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CASE REPORT
Year : 2013  |  Volume : 2  |  Issue : 2  |  Page : 147-149

Zimmermann-Laband syndrome: A rare case report of father and son


1 Department of Pedodontics and Preventive Dentistry, Sibar Institute of Dental Sciences, Guntur, Andhra Pradesh, India
2 Department of Periodontics, Sibar Institute of Dental Sciences, Guntur, Andhra Pradesh, India

Date of Web Publication21-May-2013

Correspondence Address:
Manoj K Mallela
Department of Pedodontics and Preventive Dentistry, Sibar Institute of Dental Sciences, Guntur, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.112357

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  Abstract 

The Zimmermann-Laband syndrome also known as Laband-Zimmermann syndrome or Laband's syndrome was reported by Zimmermann in the year 1928. It is a rare inherited autosomal dominant disease characterized by pathognomonic triad of extensive gingival enlargement (gingival fibromatosis), abnormalities of the nose and ears, absence or hyperplasia of the nails or terminal phalanges of the hands and feet. The syndromic characteristics are highly variable and complicated. The present study describes a case report of father and son aged 42 years and 13 years respectively, presenting features of Zimmermann-Laband syndrome.

Keywords: Gingival hyperplasia, gingivectomy, zimmermann-laband syndrome


How to cite this article:
Mallela MK, Moogala S, Polepalle LT, Reddy VC. Zimmermann-Laband syndrome: A rare case report of father and son. J NTR Univ Health Sci 2013;2:147-9

How to cite this URL:
Mallela MK, Moogala S, Polepalle LT, Reddy VC. Zimmermann-Laband syndrome: A rare case report of father and son. J NTR Univ Health Sci [serial online] 2013 [cited 2019 Oct 14];2:147-9. Available from: http://www.jdrntruhs.org/text.asp?2013/2/2/147/112357


  Introduction Top


Gingival fibromatosis is characterized by slow and progressive enlargement of maxillary and mandibular gingiva. It can be presented as an isolated feature or as a part of syndrome. [1] Hereditary gingival fibromatosis is associated with syndromes such as Rutherford syndrome, Zimmermann-Laband syndrome, Murray-Puretic-Drescher syndrome, Cross syndrome, and Ramon's syndrome. [1] Zimmermann-Laband syndrome is a rare entity characterized by a generalized enlargement of facial and lingual surfaces of the attached and marginal gingiva, abnormalities of nose or ears, absence and/or hyperplasia of the nails or terminal phalanges of the hands and feet. [2]


  Case Report Top


A 13 year-old boy reported to the department of pedodontics with the chief complaint of excessive growth of gingival tissue resulting in serious masticatory, esthetic, and phonetic problems. History revealed that the gingival growth gradually increased in size and reached to the present condition. Family history presented his father with the similar features and mother with an unusual exfoliation of all the permanent teeth by the age of 32 years. Patient's elder brother is apparently normal. Extraoral examination of the father and son showed the presence of thick eyebrows, eyelashes, bulbous nose, floppy ears, and thick lips with prominent maxilla and mandible [Figure 1]a, b, c and d. The patient had generalized hypertrichosis with deep palmar and plantar creases [Figure 2]a and high arched foot (pes cavus) [Figure 2]b. The radiographs of both hands and legs showed presence of deformity at tarsometatarsal joint [Figure 2]c. Intraoral examination of father and son presented diffuse and severe gingival enlargement in the maxilla and mandible with macroglossia and high arched palate. The gingival enlargement was so excessive that most of the teeth were embedded in gingiva [Figure 3]a, b, c and [Figure 4]a, b, c. A panaromic radiograph revealed the presence of both deciduous and permanent teeth arranged irregularly [Figure 5]a. Lateral cephalogram presented with prognathic maxilla and mandible with slight increase in gonial angle. Hematological and biochemical findings were within the normal range. Ultrasonic imaging showed no signs of hepatosplenomegaly and patient was systemically healthy. The patient was diagnosed with Zimmermann-Laband syndrome based on the clinical manifestations and investigations. Treatment was planned to extract the deciduous teeth followed by gingivectomy at regular intervals to improve mastication and phonetics. A reverse bevel gingivectomy with osseous recontouring was performed under local anesthesia at an interval of 3 weeks. A 0.12% chlorhexidine gluconate rinse was prescribed for 2 weeks postsurgically. Histopathological examination of the excised tissue showed keratinized epithelium with elongation of rete pegs. Beneath the epithelium there were dense bundles of collagenous fibrous tissue [Figure 5]b. There was compression of blood vessels due to increased collagen fiber bundles. A pathological diagnosis of gingival fibromatosis was made. The patient has been periodically followed up for plaque control, which is important in preventing recurrence after treatment [Figure 6]a, b, c.
Figure 1: Front view (a) and facial profi le (b) of the father. Front view (c) and facial profi le (d) of the son

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Figure 2: Generalized hypertrichosis (a), pes cavus or high arched foot (b), varus deformity at tarso metatarsal joint (c)

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Figure 3: Intraoral preoperative views of upper and lower arch of the father (a), (b), and (c)

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Figure 4: Intraoral preoperative views of upper and lower arch of the son (a), (b), and (c)

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Figure 5: Preoperative OPG of the son (a). Histological picture showing dense collagen bundles and compressing blood vessels (b)

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Figure 6: Intraoral postoperative view of upper and lower arch of the son (a), (b), and (c)

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  Discussion Top


Zimmermann-Laband syndrome is a very rare entity with autosomal dominant mode of inheritance. Both the sexes may be affected in equal ratio. Eleven cases from two families in the literature were well documented with evidence of autosomal dominant inheritance. [3] A total of forty cases have been reported till 2010. This syndrome is coded with phenotype MIM number 135500. The gene responsible for Zimmermann-Laband syndrome is located in 3p14.3 and implicates four likely candidate genes in this region. CACNA2D3 gene encoding a voltage dependent calcium channel, LRTM1 gene of unknown function, WTN5A gene encoding a secreted signalling protein, and ERC2 gene, which encodes for a synapse protein. [4] Gingival hyperplasia is reported to be present in patients treated with calcium channel blockers that affect intracellular calcium metabolism or transport. [3]

This syndrome is not a life-threatening disorder. The most consistent feature of the syndrome is gingival fibromatosis appearing in early childhood. It may exist as an isolated finding or as part of a genetic syndrome. In the diagnosis, generalized gingival enlargement, a detailed medical history, and systemic evaluation is necessary to differentiate gingival fibromatosis from various other types of acquired generalized gingival enlargement that can occur as a result of inflammation, pregnancy, leukemia, and in response to certain drugs such as phenytoin, diltiazem, cyclosporine, verapamil, and nifedipine. In such cases, the gingiva is usually not as enlarged or as fibrotic as in hereditary gingival fibromatosis. [5] Shah and Gupta [3] reported a case of Zimmermann-Laband syndrome in a young child of age 3½ years associated with bilateral developmental cataract. Lin et al., [2] presented with new manifestations of thymic hyperplasia evident as a soft tissue mass in front of the aorta without enlargement of lymph nodes. Other manifestations such as left ventricular hypertrophy and aortic root dilation were reported. [6] Sawaki et al., [7] stated that the major clinical findings of Zimmermann-Laband syndrome would be gingival fibromatosis, hyperplasia or absence of the terminal phalynx or nails of hand and feet, bulbous soft nose, thick lips, large ears, and enlargement of soft tissues of the face. Syndromes such as Murray-Puretic-Drescher (juvenile hyaline fibromatosis) present with multiple hyaline fibromas, osteolysis of terminal phalanges, recurrent infection, stunted growth, and premature death. Cross syndrome presents with microphthalmia mental retardation, athetosis, and hypopigmentation. Jones syndrome presents with progressive deafness. When gingival fibromatosis is associated with nose, bone, and nail defects it may characterize the presence of Zimmermann-Laband syndrome. [2]

Surgical correction of gingival fibromatosis is recommended, although there is no information on the permanence of the results of this treatment in the literature. Extraction of the deciduous teeth followed by internal bevel gingivectomy with osseous recontouring in our case will establish the necessary conditions for an effective orthodontic treatment, which may improve the facial esthetics.


  Conclusion Top


In summary, this syndrome is not a killer disease, but it needs a comprehensive medical history, physical, and systemic evaluation that is essential for correct diagnosis, treatment, and prevention of complications that can occur in patients.


  Acknowledgements Top


Dr. Ramana Reddy BV, Professor and Head, Department of Oral and Maxillofacial Pathology, Sibar Institute of Dental Sciences.

 
  References Top

1.Coletta RD, Graner E. Hereditary gingival fibromatosis: A systemic Review. J Periodontal 2006;77:753-64.  Back to cited text no. 1
    
2.Lin Z, Wang T, Sun G, Huang X. Report of a case of Zimmermann-Laband syndrome with new manifestations. Int J Oral Maxillofac Surg 2010;39:937-41.  Back to cited text no. 2
    
3.Shah N, Gupta YK, Ghose S. Zimmermann-Laband syndrome with bilateral developmental cataract - A new association? Int J Paediatr Dent 2004;14:78-85.  Back to cited text no. 3
    
4.Kim HG, Higgins AW, Herrick SR, Kishikawa S, Nicholson L, Kutsche K, et al. Candidate loci for Zimmermann-Laband syndrome at 3p14.3. Am J Med Genet A 2007;143:107-11.  Back to cited text no. 4
    
5.Holzhausen M, Ribeiro FS, Gonçalves D, Corrêa FO, Spolidorio LC, Orrico SR. Treatment of gingival fibromatosis associated with Zimmermann-Laband syndrome. J Periodontol 2005;76:1559-62.  Back to cited text no. 5
    
6.Sattur AP, Burde KN, Naikmasur VG. Zimmermann-Laband syndrome: Report of a case with early cardiac complications. J Indian Acad Oral Med Radiol 2010;22:174-6.  Back to cited text no. 6
    
7.Sawaki K, Mishima K, Sato A, Goda Y, Osugi A, Nakano M. Zimmermann-Laband syndrome: A case report. J Clin Pediatr Dent 2012;36:297-300.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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  Introduction
  Case Report
  Discussion
  Conclusion
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