Journal of Clinical and Diagnostic Research, ISSN - 0973 - 709X

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National Joint Coordinator - Advanced IAP NNF NRP Program
Ex-Member, Governing Body, National Neonatology Forum, New Delhi
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On Sep 2018




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Bengaluru.
On Aug 2018




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Dr. Rajendra Kumar Ghritlaharey

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Authors are the souls of any journal, and deserve much respect. To publish a journal manuscripts are needed from authors. Authors have a great responsibility for producing facts of their work in terms of number and results truthfully and an individual honesty is expected from authors in this regards. Both ways its true "No authors-No manuscripts-No journals" and "No journals–No manuscripts–No authors". Reviewing a manuscript is also a very responsible and important task of any peer-reviewed journal and to be taken seriously. It needs knowledge on the subject, sincerity, honesty and determination. Although the process of reviewing a manuscript is a time consuming task butit is expected to give one's best remarks within the time frame of the journal.
Salient features of the JCDR: It is a biomedical, multidisciplinary (including all medical and dental specialities), e-journal, with wide scope and extensive author support. At the same time, a free text of manuscript is available in HTML and PDF format. There is fast growing authorship and readership with JCDR as this can be judged by the number of articles published in it i e; in Feb 2007 of its first issue, it contained 5 articles only, and now in its recent volume published in April 2011, it contained 67 manuscripts. This e-journal is fulfilling the commitments and objectives sincerely, (as stated by Editor-in-chief in his preface to first edition) i e; to encourage physicians through the internet, especially from the developing countries who witness a spectrum of disease and acquire a wealth of knowledge to publish their experiences to benefit the medical community in patients care. I also feel that many of us have work of substance, newer ideas, adequate clinical materials but poor in medical writing and hesitation to submit the work and need help. JCDR provides authors help in this regards.
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Thanking you
With sincere regards
Dr. Rajendra Kumar Ghritlaharey, M.S., M. Ch., FAIS
Associate Professor,
Department of Paediatric Surgery, Gandhi Medical College & Associated
Kamla Nehru & Hamidia Hospitals Bhopal, Madhya Pradesh 462 001 (India)
E-mail: drrajendrak1@rediffmail.com
On May 11,2011




Dr. Shankar P.R.

"On looking back through my Gmail archives after being requested by the journal to write a short editorial about my experiences of publishing with the Journal of Clinical and Diagnostic Research (JCDR), I came across an e-mail from Dr. Hemant Jain, Editor, in March 2007, which introduced the new electronic journal. The main features of the journal which were outlined in the e-mail were extensive author support, cash rewards, the peer review process, and other salient features of the journal.
Over a span of over four years, we (I and my colleagues) have published around 25 articles in the journal. In this editorial, I plan to briefly discuss my experiences of publishing with JCDR and the strengths of the journal and to finally address the areas for improvement.
My experiences of publishing with JCDR: Overall, my experiences of publishing withJCDR have been positive. The best point about the journal is that it responds to queries from the author. This may seem to be simple and not too much to ask for, but unfortunately, many journals in the subcontinent and from many developing countries do not respond or they respond with a long delay to the queries from the authors 1. The reasons could be many, including lack of optimal secretarial and other support. Another problem with many journals is the slowness of the review process. Editorial processing and peer review can take anywhere between a year to two years with some journals. Also, some journals do not keep the contributors informed about the progress of the review process. Due to the long review process, the articles can lose their relevance and topicality. A major benefit with JCDR is the timeliness and promptness of its response. In Dr Jain's e-mail which was sent to me in 2007, before the introduction of the Pre-publishing system, he had stated that he had received my submission and that he would get back to me within seven days and he did!
Most of the manuscripts are published within 3 to 4 months of their submission if they are found to be suitable after the review process. JCDR is published bimonthly and the accepted articles were usually published in the next issue. Recently, due to the increased volume of the submissions, the review process has become slower and it ?? Section can take from 4 to 6 months for the articles to be reviewed. The journal has an extensive author support system and it has recently introduced a paid expedited review process. The journal also mentions the average time for processing the manuscript under different submission systems - regular submission and expedited review.
Strengths of the journal: The journal has an online first facility in which the accepted manuscripts may be published on the website before being included in a regular issue of the journal. This cuts down the time between their acceptance and the publication. The journal is indexed in many databases, though not in PubMed. The editorial board should now take steps to index the journal in PubMed. The journal has a system of notifying readers through e-mail when a new issue is released. Also, the articles are available in both the HTML and the PDF formats. I especially like the new and colorful page format of the journal. Also, the access statistics of the articles are available. The prepublication and the manuscript tracking system are also helpful for the authors.
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Overall, the publishing process with JCDR has been smooth, quick and relatively hassle free and I can recommend other authors to consider the journal as an outlet for their work."



Dr. P. Ravi Shankar
KIST Medical College, P.O. Box 14142, Kathmandu, Nepal.
E-mail: ravi.dr.shankar@gmail.com
On April 2011

Important Notice

Dentistry
Year : 2011 | Month : April | Volume : 5 | Issue : 2 | Page : 393 - 395

Gorlin-Goltz Syndrome – Case Report

PAYAL GARG*, FRENY KARJODKAR**, SHOBHIT K GARG***

*MDS, Senior Lecturer, Department of ODMR, SGT Dental College, Farukh Nagar Road, Vill.Budhera, Gurgaon, Haryana (India); **MDS, Head of Department, Department of ODMR, Nair Hospital and Dental College, Mumbai Central, Mumbai, Maharashtra (India); *** MD, FRCR, Consultant, Department of Radiodiagnosis, MEDICITY – The Medanta, Gurgaon, Haryana (India)

Correspondence Address :
Dr. Payal Garg
Address- H. No. 727, Sector 31, Gurgaon – 122001 , India.
Contact No – 9899259434, 0124- 4117785
E-mail – payalshobhit@rediffmail.com Garg.payal4@gmail.com

Abstract

The simultaneous occurrence of multiple cysts in both the jaws of a patient is rare and it usually occurs as the manifestation of a syndrome. Whenever multiple cysts are found, it is necessary to rule out an association with any syndrome, as the chances of recurrence are very high and a periodic follow-up is required for such patients. In our patient, cyst enucleation was done previously, but the association with the Gorlin-Goltz syndrome was missed and no follow-ups were advised.

Keywords

Gorlin syndrome, Odontogenic cysts, Basal cell nevus syndrome

How to cite this article :

PAYAL GARG, FRENY KARJODKAR, SHOBHIT K GARG. GORLIN-GOLTZ SYNDROME – CASE REPORT. Journal of Clinical and Diagnostic Research [serial online] 2011 April [cited: 2019 Apr 21 ]; 5:393-395. Available from
http://www.jcdr.net/back_issues.asp?issn=0973-709x&year=2011&month=April&volume=5&issue=2&page=393-395&id=1157

INTRODUCTION

Gorlin and Goltz first described the spectrum of features which are associated with the Gorlin-Goltz syndrome or the Nevoid Basal cell carcinoma syndrome (NBCCS), in 1960. It is an autosomal dominant disorder with a genetic locus on chromosome subbands and bands 9q22.3-q31, as determined with linkage analysis (1). It is also called as the fifth phakomatosis due to the presence of multiple cutaneous, skel¬etal, ophthalmic and neurological abnormalities. It comprises of skeletal features such as the bifid rib, frontal and parietal bossing and man¬dibular prognathism and cutaneous abnormalities such as multiple basal cell carcinomas and palmar and plantar keratosis. NBCCS can also include concomitant hypertelorism, mental retardation, strabismus, calcification of the falxcerebri and medulloblastomas (2). We are discussing here the possibility that the current case is an expression of NBCCS and are briefly reviewing the features of the Gorlin-Goltz syndrome.

Case Report

A 35 year old male reported to the OPD of our department with the complaint of intraoral discharge in the upper right maxillary region since 3 months. The patient had a history of pain with respect to the maxillary right molars, subsequent to which extraction of the molars was done. This was followed by a discharge from the same region. The patient also gave a history of multiple jaw surgeries for cyst enucleation in the past. An intra-oral examination revealed missing 15, 16 and on pressure application, a white creamy exudate came out from that site.

On general examination, the patient was found to be well built and his face showed frontal bossing, hypertelorism and prominent supra-orbital ridges (Table/Fig 1). An Orthopantomogram (OPG) of the patient showed multiple cysts in the jaws – three in the mandible and two in the maxilla (Table/Fig 2). Considering the possibility of the Gorlin- Goltz syndrome, further evaluation was done with chest radiographs, which revealed a bifid rib (Table/Fig 3), lateral skull radiographs which revealed the bridging of the sellaturcica (Table/Fig 4) and CT scan of the brain which showed the lamellar calcification of the falx cerebri (Table/Fig 5).
(Table/Fig 1): Frontal Profile of patient

(Table/Fig 2): Orthopantomogram showing three cystic lesions in the mandible and two involving the maxillary sinus on either side.


(Table/Fig 3): Formatted chest radiograph showing bifurcated left fifth rib.

(Table/Fig 4): Formatted lateral skull radiograph showing bridging of the sella turcica.

(Table/Fig 5): Axial CT brain image showing falx calcification.


CT scan of the jaw of the patient revealed five expansile osteolytic lesions, two in the maxilla and three in the mandible, with thinning and breech of the cortical plates. Maxillary lesions, one on either side, were seen involving the alveolus (Table/Fig 6) and were also seen to be extending into the maxillary sinus and the retrobulbar region of the orbits. An impacted tooth was seen in left maxillary lesion. On the right side, there was loss of the cortex in the maxillary tuberosity region. The mandibular lesion on the right side involved the ramus. On the left side, one lesion was seen in the periapical region with respect to the second premolar and another one was seen in the posterior part of the mandibular body, extending into the angle and the ramus. The lesions also involved the mandibular canal on the left side. Surgical enucleation of all the five cysts was done, followed by curettage. The tissues were sent for histo-pathological examination and all the cysts were found to be odontogenic keratocysts.

(Table/Fig 6): Axial CT image showing expansile lytic lesions in maxilla bilaterally with cortical breech.

Discussion

The diagnosis of the Gorlin-Goltz syndrome is made clinically by using the criteria which are suggested by Evans and others (3). Two major or one major and two minor criteria should be satisfied for a positive diagnosis.

Our patient had three major features of NBCCS, namely bifid rib, multiple odontogenic keratocysts in the jaw and lamellar calcification of the falx and minor features such as the bridging of the sella turcica, frontal bossing, prominent supraorbital ridges and hypertelorism, thus suggesting it to be a case of the Gorlin-Goltz syndrome. Regarding the site predilection, OKCs which are associated with NBCCS are more common in the mandible with 69% involvement, as compared to 31% in the maxilla. In the mandible, 43% OKCs occurs in the molar ramus region, followed by 18% in the incisor-¬canine area. In the maxilla, 14% OKCs were found to occur in the incisor-¬canine area, followed by molar tuberosity with 12%, 7% in the mandibular premolar region and 3% in the maxillary premolar region.
Regarding the male to female ratio, it was 1:0.62 for OKCs which were not associated with NBCCS and 1:1.22 for OKCs in NBCCS. This shows that simple keratocysts are more common in males, but that more females seem to have NBCCS (4).
Based on histopathological studies, parakeratiniza¬tion, intramural epithelial remnants and satellite cysts were found to be more frequent among the OKCs which were associated with NBCCS than in the solitary keratocysts (5). In our patient, the lining of the OKCs revealed the presence of parakeratinization and epithelial remnants in the connective tissue wall, thus indi-cating the association with NBCCS. Multiple satellite and daughter cysts were also seen by histopathology in our patient (Table/Fig 7).

(Table/Fig 7): Hematoxylin& Eosin (H & E) stained section shows satellite cyst.
The term “multiple cysts” does not necessarily mean that the patient must have more than one cyst at a given time; rather it refers to the occurrence of cysts over the life time of the patient (6). Our patient also had a history of multiple cyst enucleations in the past. There is no specific laboratory test to diagnose NBCCS, although the affected patients may have high levels of cyclic adenosine monophosphate and impaired phosphate diuresis on parathormone challenge (7).
The treatment of the Gorlin Goltz syndrome is in accordance with the generally accepted rules for the treatment of basal cell carcinomas and keratocysts in other patients. Radiation should be avoided, as it may trigger off the development of other tumors in the adjacent skin areas. Cystectomy, including the removal of the bony walls of the resulting cavity, is an adequate surgical treatment for the odontogenic keratocysts. In the treatment of the recurrent OKCs which are associated with NBCCS, the overlying surface epithelium should be excised along with the cystic lining to prevent recurrences from the residual epithelial islands and microcysts(8). In addi-tion, the use of Carnoy’s solution following cyst enucleation (applied only over the areas where the cyst is attached to the mucosa) and cryosurgery (because of the unique ability of liquid nitrogen to devitalize the bone in situ while leaving the inorganic framework untouched) is advocated to kill the epithelial remnants and the dental lamina within the osseous structures and to thus, prevent recurrences (9).

Conclusion

Our case highlights the importance of the awareness of this rare syndrome, especially in young patients without any skin lesions. It is useful to keep in mind the existence of this syndrome and to recognize the presence of some major criteria that are easily recognizable in the CT scan of the head and neck, to thus establish the diagnosis, to offer the opportunity for frequent follow-ups and to therefore, increase the chances for better overall survival rates (10). In this case, the patient had also undergone surgeries in the past for cyst enucleation, but the possibility of the Gorlin-Goltz syndrome was not considered at that time and therefore, no frequent follow-ups were advised to the patient. It is important to follow up the patients with diagnosed syndromes for the rest of their lives, because they can produce new odontogenic cysts and new basal cell carcinomas almost continuously. Basal Cell Carcinomas require frequent follow-up care, 3-4 times a year (or more), to achieve an early diagnosis and to plan the treatment. In young children who are at risk, medulloblastomas necessitate a neurological examination every 6 months, and intermittent MRIs should be considered in children who are younger than 7 years of age. Odontogenic keratocysts require dental follow-up visits, including a periodic radiographical evaluation every 6 months, especially in childhood and early adolescence. (11) Finally, the whole family of the patients with the Gorlin-Goltz syndrome should be examined and genetic counseling should be offered, as it is inherited as an autosomal dominant disorder.

Key Message

Multiple odontogenic keratocysts which are seen in patients with the Gorlin- Goltz Syndrome have a tendency to recur and so, a periodic follow-up is necessary in such patients.

References

1.
Gailani MR, Bale SJ, Leffell DJ, et al. Developmental defects in Gorlin syndrome related to a putative tumor suppressor gene on chromosome. Cell 1992;69(1):111-7.
2.
Lindeboom JA, Kroon FH, de Vires J, van den Akker HP. Multiple recur¬rent and de novo odontogenickeratocysts associated with oral-facial-digital syndrome. Oral Surg Oral Med Oral Pathol Oral RadiolEndod 2003; 95(4):458–62.
3.
Evans DG, Sims DG, Donnai R. Family implications of neonatal Gorlin′s syndrome. Arch Dis Child 1991; 66:1162-3.
4.
Karthiga S Kannan, Sivapatha B Sundharam, RManikandan. Nevoid basal cell carcinoma syndrome. IJDR 2006; 17(1): 50-53.
5.
Dominguez FV, Keszler A. Comparative study of keratocysts, associated and non-associated with nevoid basal cell carcinoma syndrome. J Oral Pathol1988; 17(1):39–42.
6.
Woolgar JA, Rippin JW, Browne RM. The odontogenickeratocyst and its occurrence in the nevoid basal cell carcinoma syndrome. Oral Surg Oral Med Oral Pathol 1987; 64(6):727–30.
7.
Bakaeen G, Rajab LD, Sawair FA, Hamdan MA, Dallal ND. Nevoid basal cell carcinoma syndrome: a review of the literature and a report of a case. Int J Paediatr Dent 2004; 14(4):279–87.
8.
Myoung H, Hong SP, Hong SD, Lee JI, Lim CY, Choung PH, and others. Odontogenickeratocyst: review of 256 cases for recurrence and clinico¬pathologic parameters. Oral Surg Oral Med Oral Pathol Oral RadiolEndod 2001; 91(3):328–33.
9.
Stoelinga PJ. Excision of the overlying, attached mucosa, in conjunction with cyst enucleation and treatment of the bony defect with carnoy solution. Oral MaxillofacSurgClin N Am 2003; 15:407–14.
10.
Christina Kaloaeropoulou, PetrosZampakis, SantraKazantzi, PantelisKraniotis and Nicholas S Mastronikolis. Gorlin-Goltz Syndrome – incidental finding on routine CT scan following car accident. Cases Journal 2009; 2:9087.doi:10.1186/1757-1626-2-9087.
11.
Berg Daniel. Nevoid Basal Cell Carcinoma Syndrome: Follow up [Internet] 2010.[Updated 2009 March 12; cited 2010 Jan 20] Available from http:// emedicine.medscape.com/article/1101196- follow-up

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