Syndrome de Volkmann néonatal

Abstract

Acute limb compartment syndrome or Volkmann's ischaemic contracture is an acquired ischaemia of nerve and muscle causes by raised pressure within a closed fascial space. Congenital Volkmann's ischaemic contracture (CVIC) is a rare entity. A 2-day-old girl was referred with a problem of the left forearm and arm, which exhibited cold oedema with decreased mobility. Lesions were present at birth and were rapidly complicated by skin necrosis. The mother was taking olazanpine, prazepam and valpromide throughout the entire pregnancy. Delivery was complicated by shoulder dystocia requiring obstetric procedures such as suprapubic pressure, Couder's maneuver and episiotomy. On physical examination her left hemi-thorax, left arm, forearm and hand exhibited marked oedema. A large and well-demarcated bullous, fibrous and ulcerated area of skin necrosis was observed on the elbow fold and on the inner anterior part of the arm. Digital flexion with cyanosis was present. MR angiography revealed extensive oedema of the soft tissue and muscle with fascial effusion, associated with compression of the arm arteries and reduced blood flow in the forearm. A fasciotomy was performed at Day 3 of life. The postsurgical arterial MRI was normal. At Day 10 of life, the patient developed opisthotonos involving spasms and tremors associated with numerous intercritical abnormalities evoking benzodiazepine weaning syndrome. The child's neurological status was stabilized by treatment with phenobarbital and clonazepam. She was subsequently lost to follow-up. CVIC has been ascribed to multiples causes. Mechanical compression is the main recognized factor: amniotic band constriction, umbilical cord loops, compression in utero by a deceased co-twin, malposition of the hand, arm or forearm, local or general factors that can add to extraction problems: brachypelvic disproportion, extraction with forceps, oligo/polyhydramnios, pre-term delivery, pre-eclampsia, caesarean section, premature labour, excessive maternal weight gain or diabetes. Our case emphasized three main points. First, the diagnostic value of early MR angiography in the event of associated extensive tissue oedema, multiple arterial compression and decreased vascular perfusion. Second, the role of shoulder dystocia in triggering the traumatic factor reported for the first time. Third, the role of neuroleptic and anxiolytic treatments taken by the mother during pregnancy. Prazepam is a long-acting benzodiazepine that can cause impregnation and withdraw syndromes in neonates. Impregnation "floppy infant syndrome" is an early event characterized by hypotonia, hypoventilation and lethargy. Hypotonia and decreased foetal movements may favour prolonged pressures and malposition with secondary crush injury during delivery. Maternal medication has not been cited hitherto as an aetiological factor in neonatal compartment syndrome.