Presentation of massive orbital hidrocystoma at birth: case report and review of the literature
Received: 21 September 2016
Accepted: 2 February 2017
Published: 23 February 2017
Abstract
Background
Hidrocystoma, or sudoriferous cyst, is an eyelid tumor originating from apocrine or eccrine sweat glands. Its presence in the orbit is relatively rare.
Case presentation
A full-term female child with severe right eye extrusion was referred to our department two hours after birth. We performed cyst aspiration under ultrasonic guidance; 15 cc straw-colored fluid was obtained and proptosis resolved significantly. Orbital magnetic resonance imaging (MRI) showed a large unilocular retrobulbar mass with hypo-intensity in T1 and hyper-intensity in T2. The case underwent further daily ocular assessment until day 5; when proptosis began to worsen again. On day 6, under general anesthesia, we performed aspiration and then the cyst was completely removed with an intact wall through a trans-conjunctival incision. The diameter of the aspirated cyst was about 4 cm. In pathologic consultation, a cystic cavity lined by a layer of sweat duct like epithelium with apical snouts consistent with the diagnosis of apocrine hidrocystoma was reported.
Conclusion
To date, in the literature, six other cases of orbital hidrocystoma have been reported in childhood with protean clinical pictures; none of which presented at birth. Herein, we introduce the first case report at birth and also provide a review on the literature. Our report strongly argues against the well reputed theory of traumatic origin for orbital hidrocystoma; it has been postulated that this tumor may be the result of sweat gland cells implantation through the orbit. We thereby suggest the possible presence of choristomatous ectopic sweat gland cells in the orbit during embryogenesis.
Keywords
Background
Hidrocystoma, or sudoriferous cyst, is an eyelid tumor originating from apocrine or eccrine sweat glands [1]. Presentation of this tumor in the orbit is very rare [2–6]. In 1973, Saunders reported a superficial 2 mm mass in the superior orbit of a newborn [2]. To date, six other cases have been reported in childhood with protean clinical findings; none of these presented at birth [2–6]. In this report, we describe a massive hidrocystoma at birth and review the related literature.
Case presentation
A full-term, female child with severe right eye proptosis was referred to our department in central Iran two hours after birth by Caesarian section. The baby had a birth weight of 3410 g and Apgar scores of 10 at one and five minutes. She was the second child of a 30-year old healthy mother who had undergone cesarean section for her first child as well. History of trauma and amniocentesis or similar procedures during the pregnancy period was negative. Neither the parents nor the older brother had a history of systemic or ocular problems.
Severe right eye extrusion on admission
A large unicystic retrobulbar mass with hypo-intensity in T1 and hyper-intensity in T2 (a: Sagittal T1view, b: Axial T2 view, c: axial T1 views)
The patient’s presentation on the 5th day; proptosis began to worsen again
Excisional biopsy of the cyst on the 6th day. a, b: During the surgery, c: The excised cyst and the aspirated straw colored fluid)
Histopathologic examination; a and b: a cystic cavity lined by a layer of sweat duct like epithelium (flat to cuboid) with typical apical snouts consistent with the diagnosis of apocrine hidrocystoma was reported (Hematoxylin and eosin stain)
After 3 months of follow-up
Discussion
Presentation of hidrocystoma in the orbit is extremely rare to the extent that its presentation at birth, as a micro-cyst had only been reported once [2]. The point that makes our case very impressive was its massive size, orbital location and presentation at birth.
Reported cases of orbital hidrocystoma in childhood
Case number | Author(s) | Year | Patient Age; Sex | Presentation symptoms | Cyst size | Subtype | Radiologic features |
---|---|---|---|---|---|---|---|
1 | Saunders [2] | 1973 | At birth; Male | Medial superficial orbital mass, without globe displacement no visual axis involvement | 2 mm | Apocrine | CT: cyst without bone erosion |
2 | Mims et al. [3] | 1977 | NA | NA | NA | Apocrine | NA |
3 | Haider et al. [4] | 2005 | 4 months; Male | Inferior deep orbital mass with superior globe displacement | Large | Apocrine | CT: slight bone remodeling MRI: hypointense in T1 and hyperintense in T2 |
4 | Chung et al. [5] | 2007 | 20 days; Male | Medial deep orbital mass with lateral globe displacement | About 1.3 cm | Apocrine | MRI: hypointense in T1 and hyperintense in T2 |
5 | Malihi et al. [6] | 2015 | 8 y/o; Male | Supra-temporal deep orbital mass with inferior globe displacement and 2 mm proptosis, no diplopia or visual loss | Large | Eccrine | CT: erosion of lateral orbital wall MRI: Isointense in T1 and hyperintense in T2 |
6 | Malihi et al. [6] | 2015 | 13 y/o; Female | Superior superficial orbital mass without globe displacement, no diplopia, proptosis or visual loss, history of significant blunt trauma | Large | Apocrine | MRI: Isointense in T1 and hyperintense in T2 |
7 | Malihi et al. [6] | 2015 | 2 months; Male | Medial canthal superficial mass, no other symptoms | Small | Eccrine | Not performed |
8 | Present case | 2016 | At birth; Female | Deep retro-orbital mass, globe extrusion | Huge | Apocrine | MRI: hypointense in T1 and hyperintense in T2 |
From a pathological standpoint, hidrocystoma is a benign cyst originating from a sweat gland apocrine or eccrine in nature. The apical part of cellular cytoplasm is decapitated in the apocrine type (decapitation), while it remains intact in eccrine (exocytosis) glands [6]. This difference in secretion mechanisms gives the pathologic appearance of apical ‘snouts’ in the apocrine type.
In a review by Shield and Shield, the authors believe that differentiating between the main causes of orbital cysts without bone involvement in childhood may not be possible even with CT scan or MRI [7]. Differential diagnosis of what we presented in our neonate case may include: (i) surface epithelial lesions (e.g., conjunctival, respiratory epithelial, epidermal and dermoid cysts); (ii) teratomas; (iii) Neural cysts (e.g., cephalocele, congenital cystic eye); (iv) originating from adjacent structures (e.g., mucocele and dentigerous cysts); (v) parasitic cysts [7].
Conclusions
The picture of our case strongly argues against the well reputed theory of traumatic origin for orbital hidrocystoma by which it was postulated that this tumor may be the result of sweat gland cells implantation through the orbit [6]. Presentation of a large deep orbital hidrocystoma at birth in our case suggests the possible presence of choristomatous ectopic sweat gland cells in the orbit during embryogenesis.
Taken together, orbital hidrocystoma should be considered as a differential diagnosis of small/large; superficial/deep; congenial/post-traumatic; and, childhood/adult orbital cysts.
Declarations
Acknowledgments
We are very grateful to the child's parents for their patience and cooperation throughout the study.
Authors’ contributions
BE, MAA and SHA conceived the idea for the case study and followed the patient. MAA, SHA, SAS, ZK wrote the article and SAS and ZK revised it critically for important intellectual content. All authors read and approved the final manuscript.
Competing interests
The authors declare that they have no competing interests.
Consent for publication
Written informed consents were obtained from the parents for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the editor of this journal.
Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Authors’ Affiliations
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