloped crowns. The predisposition to severe dental caries and dental malformations makes it imperative to schedule effective prevention measures, especially since root canal treatment to preserve teeth can be hampered due to underdeveloped roots.
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been described previously in the literature,
in 1958, Francois4 summarized 12 published
cases and described an additional one.
After analyzing these cases, he delineated
the characteristic features of Hallermann-
Streiff syndrome and described several
essential signs as diagnostic criteria. In
1960, Falls and Schull15 reported six further
cases under the heading of Hallermann-
Streiff syndrome. Thereafter, the condition
was generally known by Hallermann-Streiff
syndrome or Hallermann-Streiff-Francois
syndrome. In addition, synonyms includ-
ing Francois syndrome,6–9 oculomandibu-
lodyscephaly,10–13 Francois dyscephalic
syndrome,11 and oculo mandibulofacial syn-
drome14 can be found in the literature.
Aubry1 was the first to publish a report of a
patient with Hallermann-Streiff syndrome.
Two independent cases of this syndrome
were later published, recognizing it as a
distinct disease entity. Hallermann2 reported
two patients with bilateral congenital cata-
racts and bird face. In 1950, Streiff3 report-
ed a similar case and distinguished the
syndrome from progeria and mandibulofa-
cial dysostosis. Although the syndrome had
1 Department of Operative Dentistry, School of Dentistry,
University of Münster, Münster, Germany.
2 Central Interdisciplinary Ambulance, School of Dentistry,
University of Münster, Münster, Germany.
Correspondence: Dr Edgar Schäfer, Waldeyerstr 30, D-48149
Münster, Germany. Email: eschaef@uni-muenster.de
Hallermann-Streiff syndrome:
Case report and literature review
Peter Robotta, Dr Med Dent1/Edgar Schäfer, Prof Dr Med Dent2
Hallermann-Streiff syndrome is a rare genetic disorder characterized primarily by head
and face abnormalities. Patients show birdlike faces; hypotrichosis; various ophthalmic
disorders; and dental abnormalities including absence of teeth, natal and neonatal
teeth, enamel hypoplasia, and supernumerary teeth. In addition, delayed eruption of
existing teeth and severe agenesis of permanent teeth are frequent findings. Dental and
hereditary disorders can be associated with disturbances during tooth development and
cause shortened roots. Short roots are a rare developmental anomaly in the permanent
dentition, and the etiology is not well established. The generalized form is extremely rare.
Generalized diminished root formation can lead to early loss of teeth. This article provides
a case report of a 9-year-old boy with Hallermann-Streiff syndrome. Extraoral examination
revealed a brachycephalic head, proportionate short stature, sparse hair, and atrophic
skin. His face was characterized by a thin beak-shaped nose and retrognathia, resulting
in a characteristic birdlike appearance. Radiographically, all teeth of the permanent
dentition showed severely underdeveloped roots and partially underdeveloped crowns.
The predisposition to severe dental caries and dental malformations makes it imperative to
schedule effective prevention measures, especially since root canal treatment to preserve
teeth can be hampered due to underdeveloped roots. (Quintessence Int 2011;42:331–338)
Key words: anodontia, dental anomalies, Francois syndrome, short roots,
underdeveloped roots
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Hallermann-Streiff syndrome is a rare
genetic disorder—only about 180 cases
have been published to date.9,15 It is a con-
genital syndrome characterized by distinct
craniofacial abnormalities. Dyscephaly with
hypoplastic mandibula and a small beaked
nose leading to a typical birdlike face (89%
to 90%); congenital cataracts (81% to 90%);
hypotrichosis (80% to 82%); microphthal-
mia (78% to 83%); skin atrophy (68% to
70%); a short proportional stature (45% to
68%); and dental abnormalities (80% to
85%) (absence of teeth, natal and neonatal
teeth, enamel hypoplasia, supernumerary
teeth, delayed eruption of existing teeth,
and malformed permanent teeth) are the
main clinical features of Hallermann-Streiff
syndrome.16 The etiology of this syndrome
is unknown. Virtually all cases have been
sporadic, and there is no sex predilection.
Francois demonstrated marked changes in
the structure of the connective tissue (altera-
tions of the elastin) and postulated a primary
disturbance in the metabolism of glycopro-
teins. A dominant succession is presumed.
The diagnosis of Hallermann-Streiff syn-
drome is based on the presence of certain
features, including the characteristic facial,
eye, hair, skin, and dental findings.
Since the dominant symptom of patients
with Hallermann-Streiff syndrome is cata-
racts, most cases have been reported
in the ophthalmology literature.16 Although
over 180 reported cases can be found in
the literature, very few considered dental
aspects.13,17–24 The present report focus-
es on the high incidence and variety of
dental abnormalities in Hallermann-Streiff
syndrome. Most of the patients revealed
dental malformations such as supernumer-
ary and neonatal teeth, premature eruption
of the primary dentition, partial anodontia,
agenesis of permanent teeth, and enam-
el hypoplasia. In addition, malocclusion,
hypoplasia of the mandible, crowding, and
high arched palate are typical of this syn-
drome. Although these dental abnormalities
are often observed in Hallermann-Streiff
syndrome, they are poorly defined. The
particular aspect of the presented case
is the occurrence of general underdevel-
opment of the roots and agenesis of the
permanent dentition. The morphogenesis of
normal tooth root development is regulated
by epithelial and mesenchymal interac-
tions. After the growth of the dental crown
is complete, a double layer of cells referred
to as Hertwig’s epithelial root sheath is
formed by cells of the outer and inner den-
tal epithelium. The formation of root dentin
requires proliferation of epithelial cells in
the Hertwig’s epithelial root sheath to initi-
ate the differentiation of root odontoblasts
and determine the size, shape, and num-
ber of the roots. Disturbances can lead
to root deformities. Slootweg and Huber13
conducted histologic examinations of the
mandible and a removed erupted tooth of
an infant with Hallermann-Streiff syndrome.
They reported a root dentin anomaly classi-
fied as a combination of irregular dentin and
osteodentin, premature disintegration of the
dental lamina, and Hertwig’s root sheath.
With regard to dental treatment, mal-
formed crowns and roots confront the prac-
titioner with special problems. Root canal
treatment of teeth with severely underdevel-
oped roots is often impossible. The associ-
ated difficulties in dental treatment as well
as the necessity of an early diagnosis and
multidisciplinary treatment approaches are
discussed.
CASE REPORT
A 9-year-old boy with Hallermann-Streiff syn-
drome and bilateral cataracts was referred
to the Department of Restorative Dentistry,
Dental School, University of Münster,
Münster, Germany, for dental examination.
Apart from the syndrome, the boy was in
good health. He was the youngest son in
a family who emigrated from Kazakhstan
in 1994. The two older siblings, a 13-year-
old girl and a 10-year-old boy, showed no
conspicuousness. The anamnesis did not
reveal any other known syndromes in the
family of his nonconsanguineous parents.
The pregnancy and childbirth took their
normal course. His physical growth and
development was retarded; however, his
intellectual stage of development seemed
to correspond to his age. The prime con-
cern was the general mobility and progress-
ing loss of teeth.
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The extraoral examination revealed a
brachycephalic head, a proportionate short
stature, sparse hair, and atrophic skin, par-
ticularly in the nose region. His face was
characterized by a thin, beak-shaped nose
and retrognathia, resulting in a character-
istic birdlike appearance, most strikingly in
profile.
The clinical intraoral examination
revealed reduced mouth opening (24 mm),
a small tongue, and good oral hygiene.
From an orthodontic point of view, a sagittal
and transversal underdeveloped maxillary
jaw with a frontal headbite and a crossbite
on the right side was diagnosed. In general,
the development of the dentition was not in
correspondence with the age of the boy.
Primary teeth 53, 55, 63, 64, 65, 73, 74, 83,
84, and 85 (FDI) still existed and showed
moderate mobility. Permanent teeth 16, 12,
11, 21, 22, 26, 31, 41, and 46 (FDI) were in
eruption and showed a higher mobility of
grade II to III (Fig 1).
Fig 1a Maxillary view.
Fig 1b Mandibular view.
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A panoramic radiograph taken at 9 years
of age (Fig 2) revealed that all teeth of the
permanent dentition as well as the primary
teeth 55 and 85 showed severely underde-
veloped roots and partially underdeveloped
crowns. The already erupted teeth 16, 12,
11, 21, 22, 26, 31, 41, and 46 displayed at
most one third of the normal root length at
this stage of development. The teeth germs
13, 14, 15, 17, 23, 24, 25, 27, 32, 33, 34,
35, 36, 37, 38, 42, 43, 44, 45, and 47 did
not show any signs of root development.
Comparison of a panoramic radiograph
taken at 12 years of age confirmed that no
further root development had taken place.
During this 4-year period, teeth 53, 64, 85,
and 31 exfoliated, but no new tooth eruption
was detected (Fig 3).
DISCUSSION
Hallermann-Streiff syndrome is a rare con-
genital anomaly that mainly affects the head
and face.25 The seven characteristic features
of this syndrome are congenital cataracts,
dyscephaly, proportionate nanism, hypotri-
chosis, skin atrophy, bilateral microphthalmos,
and dental anomalies. In the present case,
striking craniofacial findings were noticed,
including a small, wide head with a promi-
nent forehead, a characteristic small jaw
and mouth with a pinched nose, cataracts,
sparse hair, thin skin, short stature, and
severe dental abnormalities. Differential diag-
nosis of Hallermann-Streiff syndrome includes
progeria, mandibulofacial dysostosis, and
Wiedemann-Rautenstrauch syndrome.15,26
Fig 2 Panoramic radiograph at the age of 9 years.
Fig 3 Panoramic radiograph at the age of 12 years.
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Progeria differs from Hallermann-Streiff
syndrome in that the former presents with
premature arteriosclerosis, nail dystrophy,
acromicria, chronic deforming arthritis, and
normal eyes. Wiedemann-Rautenstrauch
syndrome patients may have a somewhat
similar facial appearance, but do not show
the ocular findings of Hallermann-Streiff
syndrome. Mandibulofacial dysostosis and
Hallermann-Streiff syndrome patients both
exhibit micrognathia, high palatal vault, and
molar hypoplasia, but the former usually has
lower eyelid colobomas and associated ear
anomalies.
The radiographic verification of short
roots based on the relative root length is
based on the ratio between root length
and crown length. A ratio of 1:1 or less
is defined as a short root anomaly. If the
length of the root is shorter than the length
of the crown, the term rhizomicry is used,27
which is considered extremely rare in gen-
eralized form.28,29 The patient in the present
case revealed a generalized root-to-crown
ratio of less than 1:1. Based on the lit-
erature, generalized short root anomaly
can be divided according to the etiology
into familial, syndrome-associated, environ-
mental, and idiopathic.30 Short roots may
also be observed in dental and heredi-
tary disorders such as dentin dysplasia
type 1 and dentinogenesis imperfecta.31,32
The affected teeth appear clinically nor-
mal, but because of their short or almost
missing roots, tooth mobility is markedly
increased and spontaneous exfoliation may
occur. Short roots appear along with rare
underlying systemic conditions such as
hypoparathyroidism,33 Stevens-Johnson
syndrome,34,35 scleroderma,36 Down syn-
drome,37 and Laurence-Moon-Bardet-Biedl
syndrome.38 Some short-stature syndromes
(Aarskog syndrome,39 dwarfism of Seckel,40
and Rothmund-Thompson syndrome41)
have also been associated with short
roots. In addition, case reports described
patients with short stature or short roots or
both, but with no recognized syndrome.42,43
Environmental effects during tooth develop-
ment, such as trauma, periapical infection,
and orthodontic or surgical procedures as
well as radiation therapy and to a lesser
extent chemotherapy for childhood malig-
nancies,44,45 may result in diminished root
development. An idiopathic generalized
short-root anomaly is extremely rare.46,47
The size and morphology of dental roots
exert significant implications in several fields
of dentistry: periodontology, orthodontics,
traumatology and restorative dentistry, and
endodontics. Endodontic treatment in teeth
with short or nearly missing roots is associ-
ated with several risks and special prob-
lems. Preparation of access cavities must be
carried out with circumspection because the
floor of the pulp chamber could at the same
time be the end of the root canal system,
as in teeth 16, 55, 26, 27, 36, 37, and 46 of
the present case (Figs 4a to 4c). Root canal
preparation and obturation must be adapted
to the situation, especially in teeth with very
short, thin roots (teeth 11 and 21) (Fig 4d).
The main problem of the dental treatment
in the present case is related to the fact that
most teeth of the permanent dentition are
retained. The already erupted teeth prob-
ably cannot be conserved for a long time
due to the disturbed root growth. A toothless
jaw will be the consequence. Certainly, an
early loss of dentition is associated with ana-
tomical and functional problems. The lack of
dental support due to the upgrowth could
lead to underdeveloped alveolar processes
in addition to problems with the temporo-
mandibular joint because of overload.
In this case, the expected early loss
of teeth cannot be treated curatively. The
dental therapy approach is to care for and
conserve the mixed dentition as long as
possible. In patients with short root anom-
aly, early treatment of caries is essential to
prevent endodontic treatment. Preventive
measures such as perfecting oral hygiene
and fissure sealants are obligatory. A nutri-
tion consultation could be advantageous.
Further important aims of dental treatment
are reestablishment and normalization of
mastication, speech, and swallowing as
well as the compensation of the disturbed
physiognomy. A complex prosthetic and
orthodontic strategy for congenital abnor-
malities with oligodontia and anodontia is
necessary for adequate treatment.48–50 In the
case of growing patients, fixed partial den-
tures are not indicated, especially because
fixed dentures crossing the center line could
lead to transversal growth inhibition.51 Age
of onset, developmental stage, and patient
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compliance all may indicate that a remov-
able prosthesis is the treatment of choice.
The three main advantages of a prosthe-
sis are reestablishment of the mastication
function,52–55 improvement of esthetics52–59
through the advancement of a physiolog-
ic perioral musculature,54,56,58–63 and the
advancement of phonetic qualities.52,57,61,64,65
The continuous jaw growth and possible
dentition change necessitates close moni-
toring with 3- to 6-month recall intervals
for denture adjustments. Potential surgical
procedures for the functional and esthetic
outcomes are planned for later in life. The
final prosthetic restoration will start once the
patient reaches adulthood.
In the management of patients with
Hallermann-Streiff syndrome, interdisci-
plinary medical care is important. Periodic
ophthalmologic examination should be per-
formed for early diagnosis of eye compli-
cations. Although one should pay special
attention to ophthalmologic and upper respi-
ratory problems, early consultation with a
dentist is important. The predisposition to
severe dental caries and dental malforma-
tions makes it imperative to perform effective
prevention strategies as early as possible.
Efforts must be made to prevent root canal
treatment.
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... and craniofacial dysostosis. [15][16][17] Hence, neonatologists can utilize this information during the diagnostic workup of the newborn. ...
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... In our case report, patient has hypodontia and the erupted teeth had abnormal morphological structures and the lack of tooth structure may cause speech disorders, problems in the temporomandibular joint. 23,24 Due to the patient's nutritional problem, the progress of body development may slow down and a child prosthesis can overcome this problem by eating different kinds of nutritional products. A child prosthesis can increase the upper face height and get rid of the patient from elderly facial seem. ...
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This classic text, one of the true anchors of our clinical genetics publishing program, covers over 700 different genetic syndromes involving the head and neck, and it has established itself as the definitive, comprehensive work on the subject. The discussion covers the phenotype spectrum, epidemiology, mode of inheritance, pathogenesis, and clinical profile of each condition, all of which is accompanied by a wealth of illustrations. The authors are recognized leaders in the field, and their vast knowledge and strong clinical judgment will help readers make sense of this complex and burgeoning field. Dr. Gorlin retires as editor in this edition and co-editor Raoul Hennekam takes over. Dr. Hennekam is regarded as one of the top dysmorphologists--and indeed one of the top clinical geneticists--in the world. Judith Allanson is new to the book but is a veteran OUP author and a widely respected geneticist, and Ian Krantz at Penn is a rising star in the field. Dr. Gorlin's name has always been closely associated with the book, and it has now become part of the title. As in all fields of genetics, there has been an explosion in the genetics of dysmorphology syndromes, and the author has undertaken a complete updating of all chapters in light of the discoveries of the Human Genome Project and other ongoing advances, with some chapters requiring complete rewriting. Additional material has been added both in terms of new syndromes and in updating information on existing syndromes. The book will appeal to clinical geneticists, pediatricians, neurologists, head and neck surgeons, otolarynologists, and dentists. The 4th edition, which published in 2001, has sold 2,600 copies.
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A case of generalised short root anomaly is described. All permanent teeth had abnormally short roots, associated with microdontia, hypodontia and a dens invaginatus. Members of the patient's family were similarly affected.
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Hallermann-Streiff syndrome is a rare genetic disorder characterized by craniofacial malformations, sparse hair, eye abnormalities, dental defects, degenerative skin changes, and short stature. The syndrome has many implications for dental treatment. Patients typically present with multiple missing and poorly formed teeth. The purpose of this case report is to discuss the overall management of a patient with Hallermann-Streiff syndrome by oral maxillofacial surgery, orthodontic treatment, and prosthodontic reconstruction.
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A case report is presented of a patient in whom nearly all teeth of the permanent dentition still present had short roots, while some teeth had never been formed at all. It may be concluded from the typical differences in length between the roots of the various teeth that this condition must be due to complete cessation of the growth of the teeth at the age of 7 or 8 years. Since the patient suffered a fulminant attack of erythema multiforme (STEVENS-JOHNSON syndrome) at this age, and since no other possible explanation of the short roots has been found, it is concluded that this clinical condition may have been the reason for the short root anomaly found. Damage or even destruction of the epithelial root sheath during the disease may be assumed to be the direct cause of the failure of full root development.