Hemorrhagic shock and encephalopathy syndrome in a quadriplegic child: an argument for the triggering role of impaired thermoregulatory response.
METHODS
A case presentation of hemorrhagic shock and encephalopathy syndrome (HSES).


OBJECTIVE
To describe an unusual complication of uncontrolled fever in a tetraplegic child and to discuss possible pathophysiological mechanisms in these circumstances.


BACKGROUND
HSES is a rare and dramatic disorder of unknown origin occurring mainly in infants and young children. Clinical features of HSES associate hyperpyrexia, acute diarrhea, circulatory collapse, coma, convulsions, and multiple organ failure (MOF). Altered physiologic thermoregulatory response in infants exposed to abruptly increased core temperature or altered thermal environment, and links with heat stroke, have been mentioned in previous publications.


METHODS
We report a case of HSES occurring in a 6-year-old girl with post-traumatic C4 quadriplegia. She eventually experienced hyperpyrexia, deep shock, watery diarrhea, and severe MOF developed rapidly. Despite rapidly resolving MOF, severe brain lesions consistent with HSES were observed and resulted in permanent neurologic impairment.


RESULTS
Negative bacterial and viral screening eliminated a septic origin. In this child, impaired thermoregulatory response to acute hyperpyrexia resulting from complete quadriplegia could be the necessary condition for the development of HSES in the presence of acute hyperpyrexia of unknown origin.


CONCLUSIONS
Quadriplegic patients, especially young children, could be considered at increased risk of developing severe MOF and acute central nervous system impairment consistent with HSES, when exposed to heat stress and should be treated promptly.
Baugnon
Thomas
T
Department of Anaesthesia, Pediatric Surgical Intensive Care Unit, Hopital des Enfants Malades-Assistance Publique Hopitaux de Paris, Université Descartes Paris 5, Paris, France.
Duracher-Gout
Caroline
C
Blanot
Stéphane
S
Vecchione
Antonio
A
Guillou
Florence
F
Carli
Pierre A
PA
Meyer
Philippe G
PG
D001833:Body Temperature Regulation / Q000502:physiology; D001927:Brain Diseases / Q000150:complications* / Q000503:physiopathology; D002648:Child; D005260:Female; D006801:Humans; D018458:Persistent Vegetative State / Q000209:etiology / Q000503:physiopathology; D011782:Quadriplegia / Q000209:etiology* / Q000503:physiopathology; D012771:Shock, Hemorrhagic / Q000150:complications* / Q000503:physiopathology; D013577:Syndrome