[Congenital gangrene: a rare condition during the neonatal period: a case study]. / Gangrène congénitale entité rare en néonatalogie: à propos d'une observation.

Neonatal gangrene is a rare condition during the neonatal period. Its prognosis is generally poor. Acute ischemia of the limbs is most often caused by thromboembolic events. Although there are several predisposing factors, in the majority of cases no causative factor is found. Also the extent of the gangrene is variable; it can affect one or several fingers or toes or even the whole upper or lower limb. We here report a case of congenital gangrene of the hand treated in our Department.

Congenital gangrene of the extremities in a newborn.

Gangrene of the extremities in the newborn is extremely rare at birth. Less than 100 cases have been reported worldwide. Its etiology is obscure in many cases; however, some factors have been associated with it in the newborn, which include vascular injury and embolism. We report a case of a baby with congenital bilateral lower limb gangrene caused by thromboembolic phenomenon from retroplacental hematoma following abruptio placentae and highlight the challenges of managing such condition in resource-poor setting.

Intrauterine upper limb ischemia associated with fetal thrombophilia: a case report and review of the literature.

Neonatal extremity gangrene is rare, even rarer are those born with evidence of intrauterine vascular occlusion. Intrauterine limb ischemia has been attributed to several etiological factors which include thromboembolic disease occluding the arteries of the affected limb or compression of the limb during intrauterine life. In this report, we present a case of brachioradial arterial thrombosis associated with mild homocysteinemia and double heterozygosity of methylenetetrahydrofolate reductase 677C-T and factor V Leiden gene mutations. We suggest investigating the neonates and their mothers for possible genetic prothrombotic risk factors when they present with intrauterine thrombosis as this issue is important for management and counseling.

Intrauterine gangrene of the lower extremity in the newborn: a report of two cases.

We describe 2 cases of intrauterine gangrene involving the lower extremity in 2 unrelated neonates. Both cases were complicated by prematurity, and 1 case was complicated by an intrauterine distal femur fracture and twin-twin transfusion syndrome. Both cases resulted in profound ischemic necrosis from the knee to the foot, requiring knee disarticulation. In both cases, a follow-up period of 7 years is now completed, and no further medical or surgical complications have arisen.

Extremity gangrene in utero.

A neonate with extremity gangrene resulting from intrauterine embolization of infarcted placental substances is discussed. This rare clinical entity is thought to be most commonly a manifestation of embolic phenomenon during maturation of the neonatal circulatory system. Management of neonatal gangrene is conservative, delaying amputation as long as possible since the line of demarcation tends to migrate distally. Evidence of multiple emboli should be carefully sought prior to definitive treatment.

Peripheral ischaemia and gangrene presenting at birth.

The aetiology of non-iatrogenic causes of peripheral ischaemia and gangrene presenting either at birth or within a few hours of delivery is unknown in the majority of 56 confirmed cases. In this review of 47 cases occurring since 1941 the aetiology was clear in only 6, four due to compression by the encircling umbilical cord. There was no clear association with gestational age, birth weight, maternal age or type of delivery. Seven were infants of poorly controlled diabetic mothers and these may constitute a subgroup due to altered haemostatic mechanisms. Pregnancy hypertension was an association in 7 cases, oligohydramnios in 6. There is only limited support for birth trauma, sepsis, and thrombo-emboli from the ductus arteriosus as causes. There is indirect evidence that thrombo-emboli can migrate from the placental bed to the fetus. In recent years death from this condition has been rare with surgical thrombectomy increasingly successful in late presenting cases. When gangrene is established at birth surgical amputation, autoamputation, or some loss of function is usual. Peripheral ischaemic insults presenting at birth may be part of a wider spectrum of disorders, both prenatal and perinatal, attributable to occlusive vascular disruption.

In utero thrombosis and neonatal gangrene in an infant of a diabetic mother.

We report an infant of a diabetic mother (IDM) with in utero brachial artery thrombosis and neonatal gangrene to illustrate that there may be an increased risk for arterial as well as venous thrombosis in IDMs. The diagnosis of brachial artery thrombosis was made by using Doppler sonography flow studies and was confirmed with autopsy. The postnatal period was complicated by aortic and mesenteric artery thrombosis, with subsequent necrotizing enterocolitis, renal infarction, and death. Gangrene of a limb presenting at birth is rare, with 32 individuals reported in the literature, including this patient. Twenty-two percent (7/32) of the infants with peripartum limb gangrene were IDMs. This implies a marked increase in arterial thrombosis in IDMs over the general population. Changes in coagulation factors have been reported in newborn IDMs with poor control of maternal diabetes. Increased clotting and decreased fibrinolysis found in diabetics may lead to arterial thrombosis in IDMs in utero and postnatally. Use of anticoagulants in at-risk infants should be considered to prevent further thrombosis postnatally. Additionally, IDMs may be at increased risk for thrombotic complications from umbilical artery catheter. In utero thrombosis of the brachial artery may be one mechanism which leads to limb reduction defects in IDMs.

Infarction of the upper limbs associated with oligohydramnios and intrauterine compression.

We describe a case of neonatal infarction involving both upper extremities. Oligohydramnios, resulting in compression of both arms within the uterus, is proposed as the cause of this congenital gangrene. Despite attempted conservative management, both arms became flaccid and moist. By the third day of life, the infant's worsening cardiovascular status necessitated amputation. This rare disorder has varying degrees of presentation. Limbs have been salvaged with conservative management of the extremity, coupled with appropriate surgical intervention, and neonatal intensive care.