First aid
Pre-hospital care for lung barotrauma includes basic life support of maintaining adequate oxygenation and perfusion, assessment of airway, breathing and circulation, neurological assessment, and managing any immediate life-threatening conditions. High-flow oxygen up to 100% is considered appropriate for diving accidents. Large-bore venous access with isotonic fluid infusion is recommended to maintain blood pressure and pulse. Emergency treatment
Pulmonary barotrauma:
Endotracheal intubation may be required if the airway is unstable or hypoxia persists when breathing 100% oxygen. Needle decompression or tube thoracostomy may be necessary to drain a pneumothorax or haemothorax
Foley catheterization may be necessary for spinal cord AGE if the person is unable to urinate. Intravenous hydration may be required to maintain adequate blood pressure. Therapeutic recompression is indicated for severe AGE. The diving medical practitioner will need to know the vital signs and relevant symptoms, along with the recent pressure exposure and breathing gas history of the patient. Air transport should be below 1,000 feet (300 m) if possible, or in a pressurized aircraft which should be pressurised to as low an altitude as reasonably possible.Sinus squeeze and middle ear squeeze are generally treated with decongestants to reduce the pressure differential, with anti-inflammatory medications to treat the pain. For severe pain, narcotic analgesics may be appropriate.Suit, helmet and mask squeeze are treated as trauma according to symptoms and severity. Medication
The primary medications for lung barotrauma are hyperbaric and normobaric oxygen, hyperbaric heliox or nitrox, isotonic fluids, anti-inflammatory medications, decongestants, and analgesics.
Removal or enucleation of the eye is a last resort option if the eye already has become blind and painful.Repeated blood transfusions may be needed to control anemia, and thrombocytopenia can be managed with splenectomy. Prognosis
The neurological symptoms are progressive and can lead to severe spasticity, bulbar symptoms and dysarthria within one to two decades. The life span is shorter than normal. Death occurs between 2 and 30 years of age, depending on the severity of the syndrome. The immediate cause of death is pneumonia, fulminant intestinal bleeding or multiple organ dysfunction.If not treated, the retinal detachment can lead to ischemia and growth of new blood vessels over the iris and anterior chamber angle. This in turn can cause secondary glaucoma, cataract and, ultimately, blindness of the eye. Epidemiology
This syndrome is usually sporadic although families with two or more affected siblings of both sexes are known. History
A child who was eventually diagnosed with cerebroretinal microangiopathy with calcifications and cysts was first described in the literature in 1987. The disorder was suspected of being allelic with either the Revesz syndrome or leukoencephalopathy with calcifications and cysts.