An Unusual and Fatal Case of Perthes’ Syndrome
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The Perthes syndrome is traumatic asphyxia secondary to a crushing to the thoracoabdominal region. It is characterized by a clinical trial of the face and neck cyanosis with oedema, sub-conjunctival haemorrhage and petechial haemorrhages on the face, neck and upper chest. It is traumatic asphyxia also known as Perthes Syndrome. Usually occurs during a work accident, chest compressions during mass movement but in children, road accidents are more common.

A 3-year-old boy, victims of multiple trauma by compressing a tractor against the wall for a few minutes with the initial loss of consciousness reported 5 hours after the trauma with a Glasgow score at 13, blood pressure 100/70 mmHg, tachycardia (210 beats/min), polypnea (43 c/min) and pulsed saturation at 90%. Patient was admitted to the intensive care unit of pediatric emergencies.

The clinical examination was marked by a right temporal wound of 4 cm, petechiae of the face and neck, bilateral subconjunctival haemorrhage and epistaxis. He had a low abundance of hematuria; the left lower limb in abduction and external rotation. Body scan: cerebral level was normal. There was a low abundance of pneumothorax and hemothorax. Multiple lacerations of the left kidney (middle and polar superior) with extravasation of the contrast product; and fracture of the left femur was observed.

Suture of the wound of the scalp and orthopaedic management of the left femur were performed. The immediate evolution at 6 hours after his admission to the emergency intensive care was marked by the sudden onset of a fever at 43?C, restlessness, partial convulsive seizures, altered state of consciousness (Glasgow score 8/15), 23% desaturation and worsening of the hemodynamic state (PA 60/20 mmHg). He was intubated, ventilated, sedated and put on vasoactive drugs. The onset of precarious stability (tight myosis bilaterally with slight hemodynamic improvement).

Biological and morphological exploration carried out urgently objectifying: haemorrhage (haemoglobin from 9 g/dl to 6 g/dl), low platelet rate, and alteration of the hemostasis assessment with a low prothrombin rate. Administration of blood products (1 red blood cell consent, 2 platelet consent and 2 fresh frozen plasma) was done with an improvement at biological control. In Cerebral control CT scan no abnormality has been detected, and lesions were stable at the abdominal ultrasound. Chest X-ray showed (low abundance of bilateral pneumothorax, atelectasis, and condensation).

In the first 24 hours of hospitalization, he presented an abolition of brainstem reflexes (probable brain death) not explored by medical imaging. In conclusion, the evolution was marked by the complications of severe and acute rhabdomyolysis leading to multi-visceral failure: neurological, hemodynamic, respiratory, hematologic, renal, hepatic. Then at 36 hours of his admission, the patient died by cardiorespiratory arrest not recovered by the resuscitation measures.

Perthes syndrome is not entirely benign. The vital prognosis depends on the one hand on the duration of the compression mechanism, on the precocity of adequate treatment and on the other hand, on the severity of the associated lesions. The tolerance of traumatic asphyxia must be analyzed in a particular way in children.

Source: https://www.scirp.org/journal/paperinformation.aspx?paperid=102800
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