Congenital FVII deficiency or hypoconvertinemia is a rare and heterogeneous disorder with prevalence of around one individual per 500 000 [1]. It is an autosomal recessive disease. This deficiency is asymptomatic in 50% of homozygous and in most heterozygous patients. Severe bleeding manifestations tend to occur mainly in homozygous or compound heterozygous individual. FVII gene is located in chromosome 13. To date, molecular analysis allowed the characterization of more than 200 different mutations. However, the correlation between residual FVII coagulation activity and bleeding tendency or severity of manifestations seems to be poor [2]. Therefore, the clinical presentation is quiet variable and independent of FVII plasma levels [1,3]. A FVII deficiency is suspected when clotting screening tests reveal isolated prolonged prothrombin time with normal APTT value and confirmed using specific FVII:C assays [2].Spontaneous cord haematoma (SCH) is uncommon, but a severe life-threatening gestational accident. It represents a rare cause of acute foetal distress that may be shown by the decrease of foetal movement or foetal death [4]. Many risk factors have been reported: short cord, twisting and traction of the cord, post-maturity, infection, morphologic abnormality and alterations of the vascular wall. Meanwhile, rupture of the umbilical vein has been reported as the main cause of cord haematoma by many authors [4].We describe the case of a patient born from a normal pregnancy who presented with an SCH discovered at delivery, secondary to composite heterozygous congenital factor VII deficiency. To our knowledge, this is the first case ever reported in the literature.A female infant was born full-term from non-consanguineous parents after a caesarean section because of mother's complaint of decreased foetal movement and foetal heart decelerations. At birth, the baby was 'flat' (Apgar score of 0 at 1 min, 3 at 5 min and 7 at 10 min), resuscitation with naso-tracheal intubation and immediate mechanical ventilation in the delivery room resulted in rapid improvement and haemodynamic stabilization. The amniotic fluid was meconiumstained. On admission to our intensive care unit, infant hypertonia with some 'rolling-up' movements of upper limbs was noted and therefore treatment with Phenobarbital was commenced. Otherwise, the clinical examination revealed a massive umbilical cord haematoma at the skin junction, which was compressing the cord.Postnatal evolution showed a neurological degradation with coma, no reactivity, total hypotonia and no archaic reflex. Electroencephalogram obtained during the second day of life revealed a severe alteration in brain electrogenesis compatible with hypoxic-ischemic encephalopathy. Cerebral ultrasonography showed a diffuse cerebral oedema. The third day, magnetic resonance imaging (MRI) confirmed cortical and basal ganglia ischemic lesions.The newborn remained dependant on mechanical ventilation and died of multi organ failure on day 6 of life.Biological assessment showed a disseminated int...
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