Periorbital varicella gangrenosa: A rare complication of chicken pox.

A previously healthy six year old male child presented in pediatrics ICU in state of shock with history of fever and rashes and later was diagnosed as chicken pox. He developed right sided periorbital varicella gangrenosa which is a form of necrotizing fasciitis secondary to skin infection. Patient was treated with intravenous acyclovir, antibiotics, amphotericin B, extensive debridement and later reconstruction of upper eyelid with skin grafting. Aggressive treatment helped preventing the eyeball and orbital involvement which would have necessitated orbital exenteration. However delayed presentation resulted in necrosis of orbicularis oculi and underlying tissue which resulted in graft retraction and lid dysfunction. Clinicians should be aware of this rare but fulminating condition to minimise the sight and life threatening complications associated with it.

Introduction

Varicella, commonly known as chickenpox is a disease of childhood caused due to varicella zoster virus. Secondary infections of the skin like abscess, cellulitis, necrotizing fasciitis and gangrene are most common complications of varicella viral infections. Group A beta-hemolytic streptococcus is the most common bacterial pathogen responsible for secondary skin infections.[12] Gangrene is a rare complication of varicella with a frequency of 0.05-0.16%.[3] Periorbital skin infections are uncommon due to high vascularity of facial skin.

We came across a case of periorbital varicella gangrenosa affecting the right side in a 6-year-old male child which was treated with repeated debridement and skin grafting. As the condition is rare and only two case reports in the literatures are observed on it, we wish to report this case, its aggressive presentation and the challenges faced during its management to save the eye.

Case Report

A 6-year-old male child was admitted in pediatric intensive care unit in the state of shock with history of fever and rashes for four to 5 days, letharginess and swelling over the right upper eyelid for 1 day. Patient was diagnosed as case of varicella zoster by pediatricians. Intravenous antibiotics (Ceftazidime and Azithromycin) and acyclovir was started. The general condition of patient started improving but swelling over the eyelid worsened with associated peeling of lid skin. Over the next 10 days periorbital skin of right side developed necrotic changes. There was no history of trauma or surgery in periorbital region.

At time of presentation in the ophthalmology department the periorbital skin, orbicularis oculi and underlying tissue were necrosed [Figure 1a]. Patient was diagnosed as periorbital varicella gangrenosa on the basis of (a) development of secondary skin infection following varicella eruptions (b) necrotising infection along subcutaneous planes and (c) absence of any trauma or surgery inciting it. A blood and pus culture from the affected area was sent and debridement of necrotic tissue was planned under general anesthesia. The cultures were positive for Staphylococcus aureus, pus was also found positive for candida. Hence, treatment with intravenous vancomycin and amphotericin B was initiated and debridement was planned under general anaesthesia.

Figure 1

(a) At the time of presentation showing extensive right periorbital necrosis. (b) After first debridement showing necrotic base. (c) After second debridement showing healthy granulation and development of lagophthalmos. (d) Lateral and paramedian tarsorrhaphy to prevent corneal exposure. (e) Postoperative picture in primary gaze. (f) Postoperative picture in downgaze

During debridement examination under anaesthesia right eye showed conjunctival congestion and chemosis temporally, rest of the eyeball was normal. The periorbital necrosis was found to be extensive in area as well as depth. Superiorly it involved the upper eyelid till the eyebrow, laterally it extended beyond the lateral orbital margin, inferior extent was till the lower orbital rim and nasal extension was 5-6 mm beyond the medial canthus. In depth it involved orbicularis and underlying tissue, the bone was free. An extensive debridement was done, barely 2-3 mm of upper lid skin along with the lid margin was salvageable [Figure 1b]. The necrosis spread in subcutaneous plane, at places the healthy skin could be lifted over the subcutaneous necrotic areas. Daily wound dressing with local antibiotics and regular ocular examination was performed to look for intraorbital extension of infection. A repeat debridement was required after 3 days, following which the wound showed healthy granulation. At the same time, lagophthalmos due to contracture was noticed [Figure 1c] for which temporal and paramedian tarsorrhaphy was performed [Figure 1d]. Six weeks later release of contracture and reconstruction of upper lid with free full thickness skin graft from medial aspect of forearm skin was performed. Follow up at 12 weeks showed a well taken up graft with lagophthalmos due to residual contracture [Figure 1e] for which patient was advised lubricating eye drops.

Discussion

The term varicella gangrenosa was first coined by Hutchinson. It is a dreaded complication of varicella first described by Stokes in 1807[4] It represents one form of necrotising fasciitis consequent to subcutaneous streptococcal infection.[5] Staphylococcus and other bacteria have also been isolated from cases of periorbital necrotising fasciitis.[6] Early recognition and aggressive treatment is important to prevent complications which can lead to disfigurement and loss of vision. Necrotizing fasciitis related to varicella generally presents between 3rd and 7th day from onset of eruptions, initially it can be confused with cellulitis. Periorbital necrotizing fasciitis has also been observed following trauma and surgery.[7] It has also been suggested that administration of nonsteroidal antiinflammatory drugs in chicken pox might enhance development of necrotizing fasciitis.[8] Timely initiation of acyclovir therapy can reduce complications, recommended dose of acyclovir in an uncomplicated case is 800 mg five times a day for 7 days while intravenous acyclovir is administered as 10 mg/kg for seven-ten days in complicated cases. Repeated debridements and intravenous antibiotics are important to prevent spread of skin infections. Differentiation of this entity from mucormycosis is important in diabetic patients. Intravenous amphotericin is indicated if hyphal elements are found on sections of necrotic tissue.[9]

Varicella gangrenosa affects parts of body like trunk and extremities but facial involvement is very rare due to its rich vascular supply.[3] Most literatures related to necrotising fasciitis in periorbital region mention trauma as the initial event leading to disruption of skin barrier. Despite best efforts we found only two cases of periorbital necrotising fasciitis secondary to varicella in literatures.

Thomas et al. reported varicella gangrenosa in an adult where exenteration was performed to limit the spread of infection.[3]

Another case of necrotizing fasciitis after varicella has been reported in 7-year-old female child by Rafael Cornedor-Osorio et al. where lower lid reconstruction was performed with a post auricular graft.[10] However authors have not used the term varicella gangrenosa here.

Our case is a 6-year-old male child with extensive periorbital necrosis who was managed by repeated surgical debridement with administration of intravenous antibiotics, amphotericin B and acyclovir. Severely affected upper eyelid was later reconstructed with skin graft from forearm. As the upper lid plays the main role in blinking and closure of eye lids, dysfunction persisted despite lid reconstruction [Figure 1f] unlike the case reported by Rafael et al. where lower lid was affected.

Our main objectives in managing this patient were to prevent intraorbital extension of infection, preserving the sight and restoration of eyelid functions. We were successful in preserving the eye and sight but restoration of upperlid functions remained compromised despite lid reconstruction due to delayed presentation. Further delay in treatment could have led to disastrous complications due to orbital spread of infection. Earlier intervention would definitely have resulted in a better outcome. The present case emphasises the importance of awareness of the nature, cause and treatment of this rare but potentially devastating condition among pediatricians as well as ophthalmologists.