Compartment syndrome in all four extremities: a rare case associated with systemic capillary leak syndrome.

[systemic capillary leak syndrome]

While isolated limb injuries often lead to suspicion for and treatment of compartment syndrome , multi-limb compartment syndrome induced by a systemic disease is a rare entity , in which lack of immediate diagnosis can have catastrophic consequences to the patient . We present a patient with idiopathic systemic capillary leak syndrome (SCLS ) resulting in compartment syndrome in all four extremities . Treatment required bilateral hand , forearm , calf , and thigh fasciotomies . Presenting symptoms included pain in all four extremities , malaise , and confusion . Laboratory data included polycythemia , hypoalbuminemia , leukocytosis , and elevated creatinine , but not a monoclonal spike on serum electrophoresis as is common in SCLS . Thus , when faced with clinical evidence of multiple extremity compartment syndrome , the surgeon should consider systemic disorders , such as SCLS , and aggressively treat the compartment syndrome as well as the underlying disease .

Diseases
Validation

Diseases presenting "pain" symptom

achondroplasia

acute rheumatic fever

adrenal incidentaloma

adrenomyeloneuropathy

aniridia

aromatase deficiency

carcinoma of the gallbladder

cholangiocarcinoma

coats disease

congenital diaphragmatic hernia

congenital toxoplasmosis

cushing syndrome

cutaneous mastocytosis

cystinuria

dedifferentiated liposarcoma

dentin dysplasia

dracunculiasis

dystrophic epidermolysis bullosa

epidermolysis bullosa simplex

erdheim-chester disease

erythropoietic protoporphyria

esophageal adenocarcinoma

esophageal carcinoma

esophageal squamous cell carcinoma

fabry disease

familial mediterranean fever

focal myositis

hirschsprung disease

hodgkin lymphoma, classical

holt-oram syndrome

junctional epidermolysis bullosa

kabuki syndrome

kindler syndrome

lamellar ichthyosis

liposarcoma

locked-in syndrome

lymphangioleiomyomatosis

malignant atrophic papulosis

neuralgic amyotrophy

oligodontia

oral submucous fibrosis

papillon-lefèvre syndrome

phenylketonuria

pleomorphic liposarcoma

primary hyperoxaluria type 1

proteus syndrome

pyomyositis

scrub typhus

sneddon syndrome

systemic capillary leak syndrome

thoracic outlet syndrome

trochlear dysplasia

typhoid

von hippel-lindau disease

waldenström macroglobulinemia

well-differentiated liposarcoma

wolf-hirschhorn syndrome

This symptom has already been validated