Acute forearm compartment syndrome in a newborn caused by reperfusion after spontaneous axillary artery thrombosis

Abstract

Acute compartment syndrome of the forearm in newborns is often misdiagnosed and can be disastrous if left untreated. Here, we report a full-term infant of a diabetic mother with underlying heterozygosity for MTHFR C677T and A1298C alleles. A spontaneous thrombosis occurred in the left axillary artery immediately after birth. The patient responded well to anticoagulant (heparin) and thrombolytic (tissue plasminogen activator) agents. After reperfusion of the extremity, acute compartment syndrome developed. Emergent fasciotomy was performed. In this case, effective collaboration between pediatricians and orthopedic surgeons resulted in salvage of the extremity, with good clinical and functional results.

Clinical Case of Acute Non-Traumatic Hand Compartment Syndrome and Systematic Review for the Upper Extremity

“…This yielded 16 reports of 19 cases of spontaneous nontraumatic ACS of the upper extremity published from 1993 to 2016. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] Fifteen of the publications were isolated case reports. Of the 19 instances of nontraumatic ACS, 6 were attributed to infection, 3 were due to a bleeding disorder, 3 were reported as unknown or idiopathic, 3 were secondary to anticoagulation medication, and 4 were due to systemic sclerosis, Ehlers-Danlos syndrome, coma blister in nontraumatic rhabdomyolysis, and McArdle disease.…”

“…7 The majority of reported cases of spontaneous nontraumatic ACS of the upper extremity involved the forearm (17 forearm and 2 hand). [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] All but one of these were treated surgically with fasciotomies. The ACS that was managed nonoperatively involved a spontaneous extensor carpi ulnaris compartment due to an unknown cause.…”

Neonatal Extremity Compartment Syndrome: A Rare Diagnosis Requiring Prompt Recognition

“…The first mechanism is driven by a hypercoagulable state that causes thromboembolic events, leading to tissue ischemia, reperfusion injury, and swelling. 9,10 The second mechanism is initiated by trauma to the limb that causes skin changes and progressively worsening swelling. Ultimately, the common endpoint of these mechanisms involves severe swelling that impedes blood supply and causes further skin and tissue necrosis of the affected limb.…”

Complete Decompression of a Forearm Neonatal Compartment Syndrome Using a Volar Approach: A Surgical Technique

“…Most frequently encountered complications after surgical decompression of NCS include flexion contracture, decreased spontaneous movement, weakened grip strength, and limb length discrepancies (radius and ulna, in particular). 3,7,10,17,30 In severe situations, the autoamputation of digits can occur despite all attempts at emergent decompression. 17 In other cases, complete recovery of the hand and wrist has been reported.…”

“…Its pathophysiology is similar to that of compartment syndrome seen in adults, originating from increased pressure within a closed musculofascial space, and leading to decreased distal capillary perfusion 4. Contrary to adult patients who typically have a history of trauma (fracture or crush injury), the underlying etiology of compartment syndrome in neonates is often unknown, iatrogenic (vessel damage during catheterization), or precipitated by maternal diabetes, fetal macrosomia, oligohydramnios, multigestation, compound presentation, and hypercoagulable states 1–3,5–11…”