Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obervation Letter
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF
Letter to the Editor
2014:80:2;155-156
doi: 10.4103/0378-6323.129400
PMID: 24685857

Delleman syndrome or Haberland syndrome?

Shivcharan Lal Chandravanshi, Sujata Lakhtakia
 Department of Ophthalmology, Shyam Shah Medical College and Associated Gandhi Memorial Hospital, Rewa, Madhya Pradesh, India

Correspondence Address:
Shivcharan Lal Chandravanshi
Department of Ophthalmology, Shyam Shah Medical College and Associated Gandhi Memorial Hospital, Rewa - 486 001, Madhya Pradesh
India
How to cite this article:
Chandravanshi SL, Lakhtakia S. Delleman syndrome or Haberland syndrome?. Indian J Dermatol Venereol Leprol 2014;80:155-156
Copyright: (C)2014 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

We read the article by Nocito et al.[1] with interest and wish to make the following observations:

  1. The authors have described a dermoid cyst over the iris impairing patient′s vision in the left eye. We disagree as the location of dermoid is on the cornea and limbus, rather than iris. Iris is the intraocular pigmented structure of eye, which regulates the entry of light into the eye. Authors also state that patient is having a blue-gray dermoid cyst over the right eye sclera. We disagree with the diagnosis in the right eye since the color of dermoid ranges from yellow to gray to pink rather than blue-gray. [2] It seems to be an anterior staphyloma because the color of staphylomata is almost always blue-gray due to underlying uveal tissue. The visible blood vessels over the surface of the lesion also favor the diagnosis of staphyloma since they are known to be present in staphyloma and are always absent in dermoid cyst. Furthermore, dermoid cysts usually have hair on their surface or in rare circumstances, a bunch of hair may be visible through thinned out cyst wall. Anterior and intercalary staphyloma may develop after chronic scleritis, trauma and limbal dermoid excision. [2] Congenital anterior staphyloma has also been reported in the literature.
  2. The minimal diagnostic criteria for Delleman-Oorthuys syndrome include a central nervous system cyst or hydrocephalus, microphthalmos with orbital cyst and focal dermal hypoplasia or aplasia. [3] As two of these components, that are microphthalmos with orbital cyst and focal dermal hypoplasia or aplasia were missing from the case report of Nocito et al.,[1] it does not appear to be a typical case of Delleman-Oorthuys syndrome. The constellation of findings in this case, i.e., limbal dermoid, frontal alopecia (? nevus psiloliparus), upper eyelid coloboma, papular lesion on the eyelid and arachnoid cyst is suggestive of encephalocraniocutaneous lipomatosis or Haberland syndrome or Fishman syndrome. [4]
  3. Authors did not report the status of visual acuity and intraocular pressure in their case and have stated that enucleation of eyeball is the only possible treatment for cosmetic purposes. We think that the present case is a potential candidate for limbal dermoid excision with keratoplasty in his left eye and staphyloma repair with donor scleral grafting with or without keratoplasty in his right eye. Surgical correction of limbal dermoids should be performed as early as possible to prevent amblyopia. Even if the patient has no perception of light in either eye, enucleation is never a procedure of choice. In such cases (absent light perception or blind eye), evisceration with orbital implant may be the procedure of choice, because it provides better cosmesis in term of greater ocular prosthesis motility and less chances of orbital implant extrusion. [5] Enucleation alone can lead to post enucleation socket syndrome, which is characterized by enophthalmos, deep upper eyelid sulcus, ptosis and laxity of the lower lid. [6]
References
1.
Nocito MJ, Luna PC, Contardi ML, Mazzini MA. Delleman syndrome: Report of a case in an adolescent boy. Indian J Dermatol Venereol Leprol 2012;78:229.
[Google Scholar]
2.
Mansour AM, Barber JC, Reinecke RD, Wang FM. Ocular choristomas. Surv Ophthalmol 1989;33:339-58.
[Google Scholar]
3.
McCandless SE, Robin NH. Severe oculocerebrocutaneous (Delleman) syndrome: Overlap with Goldenhar anomaly. Am J Med Genet 1998;78:282-5.
[Google Scholar]
4.
Hunter AG. Oculocerebrocutaneous and encephalocranio cutaneous lipomatosis syndromes: Blind men and an elephant or separate syndromes? Am J Med Genet A 2006;140:709-26.
[Google Scholar]
5.
Nakra T, Simon GJ, Douglas RS, Schwarcz RM, McCann JD, Goldberg RA. Comparing outcomes of enucleation and evisceration. Ophthalmology 2006;113:2270-5.
[Google Scholar]
6.
Steinkogler FJ. The treatment of the post-enucleation socket syndrome. J Craniomaxillofac Surg 1987;15:31-3.
[Google Scholar]

Fulltext Views
164

PDF downloads
111
Show Sections