• Users Online: 218
  • Print this page
  • Email this page


 
 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 32  |  Issue : 4  |  Page : 417-419

Recurrent periorbital cellulitis secondary to cyclic neutropenia


1 Department of Otolaryngology, Head and Neck Surgery, Tracheal Research Center, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
2 Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
3 Department of Ophthalmology and Visual Science, University of Alabama Medical School, Birmingham, Alabama, USA

Date of Submission19-Jan-2020
Date of Decision24-Mar-2020
Date of Acceptance11-Apr-2020
Date of Web Publication12-Dec-2020

Correspondence Address:
Mehdi Tavakoli
Callahan Eye Hospital, 1720 University Blvd., Suite 601, Birmingham, Alabama 35233
USA
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JOCO.JOCO_16_20

Rights and Permissions
  Abstract 


Purpose: To present a 1-year-old boy with cyclic neutropenia who presented with multiple episodes of periorbital cellulitis (POC).
Methods: The child presented with three episodes of POC. In the second episode, the cellulitis was associated with nasolacrimal duct obstruction, and in the third episode, a pansinusitis was noted. He underwent a thorough systemic evaluation.
Results: Patient's evaluation revealed the diagnosis of cyclic neutropenia.
Conclusion: This report emphasizes the possibility of an underlying immunodeficiency with recurrent POC, even in an apparently healthy child.

Keywords: Cyclic neutropenia, Dacryocystitis, Orbital cellulitis, Periorbital cellulitis, Sinusitis


How to cite this article:
Raad N, Bagheri A, Eskandari Y, Tavakoli M. Recurrent periorbital cellulitis secondary to cyclic neutropenia. J Curr Ophthalmol 2020;32:417-9

How to cite this URL:
Raad N, Bagheri A, Eskandari Y, Tavakoli M. Recurrent periorbital cellulitis secondary to cyclic neutropenia. J Curr Ophthalmol [serial online] 2020 [cited 2021 Jan 20];32:417-9. Available from: http://www.jcurrophthalmol.org/text.asp?2020/32/4/417/303196




  Introduction Top


Periorbital cellulitis (POC) is an inflammatory process of the eyelids and periocular soft tissues anterior to the orbital septum. POC can be potentially dangerous because of the possibility of progression to periorbital and orbital abscess, blindness, and intracranial complications. The most common origin of the POC in children is rhinosinusitis, and children with rhinosinusitis suffer from orbital complications more commonly than adults. POC might also be caused by trauma, external ocular infections, local skin inflammations, dental infections, and periorbital malignancies.[1]

Recurrent POC is a rare condition, with a few reports in the literature. Herein, the authors present a case of recurrent POC secondary to cyclic neutropenia, associated with the review of the literature.


  Case Report Top


A 1-year-old boy was admitted to our hospital with a 2-day history of right periorbital swelling and erythema [Figure 1]c. The patient had mild rhinorrhea, nasal congestion, and low-grade fever. Extraocular motility was normal, and there were no abnormalities in the pupillary reaction and optic nerves. He had two previous episodes of POC in the left eye when he was 6 and 8 months old which was treated in the ophthalmology department. The two previous episodes improved with intravenous antibiotics within 48 h. In the second admission, he also underwent successful probing for the left nasolacrimal duct obstruction [Figure 1]a and [Figure 1]b. Written informed consent has been obtained from parents of the patient for publishing the photographs.
Figure 1: Clinical photography of the patient: (a) First episode, presenting as left preseptal cellulitis in the medial canthal area at 6 months of age. (b) Second episode, presenting as a severe extensive left preseptal cellulitis at 8 months of age which improved after antibiotic therapy and nasolacrimal duct probing. (c) Third episode, presenting as right periorbital cellulitis at 12 months of age

Click here to view


His medical history showed that he was born at 35 weeks of gestation, and he had normal growth and met all developmental milestones. His immunization was up-to-date, and the family history was unremarkable.

We performed computed tomography scan of the paranasal sinuses that revealed sinusitis in both ethmoid and maxillary sinuses and evidence of inflammation in the right preseptal region [Figure 2].
Figure 2: Computed tomography scan of the orbit and paranasal sinus showing sinusitis in underdeveloped sinuses: (a) Axial image. (b) Coronal image

Click here to view


Diagnostic nasal endoscopy revealed pus in the right middle meatus, with no significant anatomical abnormality or polyps. The nasal discharge was cultured. On the 1st day of admission, complete blood count (CBC) demonstrated a total white blood cell count of 12,100/ml with a 4% neutrophil count. In subsequent CBC recheck, episodic neutropenia was confirmed. Other hematologic indices showed monocytosis, and no morphologic abnormalities of the neutrophils were detected in the blood smear.

Immunologic assessment including immunoglobulins level, antineutrophil antibodies, nitroblue tetrazolium blood test, and other evaluations for systemic disorders showed no abnormal findings. Cystic fibrosis was ruled out by sweat chloride test, and investigations for HIV antibody were negative.

The patient received intravenous antibiotics consisting of ceftriaxone (Jaber Ebne Hayyan Pharmaceutical, Tehran, Iran) and clindamycin (Alborz Darou, Qazvin, Iran) upon admission. The POC improved significantly after 48 h of intravenous administration of antibiotics, and he was discharged subsequently with oral antibiotics. After 6 days, the neutrophil count returned to normal range. We reviewed the medical chart of his admissions in the ophthalmology ward for the prior episodes and noticed similar neutropenia in those attacks, which was overlooked because of prompt response to treatment and early discharge.

The diagnosis of cyclic neutropenia was reconfirmed by serial CBC review (3 times/week for 8 weeks) and excluded acquired causes of neutropenia and immune deficiencies. In the neutropenic phase, neutrophil count dropped <200/mm[3]. He was referred to hematologic service and received granulocyte colony-stimulating factor and prophylactic antibiotics. In a 3-year follow-up, he remained asymptomatic, with no recurrence of cellulitis episodes.


  Discussion Top


POC is a relatively common disorder in the pediatric population, and a multidisciplinary approach including an otolaryngologist, ophthalmologist, and pediatrician is necessary for the best management outcome. Although rhinosinusitis is the most prevalent cause of POC, it has rarely been presented as the source of recurrent POC. Therefore, other underlying etiologies should also be considered and investigated in these patients.[1]

Various etiologies including the anatomical abnormalities have been described in patients with recurrent POC in the literature [Table 1]. Jatana et al. reported an abnormal uncinate process in continuity with the ethmoid bulla in a 28-month-old boy with seven episodes of recurrent unilateral POC due to recurrent rhinosinusitis.[3] Infections with unusual microorganisms have also been reported as a cause of recurrent POC. Mauriello and Atypical Mycobacterial Study Group presented atypical mycobacterial infections of the periocular region after periocular and facial surgery.[5]
Table 1: Etiologies that caused or mimicked recurrent periorbital cellulitis

Click here to view


Although recurrent POC often present in the same orbit, bilateral POC has rarely been reported.[2] In our patient, there was intermittent bilateral involvement, which means that the etiology of recurrent POC was not limited to one eye.

Neutropenia can present in several clinical settings. The most common etiologies of neutropenia are acquired, including immune neutropenia, neutropenia associated with sequestration, viral infections, and various medications. Inherited etiologies of neutropenia are less common and are often more profound. Cyclic neutropenia is a rare hereditary disorder with variable presentations. This disorder is characterized by periodic neutropenia lasting for 4–6 days with a 21-day interval. According to the absolute neutrophil count (ANC), neutropenia has been classified as mild (ANC: 1000–1500/mm[3]), moderate (ANC: 500–1000/mm[3]), and severe (ANC: <500/mm[3]).[15] In this patient, clinical characteristics and laboratory investigations revealed the severe form of cyclic neutropenia.

Opportunistic infections can occur during the neutropenic period. The most common sites of infection are skin, oral mucosa, and lungs.[15] Of note, our patient never presented recurrent infections in sites other than orbit and paranasal sinuses. In fact, it seems that the neutropenic episodes caused sinusitis and dacryocystitis as the underlying condition for the recurrent POC. In addition, he had no positive family history of the recurrent or unusual infections. In each episode, POC resolved within a short period following administration of intravenous antibiotics, and in the periods with the normal neutrophil count, there were no infectious signs.

In conclusion, we presented a child with recurrent POC secondary to cyclic neutropenia. This report signifies the importance of considering unusual etiologies including the immune system deficiencies when patients with recurrent POC are encountered.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Bagheri A, Tavakoli M, Aletaha M, Salour H, Ghaderpanah M. Orbital and preseptal cellulitis: A 10-year survey of hospitalized patients in a tertiary eye hospital in Iran. Int Ophthalmol 2012;32:361-7.  Back to cited text no. 1
    
2.
Caulley L, Corsten G, Hong P. Recurrent periorbital cellulitis in a very young child: A case report and review of literature. Int J Pediatr Otorhinolaryngol 2012;76:606-8.  Back to cited text no. 2
    
3.
Jatana KR, Grischkan JM, Skomorowski MJ, Elmaraghy CA. Recurrent unilateral periorbital cellulitis in a pediatric patient – An anatomic abnormality. Int J Pediatr Otorhinolaryngol 2008;72:1577-80.  Back to cited text no. 3
    
4.
Karkos PD, Karagama Y, Karkanevatos A, Srinivasan V. Recurrent periorbital cellulitis in a child. A random event or an underlying anatomical abnormality? Int J Pediatr Otorhinolaryngol 2004;68:1529-32.  Back to cited text no. 4
    
5.
Mauriello JA Jr., Atypical Mycobacterial Study Group. Atypical mycobacterial infection of the periocular region after periocular and facial surgery. Ophthalmic Plast Reconstr Surg 2003;19:182-8.  Back to cited text no. 5
    
6.
Hoare S. HIV infection in children – Impact upon ENT doctors. Int J Pediatr Otorhinolaryngol 2003;67 Suppl 1:S85-90.  Back to cited text no. 6
    
7.
Galindo-Ferreiro A, Alkatan HM, ElKhamary SM, AlDosairi S, Cruz AAV. Recurrent orbital inflammation mimicking orbital cellulitis associated with orbitopalpebral venous lymphatic malformation. Ophthalmic Plast Reconstr Surg 2017;33:e112-4.  Back to cited text no. 7
    
8.
Akcay EK, Cagil N, Yulek F, Yuksel D, Simsek S. Congenital lacrimal sac diverticulum as a cause of recurrent orbital cellulitis. Can J Ophthalmol 2009;44:e29-30.  Back to cited text no. 8
    
9.
Sorin A, April MM, Ward RF. Recurrent periorbital cellulitis: An unusual clinical entity. Otolaryngol Head Neck Surg 2006;134:153-6.  Back to cited text no. 9
    
10.
Rappaport K, Liesegang TJ, Menke DH, Czervionke LF. Plasmacytoma manifesting as recurrent cellulitis and hematic cyst of the orbit. Am J Ophthalmol 1996;122:595-7.  Back to cited text no. 10
    
11.
El-Hakim H, Ahsan F, Wills LC. Primary non-Hodgkin's lymphoma of the frontal sinus: How we diagnosed it. Ear Nose Throat J 2000;79:738, 741-3.  Back to cited text no. 11
    
12.
Hassan HM, Mc Andrew PT, Yagan A, Jacques TS, Hayward R. Mature orbital teratoma presenting as a recurrent orbital cellulitis with an ectopic tooth and sphenoid malformation – A case report. Orbit 2008;27:309-12.  Back to cited text no. 12
    
13.
Kim JW, An JH. Extranodal natural killer/T-cell lymphoma, nasal type, of the orbit mimicking recurrent orbital cellulitis. J Craniofac Surg 2014;25:509-11.  Back to cited text no. 13
    
14.
Younes JS, Simon MR, Moore EC, Bahrainwala AH. Recurrent periorbital cellulitis and otitis media in an asthmatic child with chronic diarrhea and short stature. Ann Allergy Asthma Immunol 2002;88:164-9.  Back to cited text no. 14
    
15.
Segel GB, Halterman JS. Neutropenia in pediatric practice. Pediatr Rev 2008;29:12-23.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1]



 

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
Introduction
Case Report
Discussion
References
Article Figures
Article Tables

 Article Access Statistics
    Viewed232    
    Printed28    
    Emailed0    
    PDF Downloaded27    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]