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Case Report

Report of a case of Turners syndrome

with localized aggressive periodontitis
Suresh Kumar Kasagani, Ramesh Babu Mutthineni, Narendra Dev Jampani,
Rajasekhar Nutalapati

Department of
Periodontics, Mamata
Dental College and
Hospital, Khammam,
Andhra Pradesh, India

Abstract:
Turners syndrome is a disorder in females characterized by the absence of all or part of a normal second sex
chromosome. It is typically characterized by the combination of physical features and cytogenetics in females.
Physical features include short stature, primary amenorrhea, hypogonadism, low hair line at the back of the
neck, and digital anomalies. Most affected patients have a 45XO monosomy, but the presence of an abnormal
chromosome or mosaicism of 45X with another cell line can also fulfill the criteria. Features seen in the oral
cavity of patients diagnosed with Turners syndrome include high palatal vault, hypoplastic mandible, prematurely
erupting teeth, and orthodontic anomalies. A case of Turners syndrome with localized aggressive periodontitis
has been reported here.
Key words:
45XO, flap surgery, hydroxyapatite, hypogonadism, localized aggressive periodontitis, short stature, Turners
syndrome

INTRODUCTION
Access this article online
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DOI:
10.4103/0972-124X.84389
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Address for
correspondence:
Dr. Rajasekhar Nutalapati,
Department of
Periodontics, Mamata
Dental College and
Hospital, Khammam,
Andhra Pradesh, India.
E-mail: rnutalapati@
rediffmail.com
Submission: 26-12-2009
Accepted: 07-12-2010

enry Turner in 1938 described seven cases

of rather uniform appearance, i.e. women
with short stature, sexual infantilism, webbing
of neck, and cubitus valgus. He named the
condition Turners Syndrome.[1] In 1959, it
was recognized that Turners syndrome resulted
from the loss of an X chromosome or essential
parts of it.[2] This syndrome is characterized by
the presence of only one normal functioning
X chromosome. The other sex chromosome
can be missing or can be abnormal. [3] Most
affected patients have a 45XO monosomy,
but the presence of an abnormal chromosome
or mosaicism of 45X with another cell line
can also fulfill the criteria. Growth failure is
a consistent finding at birth in infants with
Turners syndrome. However, the time of onset
and pattern of growth deficiency is unknown.[4,5]
The presenting clinical features of Turners
syndrome can vary widely among the affected
individuals. Consequently, whereas short stature
and gonadal dysgenesis are almost universal in
Turners syndrome, many other organ systems
are also affected to varying degrees and at
different stages of life.[4] Many features in the
oral cavity typical of the 45XO phenotype have
previously been reported. These include high
arched palate, [6] hypoplastic mandible, thin
enamel and decreased amount of dentin,[7] tooth
mobility and periodontal pockets,[3] reduced
tooth size,[8] small mesiodistal dimensions of
teeth,[9] prematurely erupted teeth, and various
malocclusions.[10]

Journal of Indian Society of Periodontology - Vol 15, Issue 2, Apr-Jun 2011

A 21-year-old female patient who was

prediagnosed with Turners syndrome and
presented with localized aggressive periodontitis
has been reported here.

CASE REPORT
A 21-year-old female reported to the Outpatient
Department of Periodontics, Mamata Dental
College and Hospital, Khammam, with the
chief complaint of multiple mobile teeth in the
mouth since 1 year. On physical examination,
it was seen that the patient was of short stature
and had webbed neck with a low hair line at the
back of the neck. Medical history revealed that
the patient had been diagnosed with Turners
syndrome 5 years back [Figure 1]. Ultrasound
report of the abdomen revealed hypogonadism
[Figure 2]. On intraoral examination, the patient
presented with midline diastema [Figure 3],
high-arch palate [Figure 4], grade III mobility of
maxillary left first molar (tooth 26), maxillary left
second premolar (tooth 25) and mandibular left
central incisor (tooth 31), grade II mobility of the
mandibular left and right, first and second molars
(teeth 36, 37, 46, 47) with a probing depth of >8
mm except for tooth 47 that showed a probing
depth of >10 mm. Clinical recession was not seen
except for tooth 31, which showed Millers class
II recession. Grade III furcation involvement was
seen in teeth 26, 36, and 37. Grade I furcation
involvement was seen in teeth 46 and 47.
Radiographic examination [Figure 5] revealed
extensive bone loss extending up to the root apex
in relation to teeth 25 and 26; bone loss extending
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Kasagani, et al.: Turners syndrome with LAP

Figure 1: Cytogenetics report showing a missing chromosome (45XO) diagnostic of

Turners syndrome

Figure 2: Ultrasonogram of the abdomen revealing hypogonadism

Figure 3: Intraoral picture showing midline diastema (arrow)

Figure 4: Intraoral picture showing a high-arch palate (arrow)

to more than two-thirds of the root length on both mesial and

distal aspects in relation to tooth 31; bone loss extending up
to half of the root length in relation to teeth 36, 37, and 46; and
bone loss extending up to the apical one-third only on the
mesial aspect of tooth 47.

Figure 5: Preoperative orthopantomogram revealing extensive bone loss in the first

molar areas
174

A full-mouth flap surgery along with regenerative procedures

was planned. Routine blood investigations were performed.
All blood reports were within the normal range. Complete
oral prophylaxis was carried out and the patient was given
thorough oral hygiene instructions. The patient was prescribed
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Kasagani, et al.: Turners syndrome with LAP

100 mg Doxycycline for 10 days (two tablets on day 1 and one

tablet daily for the remaining 9 days) and Metronidazole 400
mg for 5 days (thrice daily).
Teeth with poor prognosis, i.e. 25, 26, and 31 (grade III mobile)
were extracted. After phase I therapy, root canal treatment
was performed in tooth 47. During flap surgery, the areas
of bone loss in relation to 16, 36, 37, and 46 were grafted
with porous hydroxyapatite (Osteogen Impladent Ltd.,
Holliswood, New York, USA) [Figure 6]. The mesial portion
of 47 was hemisected and the area was grafted with porous
hydroxyapatite (Osteogen).
The patient was recalled after 6 months for re-evaluation of
oral hygiene, gingival status, mobility, and pocket depths.
Oral hygiene was found to be good, with probing depths
reduced to <5 mm in all treated teeth. All teeth were firm
with no mobility. The midline diastema that was present
preoperatively closed postoperatively (after periodontal
therapy) by Reactive Positioning or Spontaneous
Repositioning [Figure 7]. Follow-up after 1 year showed
good amount of bone fill in the bone-grafted areas on
radiograph [Figure 8]. The patient was fully satisfied with
the treatment outcome.

Figure 6: Osteogen (porous hydroxyapatite)

The baseline status of the treated teeth, the treatment

administered, and their postoperative status has been
summarized in Table 1.

DISCUSSION

Figure 7: Reactive positioning causing closure of the midline diastema (arrow)

Turners syndrome is a complex medical condition characterized

by oral, physical, and psychological features.[7] It is the most
common sex chromosome abnormality in females, affecting an
estimated 3% of all females conceived.[11] The frequency among
live-borne females is 1 in 2000 to 1 in 3000.[4] Approximately
9899% of Turners syndrome fetuses are spontaneously
aborted, and about 20% of all spontaneously aborted fetuses
have Turners syndrome.[7]
The human genome normally consists of 46 chromosomes:
22 pairs of autosomes plus the X and Y chromosomes.
Women are born with two complete X chromosomes.
Usually, Turners patients have only 44 autosomes plus one
X chromosome.[7] Girls with Turner syndrome are either
missing one X chromosome (45X) or a part of it. Sometimes,
parts of the body have normal cells that contain two X
chromosomes (46XX), while other cells are missing one X
chromosome (45X). This is known as mosaicism. Turners
syndrome is not hereditary.[10]
Turners syndrome can be diagnosed before birth through
chromosome analyses on cells from amniotic fluid. [3,12]
One-fifth to one-third of the affected girls receive diagnosis
as newborns.[5] The diagnosis of Turners syndrome may
be delayed into late childhood, adolescence, or even
adulthood. [4] Approximately one-third of the girls with
Turners syndrome receive the diagnosis in midchildhood
on investigation of short stature. In most other patients
with Turners syndrome, the condition is diagnosed either
in adolescence or in adulthood. [5] Noonan syndrome
Journal of Indian Society of Periodontology - Vol 15, Issue 2, Apr-Jun 2011

Figure 8: Postoperative orthopantomogram revealing good bone fill in the treated

areas (arrows)

Table 1: Baseline status, treatment administered, and

postoperative status of the treated teeth
Teeth

Baseline

Treatment
6 months
administered

1 year

25, 26,
31
36, 37,
46

Grade III
mobile*
Grade II
mobile*
PD>8 mm
Grade II
mobile*
PD>10 mm

Extracted

Grafted with
Osteogen

No mobility Radiographically,
PD<5 mm good amount of
bone fill apparent
No mobility
PD<5 mm

47

RCT done
Hemisected
Grafted with
Osteogen

*Millers Tooth Mobility Index

is an autosomal-dominant inherited disorder that has

characteristic abnormalities resembling those in Turners
syndrome. [13] However, Noonan syndrome affects both
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Kasagani, et al.: Turners syndrome with LAP

males and females.[13,14] The observation that patients with

Noonan syndrome have normal karyotypes (46XX or 46XY)
is important in allowing the distinction to be made between
the two syndromes.[13]
The case reported here represents a typical case of Turners
syndrome. Classical findings of short stature, gonadal
dysgenesis, primary amenorrhea, and cytogenetic studies
confirmed the diagnosis of Turners syndrome. The karyotype
was found to be typical of Turners syndrome (45XO) and
hence Noonan syndrome was excluded from the differential
diagnosis.

CONCLUSION
In the present case, surgical and regenerative periodontal
therapy in a female with Turners syndrome showed a
successful outcome. A reduction in mobility of teeth was
observed and the patient was highly satisfied with the result.
We conclude that patients with Turners syndrome respond to
surgical and regenerative periodontal procedures in much the
same way as would patients with a normal phenotype.

REFERENCES
1.

Several oral manifestations of Turners syndrome have been

reported in the literature. A high-arch palate was found in
the present case. However, hypoplasia of the mandible [7]
or reduction in tooth size [8] was not seen. Oral Hygiene
Index-Simplified (OHI-S) scores were low and the gingiva
appeared normal, with mild inflammation. Mobility and deep
periodontal pockets were observed in relation to the molars and
incisors, as would be expected in a case of localized aggressive
periodontitis.
In the present case report, antimicrobial therapy followed
by regenerative periodontal therapy was carried out and the
patient was followed-up for a period of 1 year. A satisfactory
outcome was achieved. The fact that the patient was a female
with Turners syndrome and had underdeveloped gonads did
not affect the treatment outcome.
The literature has many case reports describing Reactive
Positioning or Spontaneous Correction of pathologic
migration following periodontal treatment.[15,16] These reports
indicate that migrated teeth sometimes move back to their
normal position following nonsurgical periodontal treatment
alone or, in some instances, when combined with surgical
methods.[17] In the present case, a midline diastema was present
at baseline. However, posttreatment, it was noticed that the
midline diastema had closed and the maxillary central incisors
approximated each other. Thus, in the present case, midline
diastema disappeared by reactive positioning after surgical
periodontal therapy [Figure 7].
Dental hygiene treatment for the Turners syndrome patient
offers many challenges.[7] Early diagnosis of oral manifestations
is of utmost importance. [3] Observing the patient in the
reception area and treatment room, taking a complete and
thorough medical history, documenting medications, and
assessing vital signs allow for comprehensive care and the
formulation of individualized treatment plans.[7] Orthodontic
evaluation should be considered for all girls with Turners
syndrome when permanent teeth have erupted.[8] The family
of any girl diagnosed with Turners syndrome must be wellmotivated to take good care of her dental health in addition
to her general health.[3]

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How to cite this article: Kasagani SK, Mutthineni RB, Jampani

ND, Nutalapati R. Report of a case of Turner's syndrome with
localized aggressive periodontitis. J Indian Soc Periodontol
2011;15:173-6.
Source of Support: Nil, Conflict of Interest: None declared.

Journal of Indian Society of Periodontology - Vol 15, Issue 2, Apr-Jun 2011