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Year : 2022  |  Volume : 3  |  Issue : 2  |  Page : 50-53

Klippel-trenaunay syndrome: A pedodontist perspective

Department of Pedodontics and Preventive Dentistry, Dr. R Ahmed Dental College and Hospital, Kolkata, West Bengal, India

Date of Submission21-Nov-2021
Date of Acceptance20-Dec-2021
Date of Web Publication9-Mar-2022

Correspondence Address:
P N Nanmaran
Department of Pedodontics, Dr. R. Ahmed Dental College and Hospital, Kolkata - 700 014, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpcdoh.jpcdoh_43_21

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Klippel–Trenaunay syndrome (KTS) is a congenital, uncommon but a well-recognized disorder, characterized by cutaneous hemangiomas, varicosities, and unilateral bone and soft tissue hypertrophy with different localization. Generally diagnosed at birth or first few years of life. KTS represents a syndrome of interest for those in the field of oral and maxillofacial radiology and pathology because of high incidence of variations seen in the orofacial region in affected patients. A case of a 3-year-old female patient with KTS reported to the Department of Pedodontics and Preventive Dentistry for extraction, and we have documented and tabulated the Orodental manifestation of this patient.

Keywords: Genu valgum, Klippel–Trenaunay syndrome, macrodactyly, portwine stain

How to cite this article:
Nyodu T, Pal S, Gayen K, Nanmaran P N, Sarkar S. Klippel-trenaunay syndrome: A pedodontist perspective. J Prim Care Dent Oral Health 2022;3:50-3

How to cite this URL:
Nyodu T, Pal S, Gayen K, Nanmaran P N, Sarkar S. Klippel-trenaunay syndrome: A pedodontist perspective. J Prim Care Dent Oral Health [serial online] 2022 [cited 2022 May 27];3:50-3. Available from: http://www.jpcdoh.org/text.asp?2022/3/2/50/339311

  Introduction Top

Klippel–Trenaunay syndrome (KTS) is a rare, complex, congenital illness, In 1832 Geoffroy-saint Hilaire may have been the first to record a case of KTS in the literature,[1] and documented by Maurice Klippel and Paul-Trenaunay in 1900.[2] It has two or more of the following characteristics; capillary malformation (port-wine stain), Varicose veins or venous malformation, soft tissue or bony hypertrophy (or both)[1] and asymmetric overgrowth of the limbs and extremities.[2] It has no sex predilection with a frequency of about 2–5 per 1,00,000 children, however other research suggests that males are more affected than females.[3]

We report a case of KTS, with a focus on the orofacial clinical characteristics and their clinical importance.

  Case Report Top

A 3-year-old female patient presented to the department of pediatric Dentistry with the major complaint of a mobile tooth in the maxillary anterior region, and this was her first dental visit. A written consent form was obtained from the parents.

A complete medical history of the patient was obtained and her mother had a full-term pregnancy and delivered through cesarean section. There was no history of a similar condition in the family. The patient's medical history revealed that at the age of one, she was transferred to a polio hospital because she was unable to walk and stand independently and her left knee was bended inward, Later the patient was diagnosed with Genu valgum. Hence, the patient was provided with Mermaid splint and Unibar KAFO without a knee joint.

Extraoral examination reveals stasis dermatitis as well as extensive flat cutaneous hemangioma (port-wine stains) on the left half of the neck [Figure 1]. Her left hand's third finger was found to have macrodactyly (A digit deformity) [Figure 2]. Her left leg was bigger and longer than the right leg (Limb length discrepancy) and the left joint was deviated medially [Figure 3]. The right side of the face was slightly larger than the left side but without severe facial asymmetry. A round protruding firm growth was seen in the occipital region of the patient's head [Figure 4].
Figure 1: Extra oral examination reveals statis dermatitis, extensive flat cutaneous hamangioma (port wine stains) on the left half of the neck

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Figure 2: Digit deformity seen with macrodactyly of middle finger of patient's left hand

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Figure 3: Left leg bigger and longer than the right leg (limb length discrepancy) and the left joint was deviated medially

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Figure 4: A round protruding firm growth was seen in the occipital region of the patient's head

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Soft tissue overgrowth in the lower anterior teeth region [Figure 5]a was discovered during an oral examination, as was an enlarged maxillary arch with generalized spacing (as she reported at a very young age). According to the history given by the parents, the stage of the eruption of primary teeth was normal, but a premature early supraerupted permanent central incisor was found in the lower anterior teeth region [Figure 5]b multiple carious teeth and developing anterior crossbite were noted, oral hygiene was fair with gingival inflammation.
Figure 5: (a) Soft tissue overgrowth in the lower anterior teeth region. (b) Premature early supraerupted permanent central incisor in the lower anterior teeth region

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However, the patient was well oriented in terms of time, place, and person, and her eyesight and hearing were normal, however, the patient struggled to understand simple directions. The radiographic examination could not be performed because the patient was unable to follow the instructions and did not cooperate sufficiently.

A complete hematological investigation was performed to rule out any complication linked with KTS during dental intervention, such as bleeding disorder and delayed wound healing, which could constitute a possible hazard while dealing with a KTS patient. As the patient's laboratory investigation (complete blood count, blood chemistry) did not reveal any abnormalities, we proceeded with the treatment. Local anesthetic with adrenaline was administered, and the deciduous central incisor of the first quadrant was extracted atraumatically. The patient was given a pressure pack and was kept under observation for 30 min. After 1 week a follow up was done, and healing was found to be satisfactory.

  Discussion Top

The specific cause of this syndrome is unknown, but it is stated in some literature as somatic mutations of the genes responsible for embryonic angiogenesis, however, You et al., and Leiber et al., postulated that an intrauterine disturbance present at the time of vascular differentiation between the 3rd and the 6th week of gestation.[4],[5] According to Aelvoet et al., while there have been a few familial cases of KTS have been recorded, the bulk of the cases are sporadic and no obvious genetic pattern can be found.[6] Vahidnezhad et al., hypothesized that KTS could be caused by mutations in the PIK3CA gene implying that KTS, rather than being a distinct clinical entity, is part of the PIK3CA related over growth spctrum.[7]

The symptoms can range in severity from minor (birth scars, varicose veins) to severe (limb length discrepancy, gross gigantism, and deformity of extremity). The differential diagnosis of KTS includes – Sturge weber syndrome, Proteus syndrome, Maffucci syndrome, and neurofibromatosis.[8],[9]

The hypertrophy of the soft tissues as well as the bone, resulting in increased length and girth of affected extremities. In contrast to clinical features seen with respect to extremities, varicose veins was not found in the presented case.

Despite the fact that the clinical signs of KTWS have been thoroughly studied and acknowledged, there have been very few reports of orodental sequelae. It is possible that the orodental findings go unnoticed since they are overwhelmed by the more visible limb abnormalities. Asymmetric growth of the craniofacial skeleton may lead to jaw malformation and, as a result, malocclusion, a unilateral growth in facial dimension of both hard and soft tissues, particularly the lips, teeth, and periodontal tissues[10],[11],[12],[13] and Palatal anomalies have also been documented.[14] Ita et al., noted a case of prolonged post extraction bleeding resulting in Disseminated Intravascular Coagulation.[15]

Attention must be paid to the dental care of these patients. The three most prevalent oral findings that demand implication for dentists include early dental development and eruption necessitates orienting and dental treatment to the patient's dental rather than chronological age, hypertrophy of the bone and gingiva, which can cause severe malocclusion and present challenges in developing and utilizing prosthesis. The diagnosis of KTS is usually based on the presence of any two of the three clinical characteristics. Typical oral manifestations of KTS and other findings in our case based on which the diagnosis was made is given in [Table 1].
Table 1: Typical oral manifestations of Klippel-Trenaunay syndrome and other findings in our case

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  Conclusion Top

Hence, it is very important to evaluate patients with KTS at a young age to implement preventive procedures and treat the teeth as soon as possible to avoid extraction and subsequent excessive hemorrhage and delayed healing. Although this syndrome is rare, general practitioners and pediatric dentists should be aware of its general and orofacial manifestations before carrying out any procedure to minimize complications.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Mueller Lessmann V, Behrendt A, Wetzel WE, Petersen K, Anders D. Orofacial findings in the Klippel Trénaunay syndrome. Int J Paediatr Dent 2001;11:225-9.  Back to cited text no. 1
Al-Saleem A, Al-Jobair A. Dental management of Klippel–Trenaunay Syndrome. King Saud University J Dental Sci 2013;4:87-90.  Back to cited text no. 2
Alwalid O, Makamure J, Cheng QG, Wu WJ, Yang C, Samran E, et al. Radiological aspect of Klippel Trénaunay syndrome: A case series with review of literature. Curr Med Sci 2018;38:925-31.  Back to cited text no. 3
You CK, Rees J, Gillis DA, Steeves J. Klippel Trenaunay syndrome: A review. Can J Surg 1983;26:399-403.  Back to cited text no. 4
Leiber B. Klippel-Tre´naunay-Symptomencomplex. In: Urban and Schwarzemberg, Die klinischen syndrome. 7th ed. Germany: Mu¨nchen; 1990. p. 391-2.  Back to cited text no. 5
Aelvoet GE, Jorens PG, Roelen LM. Genetic aspects of the Klippel Trenaunay syndrome. Br J Dermatol 1992;126:603-7.  Back to cited text no. 6
Vahidnezhad H, Youssefian L, Uitto J. Klippel Trenaunay syndrome belongs to the PIK3CA Related Overgrowth Spectrum (PROS). Exp Dermatol 2016;25:17-9.  Back to cited text no. 7
Huang WJ, Creath CJ. Klippel Trenaunay Weber syndrome: Literature review and case report. Pediatr Dent 1994;16:231-5.  Back to cited text no. 8
Fakir E, Roberts T, Stephen L, Beighton P. Klippel Trenaunay Weber syndrome: Orodental manifestations and management considerations. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;107:754-8.  Back to cited text no. 9
Gloviczki P, Hollier LH, Telander RL, Kaufman B, Bianco AJ, Stickler GB. Surgical implications of Klippel Trenaunay syndrome. Ann Surg 1983;197:353-62.  Back to cited text no. 10
Defraia E, Baccetti T, Marinelli A, Tollaro I. Biometric and magnetic resonance imaging assessment of dentofacial abnormalities in a case of Klippel Trénaunay Weber syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;97:127-32.  Back to cited text no. 11
Bolan M, Ferreira MC, Almeida IC, D'Agostini Derech C, Ribeiro GL. Palatal expansion and the Klippel Trénaunay Weber syndrome. Am J Orthod Dentofacial Orthop 2005;128:385-7.  Back to cited text no. 12
Bathi RJ, Agarwal N, Burde KN. Klippel Trénaunay syndrome (angio osteohypertrophy syndrome): A report of 3 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;93:276-80.  Back to cited text no. 13
Sciubba JJ, Brown AM. Oral facial manifestations of Klippel Trenaunay Weber syndrome. Report of two cases. Oral Surg Oral Med Oral Pathol 1977;43:227-32.  Back to cited text no. 14
Ita M, Okafuji M, Maruoka Y, Shinozaki F. An unusual postextraction hemorrhage associated with Klippel Trenaunay Weber syndrome. J Oral Maxillofac Surg 2001;59:205-7.  Back to cited text no. 15


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1]


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