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* Corresponding author: Bishara Atiyeh, MD (FACS), Professor of Surgery, American University of Beirut Medical Center, Division of Plastic & Reconstructive Surgery,Beirut, Lebanon. Tel.: +961 3 340032; email: batiyeh@terra.net.lbManuscript: submitted 06/08/2019, accepted 07/08/2019

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CASE REPORT

FROM ACUTE MANAGEMENT TO ULTIMATE RECON-STRUCTION: A 15-YEAR FOLLOW-UP OF A PEDIATRIC PA-TIENT WITH NECROTIZING FASCIITIS SECONDARY TOVARICELLA INFECTION

DU TRAITEMENT INITIAL À LA RECONSTRUCTION: SUIVI SUR 15ANS D’UN ENFANT VICTIME D’UNE FASCIITE NÉCROSANTE APRÈSUNE VARICELLE

Habr N.,1 Chahine F.M.,2 Atiyeh B.1�

1 American University of Beirut Medical Center, Beirut, Lebanon2 Trad Hospital Medical Center, Beirut, Lebanon

SUMMARY. Necrotizing fasciitis is a rapidly progressive, life-threatening soft tissue infection. The usuallyaggressive surgical debridement often leaves disfiguring sequelae. We hereby present the case of a 4-year-old boy who presented with post varicella zoster necrotizing fasciitis over his trunk, and follow his journeypost reconstruction with split thickness skin grafts and the sequelae of grafting over the trunk. While thecase itself is not unique in its presentation, this is the first report of a long-term follow up, with managementof the long-term reconstructive sequelae.

Keywords: necrotizing fasciitis, tissue expansion, abdominal reconstruction

RÉSUMÉ. Les fasciites nécrosantes (FN) sont des infections suraiguës graves des tissus cutanés et souscutanés. Leur traitement chirurgical, fait de débridements agressif, laisse le plus souvent des séquellesdélabrantes. Nous présentons ici le cas d’un enfant ayant subi à l’âge de 4 ans une FN du tronc consécutiveà une varicelle, au long de son parcours de reconstruction par greffes et sur ces greffes. Bien que ce cassoit initialement non exceptionnel, c’est la première fois que la reconstruction est présentée.

Mots-clés: fasciite nécrosante, expansion cutanée, reconstruction abdominale

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Introduction

Necrotizing fasciitis is a rapidly progressive, life-threatening soft tissue infection.1 Although classifiedinto two types based on the causative bacterial orga-nisms,2 it is rarely reported as a dreaded and deadlycomplication following primary varicella infection inimmunocompetent children.3

The management of necrotizing fasciitis is timesensitive, and wide surgical debridement remains thelife-saving measure, in addition to the requirement forintensive care monitoring as well as medical treatmentwith antimicrobial agents.4 The usually aggressivesurgical debridement often leaves disfiguring seque-lae. In addition, reconstructive efforts usually involveskin grafting the extensive wounds.5

We present the case of a 4-year-old boy who presen-ted with post varicella zoster necrotizing fasciitis overhis right flank and abdomen, and follow his journey postskin grafting of the open wounds and subsequent scarrevision and reconstruction. While the case itself is notunique in its presentation, this is the first report of along-term follow up of a pediatric patient with necroti-zing fasciitis secondary to varicella infection.

Case presentation

A previously healthy 4-year-old boy presented to thepediatric service at the American University of BeirutMedical Center with high-grade fever and increased ab-dominal girth, associated with vesicular lesions over theupper and lower extremities (Fig.1A). He was diagno-sed with varicella and discharged home on antihista-mine.

He presented again two days later with persistenthigh-grade fever and a progressive right lower quadrantviolaceous discoloration, associated with crepitus(Fig.1B).

He was admitted to the pediatric intensive care unitfor observation and management. His condition dete-riorated over the following two days with extension ofthe violaceous discoloration to the right flank and back.Necrotizing fasciitis was suspected. CT scan showedsubcutaneous fluid collection in the abdominal wallwith a circumferential distribution associated with ab-dominal wall edema and evidence of bowel distention.

The patient was rushed to the operating theatre,where he underwent wide debridement of all non-viable and grossly infected abdominal skin and sub-cutaneous tissues (Fig.2A).

Histologic examination of the debrided tissues re-vealed inflamed tissues infiltrated with polymorpho-nuclear leukocytes and superficial subdermalexudates. The dermis exhibited also extensive bandsof fibrosis and necrosis, and the blood vessels sho-wed intraluminal thrombi. A mixture of neutrophilsand eosinophils were noted along the whole thick-ness of the skin. Bacterial overgrowth was also notedin the tissues. Cultures grew beta hemolytic strepto-coccus, group A, heavy growth.

Ultimately, with topical wound care and dailydressings, the extensive defect of the anterior abdo-minal wall and right lateral trunk developed granu-lation tissue (Fig.2B) that was later covered withsplit thickness skin graft (Figs.2C and 2D).

Fig. 1 - (A) Chicken pox vesicular lesions over trunk and upper extre-mity upon patient’s initial presentation and (B) Violaceous discolorationover lower abdomen upon presentation with necrotizing fasciitis

Fig. 2 - Abdominal wound (A) after debridement (B) following granu-lation tissue formation, and (C, D) ultimate split thickness skin grafting

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The patient was seen regularly for follow up and hisgrowth was monitored for any developing deformity orcontracture that necessitated immediate correction.

Final scar revision was delayed until the child be-came more cooperative, since anticipated reconstruc-tion required at least two stages of prolonged tissueexpansion.

Thirteen years later, as a teenager, he became seve-rely embarrassed by his scars and the resultant defor-mity (Fig.3). Though his main concern was aesthetic,he did complain of shortness of breath on exertion.

On physical examination, the patient had a pronoun-ced hourglass deformity of his trunk, with tight abdomi-nal skin grafts, extending from the lower chest to theinguinal area. CT scan of his chest, abdomen and pelvisshowed a markedly elevated diaphragm with displacedabdominal organs into the chest (Fig.4A).

Two rectangular 800 mL unidirectional expanderswere placed in the first stage.

After three months of serial expansion and overex-pansion to 1200 mL, the skin grafts were excised par-tially over the abdominal wall, and the expanded flapswere advanced. Further mobilization of the flaps couldbe achieved by liposuction of the adjacent subcutaneoustissues resulting in what has already been reported as re-verse expansion.6

A repeat CT scan of the abdomen and pelvis area sho-wed a clear release of the waist constriction with netdownward displacement of the diaphragm and return ofabdominal organs to the abdominal space (Fig. 4B). Itrevealed also an epigastric hernia that developed follo-wing excision of the tight scar overlying the linea albaover the upper abdominal wall.

A second cycle of tissue expansion was performedsix months later to excise the remaining skin graft andreconstruct the residual defect on the right flank and backand lower abdomen (Fig.5). A simultaneous correctionof the epigastric hernia was undertaken.

Discussion

More than 90% of primary varicella infectionsoccur in the first fifteen years of life.7 Only about 7.6per 100,000 require hospitalization. About half of ho-spitalizations are caused by superinfection causing cel-lulitis, abscess formation or necrotizing fasciitis. It isestimated that less than 1% of admissions for varicellaare attributable to NF. Other less common complica-tions include neurological sequelae (8%) or pulmo-nary complications, including acute respiratorydistress syndrome or pneumonitis (3.1%).8

While group A Streptococcus is the most com-monly isolated organism in NF,7 varicella infection hasbeen reported to be the most common initiating factorin children diagnosed with NF.8 Some reports suggesta mortality rate as high as 60% (>80% in the neonatalperiod). This is attributed to disseminated intravascu-lar coagulation and widespread thrombosis that pre-vents antibiotic penetration into infected tissue.9

Immunosuppression, hypothermia, hypotensionand shock have been shown to be statistically signi-ficant predictors of mortality in children with NF.10

In addition, more than 90% of varicella-induced

Fig. 4 - (A) Preoperative CT scan 13 years later, showing evidenceof constriction at the level of lower abdomen causing displacementof intraabdominal organs into the thorax, and (B) Post-operative CTscan after excision of skin grafts and ultimate reconstruction, showingresolution of constriction

Fig. 3 - Patient upon presentation 13 years laterFig. 5 - One month after second round tissue expansion and flap ad-vancement

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necrotizing fasciitis occurs in children with no im-munodeficiency, and the diagnosis is usually dela-yed due to nonspecific clinical signs and symptoms.

Nevertheless, the classic treatment involves ur-gent and aggressive debridement of all necrotic tis-sues as early as possible.11 Subsequent surgeries maybe required to ensure that the wound is clean beforewound coverage is planned.

Wound coverage post necrotizing fasciitis is chal-lenging, especially in the pediatric age group, with localor regional flap transfers typically reserved for relati-vely small wounds. Generally, in the pediatric popula-tion with extensive soft tissue defects, skin grafting isthe most suitable option.12

It is estimated that 78-91% of children who surviveNF have significant long-term sequelae, including scar-ring, functional joint limitations and amputations.12

Although our patient presented with an aestheticcomplaint, he was actually suffering from a fun-ctional limitation, which was overshadowed by anaesthetic deformity that was causing him severeembarrassment. Excision of the tight scar pannusof the abdominal wall with advancement of expan-ded skin and subcutaneous tissue flaps has not onlyresulted in aesthetic improvement but also in therelease of the severe abdominal wall constrictionwith subjective clinical improvement in pulmonaryfunction on exertion. Combining tissue expansionwith reverse expansion by liposuction is an origi-nal technique that has allowed reconstruction of anextensive flank and abdominal wall defect withonly 2 operative settings. The patient ultimately re-ports excellent outcome, aesthetically and functio-nally.

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