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Indian J Ophthalmol. 2010 Sep-Oct; 58(5): 448–449.
PMCID: PMC2992935

Authors’ reply

Dear Editor,

The authors wish to thank Gupta et al.[1] for their interest in our article[2] and for their comments. Point-wise clarification of the queries is given herewith:

  1. In this case, we can observe prominent veins and thinning of skin with some hypopigmented lesions, which in itself is suggestive of hypoplasia. Moreover, the texture of the skin was felt to be hypoplastic and it is difficult to notice it in a two-dimensional image.
  2. We do not agree with the statement that only microphthalmia is the feature of Delleman syndrome, as the authors[3] themselves and other authors who have studied and compared their cases with previous reported cases have always mentioned anophthalmia / microphthalmia as a feature of the oculocerebrocutaneous syndrome. Hennekam[4] and Moog et al.[5] have also described multiple features associated with this syndrome.Therefore we still believe that this is a case of Delleman Oorthuys syndrome.
  3. The third image was only included as per recommendations of a peer reviewer and there was no recurrence of cystic swelling as the case is still on follow-up. However, we do agree that the gradually expanding custom-made prosthesis is recommended in such cases, which is being followed in this case as well.


1. Gupta VP, Bhattacharya SN, Gupta P, Gupta R. Is it congenital cystic eye with dermal appendages and cerebral anomalies? ’ Indian J Ophthalmol. 2010;58:448. [PMC free article] [PubMed]
2. Arora V, Kim UR, Khazei HM. Delleman Oorthuys syndrome: ‘Oculocerebrocutaneous syndrome’ Indian J Ophthalmol. 2009;57:387–9. [PMC free article] [PubMed]
3. Delleman JW, Oorthuys JW. Orbital cyst in addition to congenital cerebral and focal dermal malformations: a new entity? Clin Genet. 1981;19:191–8. [PubMed]
4. Hennekam RC. Scalp lipomas and cerebral malformations: overlap between encephalocraniocutaneous lipomatosis and oculocerebrocutaneous syndrome. Clin Dysmorphol. 1994;3:87–9. [PubMed]
5. Moog U, Krüger G, Stengel B, De Die-Smulders C, Dykstra S, Bleeker-Wagemakers E. Oculocerebrocutaneous syndrome: a case report, a follow-up, and differential diagnostic considerations. Genet Couns. 1996;7:257–65. [PubMed]

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