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

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]

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]