Periorbital necrotising fasciitis in infants: Presentation and management of six cases

Introduction

Necrotising fasciitis (NF) is a rare but life-threatening soft tissue infection. It starts as a cellulitis with erythema and oedema of the skin and subcutaneous tissues, and spreads rapidly leading to necrosis of the fascia and the overlying soft tissue. ¹ The infection can spread along the facial planes to vital structures leading to shock and organ failure.^1–5 It usually involves the extremities, abdominal wall and groin.

Periorbital NF is relatively rare with over 100 documented cases reported thus far.^3,5 It is essentially a disease of adults with significant ocular morbidity and facial disfigurement.^3,5 Though mortality may be infrequent in localized periorbital disease, severe lid deformities, visual loss due to orbital invasion and/or central retinal artery occlusion have been reported, there is rarely a need for exenteration.^2–5 Only a few sporadic cases have been reported in children.^3,6–10 We present a series of six infants with periorbital NF that were managed at our centre and which, to the best of our knowledge, is the largest series in the literature.

Methods

Children diagnosed with periorbital NF and managed at our centre over a period of 2 years were included in the series. The presenting features, systemic work-up, management and rehabilitative surgery carried out were analysed.

Case reports

Case 1

A 5-month-old girl presented with a painful swelling involving the left upper and lower eyelids of 3 days’ duration. The eyelids were tense, erythematous with large areas of necrosis sparing the eyelid margin. The induration and erythema extended to the lower face (Figure 1). At presentation, the child was febrile and lethargic. There was no history of any previous systemic illness, antecedent trauma or precipitating lesion prior to the onset of the eyelid inflammation. She was immunised for her age. Systemic examination did not reveal any other focus of infection. OPEN IN VIEWER

Figure 1.

Clinical photograph of case 1 showing necrosis of left sided eyelids.

A clinical diagnosis of NF was made and the child was admitted and started on broad spectrum intravenous (vancomycin and ceftriaxone). Surgical debridement of the necrotic skin and subcutaneous tissue was done the next day. The anterior and posterior segment of the eye was normal on examination under anaesthesia. A full blood count showed leucocytosis. Group A β-haemolytic Streptococci were isolated.

Intravenous antibiotics were continued for 10 days along with repeated debridement of the necrotic area (Figure 2). Oral (amoxycillin-clavulanic acid) were further continued for another week. The lesion healed over 2 weeks with cicatricial ectropion of the upper eyelid and a scar in the lower eyelid. Full thickness skin harvested from the retroauricular area was subsequently grafted for the upper eyelid at 4 weeks. Adequate globe coverage and eyelid motility was found up to a follow-up of 9 months (Figure 3). OPEN IN VIEWER

Figure 2.

Photograph of the child one week after surgical debridement and antibiotics.

OPEN IN VIEWER

Figure 3.

Shows adequate globe coverage with minimal lagophthalmos at 9 months follow up after full thickness skin grafting of upper eyelid.

Case 2

A 10-month-old boy presented with extensive swelling and necrosis involving the left upper and lower eyelids, lateral canthus and cheek (Figure 4). The child was febrile, suffering from severe malnutrition and unimmunised. The inflammation had progressed rapidly over 4 days with no response to oral antibiotics that had been started elsewhere. The child was admitted and started on intravenous (vancomycin and ceftriaxone). Surgical debridement was carried out within 24 h. OPEN IN VIEWER

Intraoperatively, extensive necrosis of periorbital and cheek tissues with complete loss of lower eyelid was noted. The globe was fixed medially. A full blood count showed leucocytosis with raised ESR. CT scanning revealed involvement of the anterior orbit. A repeat debridement was performed after another 2 days following which a rapid healing of the lesion was seen subsequently (Figure 5). Wound cultures grew Pseudomonas aeruginosa. Oral ciprofloxacin was substituted after 1 week and continued for a further 2 weeks by which time the lesion had healed with total loss of lower eyelid and medial canthal tissue, mechanical ptosis of upper eyelid, restrictive esotropia and residual corneal opacity. Reconstruction of the lower eyelid using a Hughes tarso-conjunctival flap with a full thickness skin graft (harvested from the retroauricular area) failed. Repeated reconstructive interventions were performed over 1 year. However, owing to extensive scarring of the periorbital tissue, the child has persistent lagophthalmos and esotropia (Figure 6). OPEN IN VIEWER

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Figure 6.

Shows the child at 1 year follow up with failure to form lower eyelid despite multiple surgeries.

Case 3

A 7-month-old girl presented with an erythematous tender swelling of the left upper and lower eyelids of 2 days’ duration. There was a large area of necrosis in the upper eyelid sparing the lid margin. The eyeball was normal with no signs of orbital involvement. The child was febrile with poor oral intake. She was immunised with no history of predisposing lesion or trauma.

She was admitted and treated with intravenous vancomycin and ceftriaxone for 1 week. Debridement of the necrotic tissue was performed the same day. Group A β-haemolytic Streptococci were cultured. Oral amoxycillin-clavulanic acid was substituted and given for 2 further weeks by which time the lesion had healed with severe cicatricial ectropion of the upper eyelid and lagophthalmos. A tarsorrhaphy to prevent corneal exposure failed. A full thickness skin graft was performed at 3 weeks. However, a repeat graft was required for residual lagophthalmos at 6 months although an adequate cosmetic outcome with eyelid motility was achieved.

Case 4

A 6-month-old boy presented with progressive swelling and necrosis of the left upper eyelid over a period of 3 days. There was associated induration of the lower eyelid and cheek. The eyeball examination was within normal limits. The child was febrile with no other focus of infection and adequately immunised.

He was treated with intravenous (vancomycin and ceftriaxone) and repeated surgical debridement for 1 week followed by oral amoxycillin-clavulanic acid for a further week. Staphylococcus aureus and β-haemolytic Streptococci were isolated. The inflammation healed within 10 days with an upper eyelid scar resulting in ectropion and lagophthalmos. A full thickness skin grafting was subsequently performed after 5 weeks which resulted in adequate globe coverage and functional recovery at follow-up of 6 months.

Case 5

A 9-month-old boy presented with NF involving the right upper eyelid and associated swelling of the lower eyelid and face for 4 days. Except for ocular surface congestion, the eyeball examination was normal. He was febrile at presentation. There was no systemic illness prior to the onset of eyelid necrosis. Systemic examination was otherwise normal and child was immunised.

After surgical debridement of the necrotic tissue and 1 week of intravenous vancomycin and ceftriaxone followed by oral amoxycillin-clavulanic acid for another week, the lesion healed with a residual cicatricial ectropion. Group A β-haemolytic Streptococci were grown. A full thickness skin grafting of the eyelid was performed at 3 weeks, followed by a repeat graft for residual lagophthalmos after 5 months. Globe coverage and eyelid motility were adequate at a follow-up of 1 year.

Case 6

An 11-month-old boy presented with a tender swelling of the left upper and lower eyelids associated with fever and lethargy of 3 days’ duration. There was violaceous skin discoloration with necrosis of the upper eyelid. No clinical signs of orbital involvement were found and fundus examination was normal. Systemic examination did not show any other abnormality.

He was treated with intravenous vancomycin and ceftriaxone for 10 days followed by oral amoxycillin-clavulanic acid for 1 week and repeated surgical debridement. Group A β-haemolytic Streptococcus was the causative organism isolated. A full thickness skin graft was performed for cicatricial ectropion of the upper eyelid at 3 weeks. At a follow-up of 4 months, the eyelid was mobile and there was minimal lagophthalmos.

Discussion

Periorbital NF is a rare infection of adults and even rarer in children. A systematic review by Lazzeri et al. of cases reported in the published literature between 1950 and 2008 identified 103 cases involving the eyelids and periorbita. The average age was 50.2 years ± 19.3 SD. ³ In a prospective observational study published in 2014, 30 cases of NF were identified in the UK over a 2-year period. The age of patients was in the range of 33–88 years, with a median age of 68 years. ⁵ Only a few isolated cases have been reported in children.^3,6–10 All six children in our study were infants. Thus, though rare and largely unreported, this potentially fatal and disfiguring disease can involve very young children. The initial non-specific appearance of the disease may be confused with other inflammatory conditions such as orbital and preseptal cellulitis which are more commonly seen in children. Hence, remembering the possibility of NF in the differential diagnosis is important for early recognition and treatment.

The most common causative organism in periorbital NF is β-hemolytic Streptococcus (53%, ³ 76% ⁵ ) as was also found in four of our cases. Other rare causative organisms reported include S. aureus (isolated in one case), Pseudomonas aeruginosa (one case), Klebsiella, E.coli, Bacteroides and Moraxella catarrhalis.^2–5 Unlike NF of the extremities and trunk, polymicrobial infection in the periorbital area is rare.^3,5 One child in our series had this.

Various risk factors have been described in adults for NF including alcoholism, diabetes mellitus, rheumatologic disease, systemic malignancy and corticosteroid use.^3,5 According to a review, about half of the reported patients, however, were healthy with no predisposing conditions. ³ These may be trauma, surgery, respiratory infection, dental extraction and insect bite.^2,3,5 In children, NF can be found involving different parts of the body without predisposing immunosuppression. ¹¹ Some of the earlier cases with periorbital involvement were reported in healthy children.^6–8 In our series, one child was severely malnourished. Moreover, he had extensive involvement of the eyelids, orbit and face with loss of vision. Pseudomonas being the causative organism in this particular case may have contributed to a more fulminant infection. There are two previous similar reports of Pseudomonal NF involving the periorbital region in a 16-month-old girl with malnutrition and an infant with leukocyte adhesion defect, respectively.^9,10 The five other children in our series were healthy prior to onset of infection with no initiating factors that could be identified.

NF initially presents as cellulitis and progresses rapidly as the infection spreads subcutaneously. Within 1–2 days, the skin becomes cyanotic with erythematous borders followed by development of gangrene over the next 4–5 days. ² The clinical course of the disease is different in the eyelids owing to thin eyelid skin which results in early detection of infection. Moreover, the well vascularised orbicularis oculi muscle acts as a barrier between the skin and the underlying spreading periorbital infection. This may help to decrease the extent of skin involvement.^2,3 All our cases presented within 4 days from the onset of the infection with frank cutaneous gangrene. Moreover, sparing of the eyelid margin was seen in all except one child who had fulminant Pseudomonal infection. Two studies have reported bilateral involvement of periorbital NF in 86% and 33% of cases, respectively, probably because the subcutaneous tissue over the nose does not provide much resistance to the spread of infection.^2,5 None of our cases had bilateral involvement, though involvement of both upper and lower eyelids along with spread of inflammation to the lower part of face was seen in all cases.

NF is a potentially fatal infection with a mortality rate of 30–70% if left untreated. ⁴ In patients with periorbital involvement, the mortality rate has been reported to be 14%.^2,3 Moreover, there is risk of central retinal artery occlusion with loss of vision, the need for exenteration for deep orbital involvement and severe disfigurement.^2,4 In our series, orbital involvement was found in only one case (case 2). This child developed a residual corneal opacity and restrictive esotropia. He was unable to fixate on the largest Cardiff card with this eye. According to a recent report, orbital involvement was the single biggest risk factor for poor visual outcome. ⁵ In the other five cases, where the infection was preseptal, the globe was normal and the cornea remained clear with no visual loss.

The key to successful management is early diagnosis and intervention.^2,3,5 This is even more the case in children where the diagnosis may be confused with preseptal or orbital cellulitis. While antibiotics are required to treat both conditions, the mainstay of treatment for NF is urgent surgical debridement.^2–5 It is essential to remove all necrotic tissue which serves as a nidus for infection. It is recommended that the debridement until viable tissue is reached, this should mainly be done for the subcutaneous tissue while preserving as much viable skin as possible in the facial region. ¹²

There have been, however, reports of patients of periorbital NF treated conservatively, without surgical debridement. ⁵ We agree with the authors of a recently published study that prompt debridement is a crucial part of treatment in NF where sight as well as life is threatened. ⁵ Moreover, in our series, all were infants who presented with fulminant and rapidly progressive eyelid inflammation and necrosis. These children were febrile, lethargic and not taking adequate feeds.

In view of a life-threatening infection in a much younger age group, we used a combination of vancomycin and ceftriaxone empirically. We performed surgical debridement within 24 h. Thereafter, repeated bedside debridement of necrotic tissue was done as and when required. Treatment for underlying malnutrition was started simultaneously in one child.

A line of demarcation starts forming at 2–3 days and healing was completed in all cases by 2–3 weeks. In one child with Pseudomonas infection, the lesion healed with restrictive esotropia, corneal opacity and severe lagophthalmos.

Unilateral involvement in all cases and orbital spread in one child in our series, suggests that probably the severity and spread of disease is slower in infants than in adults where bilateral involvement is frequently seen.^2,3,5 Moreover good response to treatment was seen within 48 h with no mortality in our series.

Reconstruction for the residual cicatricial ectropion and lagophthalmos needs to be done once the infection has resolved. Excision of the scar tissue followed by skin grafting for the resultant defect is usually sufficient as the eyelid margin is spared in most cases.^3,5,14 In five of our cases, a full thickness skin grafting was done after 3–5 weeks in order to provide globe coverage and cosmesis. A repeat graft was required in two of these cases for residual lagophthalmos. In one child with Pseudomonal infection, there was complete loss of lower eyelid along with the eyelid margin. This required several reconstructive interventions.

To conclude, periorbital NF can occur in infants often without any predisposing factors. Early diagnosis and aggressive treatment is vital to prevent mortality and to reduce morbidity. The infection heals with severe scarring that needs timely reconstructive surgery for cosmetic and functional rehabilitation. The overall prognosis in infants should be good.