We present the case of a newborn, born at 38 weeks and 5 days, of female sex, from non-consanguineous parents. The pregnancy took place without any particularity except for a gestational diabetes balanced under diet. Antenatal ultrasound screenings and NIPT genetic screening did not reveal any anomalie. A cesarean section was performed for fetal bradycardia due to the presence of a circular cord easily reduced at birth. The child presented an Apgar score of 9-10-10 with good immediate adaptation to extrauterine life. Shortly after delivery, cyanosis and edema of the right forearm with bullous lesions were objectified. A bone X-ray did not reveal any fracture of the limb, but a Doppler ultrasound of the upper limb showed a total absence of right radial and ulnar arterial flow.
Following this discovery, the newborn was transferred to our institution for optimal care.
On her arrival after 16 hours of life, the clinical examination revealed cyanosis, edema, coldness and functional impotence associated with an erosive lesion post-bullosa of the right forearm. An absence of capillary pulse was also noted. An emergency angio-scan revealed an absence of enhancement of the arterial and venous vessels from the elbow without associated thrombus. This radiological assessment is compatible with compartment syndrome. Different blood tests have excluded sepsis as well as coagulopathy. The diagnosis of neonatal Volkmann syndrome was therefore made.
Fasciotomies of the right forearm and right hand associated with ulnar and radial arteriolysis were performed in an emergency. Unfortunately, given the extent of extensive tissue necrosis, amputation of the forearm was necessary on the 9th day of life.
Neonatal Volkmann syndrome or neonatal compartment syndrome is exceptional with a few dozen cases documented to date. It corresponds to neuromuscular ischemia and skin lesions due to an increased pressure in a well-defined anatomical compartment.
The exact incidence of this syndrome is still unknown as is the pathophysiology.
However, many theories have been proposed.
These causes are external compression (constrictive amniotic band, constriction of the upper limb by the umbilical cord, compression by a twin, malposition of the arm, fracture, oligoamnios), factors causing delivery difficulties and intrauterine suffering (diabetes mellitus), pregnancy, premature rupture of the amniotic sac, pre-eclampsia, premature birth, prenatal respiratory distress, uterine abnormality, excessive maternal weight gain, difficult extraction, instrumental delivery, occipito-transverse presentation), an iatrogenic cause (arterial catheterization, consumption of psychotropic drugs during pregnancy), an intrinsic cause (hypercoagulable state), a thrombotic or even septic cause.
Regarding our patient, gestational diabetes, fetal respiratory distress and circular umbilical cord are positive factors for the occurrence of this syndrome.
Neonatal Volkmann syndrome mainly affects the upper limb and is characterized by trophic disorders and a neuro-motor deficit.
Skin manifestations are present at birth and can appear in various forms such as oozing, edema, erosions, ulcers, blisters, bluish or necrotic plaques, often with the persistence of pulses.
These skin lesions can be easily confused with other medical entities; the dermatologist plays a key role in the diagnosis.
The main differential diagnoses are ischemic neonatal gangrene of the extremities, aplasia cutis congenita, necrotizing fasciitis, congenital varicella, fatty subcutaneous necrosis, brachial plexus injury, fracture of the clavicle or humerus, an iatrogenic cause or an epidermolysis bullosa.
Regarding the management of the Volkmann’s syndrome, there is no consensus but an urgent fasciotomy seems to be the treatment of choice to reduce intracompartmental pressure and promote reperfusion.
Unfortunately, a delay in diagnosis often prevents the completion of an aponeurotomy within an acceptable timeframe, thus leading to major tissue ischemia.
In the event of a delayed diagnosis, adverse sequelae results may be obtained such as muscle atrophy with a retractable position, nerve damage, abnormal bone development. Long-term clinical and radiological follow-up is therefore important.
Dermatologists therefore play an important role in identifying neonatal Volkmann syndrome and excluding other causes. Early recognition of this entity as well as a fasciotomy in emergencies are indeed the key to a better prognosis.
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Marie-Sophie André1, Déborah Salik1, Diane Franck2, Chantal Dangoisse1
1 Department of Pediatric Dermatology, Queen Fabiola Children’s University Hospital, Brussels, Belgium
2 Department of Pediatric Plastic Surgery, Queen Fabiola Children’s University Hospital, Brussels, Belgium