Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 
  • Users Online: 1020
  • Home
  • Print this page
  • Email this page

   Table of Contents      
BRIEF COMMUNICATION
Year : 2017  |  Volume : 65  |  Issue : 9  |  Page : 884-886

Giant eyelid eccrine hidrocystoma-induced progressive ptosis in childhood


Dr. Agarwal's Eye Hospital and Eye Research Centre, Chennai, Tamil Nadu, India

Date of Submission02-Feb-2017
Date of Acceptance03-Jul-2017
Date of Web Publication14-Sep-2017

Correspondence Address:
Amar Agarwal
Dr. Agarwal's Eye Hospital and Eye Research Centre, 19, Cathedral Road, Chennai - 600 086, Tamil Nadu
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_78_17

Rights and Permissions
  Abstract 

An upper lid eccrine hidrocystoma presenting as early childhood progressive ptosis is very rare. We present a 9-year-old female child with droopy right upper lid since birth and progressive increase in symptoms. She had right upper lid ptosis (marginal reflex distance 1 of −1 mm) with fair levator function (8 mm) and abnormal cystic change on the conjunctival side. Computerized tomography imaging delineated the well-defined cystic lesion with homogeneous cavity with no contrast enhancement. Following the cyst excision, a giant eccrine hidrocystoma measuring 25 mm × 15 mm was removed, the largest reported in pediatric eyes. The case demonstrates the possibility of giant lid eccrine hidrocystomas presenting as progressive ptosis at a pediatric age and the need for early surgical intervention to prevent amblyopia.

Keywords: Childhood acquired ptosis, eccrine hidrocystoma, giant hidrocystoma, progressive ptosis


How to cite this article:
Kumar DA, Agarwal A. Giant eyelid eccrine hidrocystoma-induced progressive ptosis in childhood. Indian J Ophthalmol 2017;65:884-6

How to cite this URL:
Kumar DA, Agarwal A. Giant eyelid eccrine hidrocystoma-induced progressive ptosis in childhood. Indian J Ophthalmol [serial online] 2017 [cited 2020 Jul 10];65:884-6. Available from: http://www.ijo.in/text.asp?2017/65/9/884/214659

Cystadenomas or hidrocystomas arising from eccrine glands are benign cystic or nodular lesion often seen in the periorbital skin.[1],[2] It is rarely multiple and may be associated with Goltz syndrome or ectodermal dysplasia.[3] Commonly, the periocular or lid hidrocystomas are small to moderate in size, about 5 mm and rarely larger. Although giant “orbital” eccrine hidrocystoma is known to occur in early infancy, the “eyelid” is a very rare site in children.[1],[2],[4],[5],[6],[7] We present a giant eccrine hidrocystoma of upper lid presenting in a child as progressive ptosis.


  Case Report Top


A 9-year-old female child presented to our Oculoplasty Clinic with painless, progressive drooping right eye (OD). Her parents had noticed the drooping since birth, and there had been significant increase in the past 2 years. On ocular examination, her unaided visual acuity was 20/30 in OD and 20/20 in the left eye, respectively. Right upper lid ptosis was observed with marginal reflex distance (MRD 1) of −1 mm with significant reduction in vertical fissure height (5 mm) [Figure 1]. The MRD in the fellow eye was 4 mm. Abnormal cystic change was detectable on the conjunctival side of the upper lid [Figure 1]. However, no discrete mass was palpable. A fair levator muscle action (8 mm) in the right eye and good Bell's phenomenon was elicited. The extraocular movements were normal and the eyes were orthophoric. Her dilated fundus examination and intraocular pressure were within normal limits in both eyes. B-scan ultrasound examination of upper lid demonstrated fluid-filled cavity with no internal echoes [Figure 2]. Computerized tomography showed well-defined cystic mass with four regular margins measuring 21 mm × 19 mm × 6 mm with no contrast enhancement [Figure 2].
Figure 1: Preoperative clinical photograph showing the ptosis (a) and cystic conjunctival surface (b)

Click here to view
Figure 2: Intraoperative picture of the giant eccrine cystic mass (a and b) measuring 25 mm × 15 mm

Click here to view


Upper lid mass excision was performed [Figure 3], and the histopathology confirmed and showed 25 mm × 15 mm cystic mass [Figure 4] lined by double-walled (1 mm thick) cuboidal epithelium as eccrine hidrocystoma. The cyst cavity was filled with clear fluid. The postoperative period was uneventful with mild lid edema and conjunctival congestion that resolved spontaneously. There was significant improvement in the MRD (3 mm) after excision. At 1-month postoperative follow-up, the MRD was stable at 3 mm with no lid edema or recurrence at 6 months [Figure 5].
Figure 3: Ultrasound B-scan (a) and contrast-enhanced computerized tomography scan (b) showing cystic lesion of the upper lid

Click here to view
Figure 4: Histopathology showing the double-walled cyst lined by cuboidal epithelium. (a) (H and E, ×40) (b) (H and E, ×400)

Click here to view
Figure 5: Clinical photograph showing immediate day 1 (a), 1-month (b), and 6 months (c) postoperative period

Click here to view



  Discussion Top


Sweat glands can be eccrine (open in epidermis) or apocrine (open through follicle) in type. Hidrocystomas are benign cystic masses that emanate from an obstructed sweat gland duct with subsequent fluid retention.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10] Moreover, it is reported to constitute only 1% of childhood eyelid tumors.[4] Although giant orbital eccrine hidrocystomas have been observed in pediatric age, isolated giant eyelid eccrine hidrocystomas are less common. Orbital eccrine hidrocystoma in two pediatric cases has been reported by Malihi et al.[1] Of the two, one was an infant presenting 2 weeks after birth with orbital eccrine hidrocystoma. Chung et al. reported a 20-day-old child with dystopia and complete lid closure following a congenital superior orbital hidrocystoma.[7] Congenital hidrocystomas are often apocrine and present commonly as orbital mass and rarely as ptosis.[2] Eshrahi reported orbital hidrocystoma of apocrine type presenting at birth as proptosis.[10] Sheth and Raina in their report has shown ptosis in adult-onset giant eccrine hidrocystoma presenting along with epiphora.[6] Similarly, Rodallec et al. reported adult-onset hidrocystoma with progressive ptosis.[5] Mukherjee et al. reported giant apocrine hidrocystoma presenting with ptosis and dystopia in an adult.[8] Ferraz et al. showed isolated ptosis in adult-onset orbital hidrocystoma which presented as occult in two cases and as subcutaneous mass in one.[11] Eccrine hidrocystoma of the eyelid as studied by Singh et al. has been noted to have a median size of 1 mm and majority are adult-onset (mean age 59 years).[12] In our literature search (PubMed and Google search using keywords), we noted no clinical case report that showed progressive ptosis in a child as the presenting complaint in giant eyelid eccrine hidrocystoma. Although atypical presentations of hidrocystoma, where an apocrine hidrocystoma presented as a lacrimal gland mass with mechanical ptosis in adults, have been reported, those of an eccrine nature are very few.[8]

While favored management for an asymptomatic small hidrocystoma in children is only observation, surgical excision is advocated in cases with vision-threatening symptoms or unacceptable cosmetic appearance. Unlike orbital cysts, which are frequently symptomatic at the beginning, eyelid lesion may be asymptomatic until it grows in size to induce symptoms such as ptosis. Progressive ptosis has probably induced the drop of one line in the affected eye due to sensory deprivation and pressure. Although large or giant eccrine hidrocystomas are known to originate in orbit, it is rare to notice the giant eccrine mass on the eyelid, and as a matter of fact, this is the largest eccrine eyelid hidrocystoma (25 mm × 15 mm) removed from the pediatric age group.


  Conclusion Top


Eccrine hidrocystoma can be considered one of the differential diagnoses in progressive ptosis in the pediatric age group and timely imaging can aid diagnosis. Early interventions in such symptomatic cases also prevent amblyopia due to mechanical ptosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Malihi M, Turbin RE, Mirani N, Langer PD. Giant orbital hydrocystoma in children: Case series and review of the literature. Orbit 2015;34:292-6.  Back to cited text no. 1
    
2.
Shields JA, Shields CL. Orbital cysts of childhood – Classification, clinical features, and management. Surv Ophthalmol 2004;49:281-99.  Back to cited text no. 2
    
3.
Sarabi K, Khachemoune A. Hidrocystomas – A brief review. MedGenMed 2006;8:57.  Back to cited text no. 3
    
4.
Mims J, Rodrigues M, Calhoun J. Sudoriferous cyst of the orbit. Ophthalmol 1977;12:155-6.  Back to cited text no. 4
    
5.
Rodallec T, Abada S, Nordmann JP. Atypical giant hidrocystoma of the eyelid. J Fr Ophtalmol 2006;29:226-30.  Back to cited text no. 5
    
6.
Sheth HG, Raina J. Giant eccrine hidrocystoma presenting with unilateral ptosis and epiphora. Int Ophthalmol 2008;28:429-31.  Back to cited text no. 6
    
7.
Chung JK, Lee SJ, Kang SK, Park SH. Congenital sudoriferous cyst within the orbit followed by esotropia. Korean J Ophthalmol 2007;21:120-3.  Back to cited text no. 7
    
8.
Mukherjee B, Desai A, Krishnakumar S, Biswas J. A giant apocrine hidrocystoma presenting as lacrimal gland mass. Orbit 2015;34:342-4.  Back to cited text no. 8
    
9.
Saunders JF. Congenital sudoriferous cyst of the orbit. Arch Ophthalmol 1973;89:205-6.  Back to cited text no. 9
    
10.
Eshraghi B, Abtahi MA, Sonbolastan SA, Kasaie Z. Presentation of massive orbital hidrocystoma at birth: Case report and review of the literature. Eye Vis (Lond) 2017;4:5.  Back to cited text no. 10
    
11.
Ferraz LB, Burroughs JR, Satto LH, Natsuaki KL, Meneguin RL, Marques ME, et al. Three adult cases of orbital hidrocystoma presenting with blepharoptosis. J Clin Med 2015;4:150-8.  Back to cited text no. 11
    
12.
Singh AD, McCloskey L, Parsons MA, Slater DN. Eccrine hidrocystoma of the eyelid. Eye (Lond) 2005;19:77-9.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
Abstract
Case Report
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed1016    
    Printed21    
    Emailed0    
    PDF Downloaded124    
    Comments [Add]    

Recommend this journal