From skin to syndrome: The progression of primary cutaneous aspergillosis to extremity compartment syndrome in a premature infant. Case report and review of the literature

Abstract

Introduction and importance: Fungal infections in neonates, particularly primary cutaneous aspergillosis (PCA), pose significant risks to premature infants with very low birth weight. This report chronicles the case of a 22-week gestational age female, with a birth weight of 430 g, who developed extensive cutaneous aspergillosis leading to extremity compartment syndrome- a first in the medical and surgical literature.

Case presentation: A female micro preemie born at 22 weeks and 2 days was critically ill during her first week of life. On first day of life, attempts were made to establish intravenous access in all extremities. Eventually, the neonatal team established a peripherally inserted central catheter (PICC) in the left upper extremity. She had surfactant therapy due to lung immaturity and was on high frequency oscillatory ventilation. A large patent ductus arteriosus was treated soon after birth with intravenous acetaminophen. On day seven of life, she developed skin blisters rapidly progressing to necrotic eschars, particularly on the right lower extremity, leading to compromised limb circulation. Consultations with pediatric surgery and orthopedics one week later resulted in bedside medial and lateral escharotomies to salvage the limb. Tissue biopsy confirmed Aspergillus species, prompting a dual antifungal treatment with liposomal Amphotericin B and Voriconazole. The lesions gradually resolved with diligent wound care and aggressive physical therapy, yet a residual contracture of the right foot's dorsal area persisted, necessitating splinting.

Clinical discussion: Over the course of treatment, her cutaneous lesions resolved, and no further debridement was required. Aggressive physical therapy was initiated for residual contractures that the infant developed due to full thickness necrosis of the cutaneous infection. Due to concurrent pulmonary complications and tracheomalacia, surgical intervention for the contracture has been postponed.

Conclusion: This case highlights the critical importance of early detection and treatment of PCA in preterm infants, the challenges in managing complex cases in the NICU and the need for a multidisciplinary approach to care.

Keywords: Case report; Escharotomy; Extremity compartment syndrome; Premature infants; Primary cutaneous aspergillosis.

Fig. 1

Primary cutaneous aspergillosis and constrictive eschar.

Fig. 2

a and b. Post operative appearance of escharotomies. a. Lateral escharotomy. b. Medial escharotomy.

Fig. 3

a and b. Resolution of cutaneous infection with residual contracture. a. Lateral escharotomy wound on pod #35. b. Contracture of right foot dorsum 15 weeks post treatment.