Neonatal Extremity Compartment Syndrome in a Pre-term Neonate

Introduction: Neonatal compartment syndrome (NCS) is a rare diagnosis, with less than 100 cases reported in the literature. Almost all neonatal cases involve the upper extremities and present with swelling and skin changes, including desquamation, blister or bullae formation, and skin necrosis if not promptly treated. Risk factors for NCS include the hypercoagulability (maternal diabetes, sepsis or inherited disorders) or trauma, though the etiology remains poorly understood. Despite prompt treatment, most neonates with NCS have some long term disability.

Case Description: Baby boy was born at 28 weeks to a 29 year old mother via cesarean delivery for fetal distress and malpresentation in the setting of preterm premature rupture of membranes. A right humerus fracture was noted at delivery. He was intubated and a UVC was placed before the patient was transferred emergently transferred to a quaternary care hospital for evaluation by vascular and orthopedic surgery. At 12 hours of life, the right arm, forearm, and hand were swollen and ischemic appearing with faintly dopplerable pulses. Due to concern for NCS, fasciotomies of the volar forearm, dorsal forearm, volar hand, and dorsal hand were performed. Multiple areas of muscle were noted to be pale due to hypoperfusion but became pink/red upon fascial release, and no necrotic muscle was discovered. Skin perfusion and swelling improved rapidly after fasciotomy. He was treated prophylactically with vancomycin and opioids. He underwent fasciotomy closure on day of life 2. One month post operatively, he was fitted for a right hand splint present to promote neutral wrist positioning and to prevent wrist flexion contracture. He required a prolonged NICU stay due to lung disease and was discharged at age 7 months. He requires ongoing OT, PT, and orthopedic surgery follow-up; he is currently is able to use his right arm and does not have movement limiting contractures.

Discussion: Due to his prematurity and instability, surgery was performed at the bedside in the NICU.
There was a multidisciplinary approach with pediatric orthopedic surgery as well as the adult vascular surgery team. Given family religious preferences to avoid blood products, the team also took steps to increase hematopoeisis including iron and epoetin in the post-operative period.

Conclusion: Early recognition of neonatal compartment syndrome and fasciotomy is critical to prevention of long-term complications of compartment syndrome. In pre-term infants requiring respiratory interventions and unstable for transport to the operating room, prompt treatment with fasciotomy may be delayed for stabilization but remains emergent.