TARP syndrome
Overview
A very rare syndrome characterized mainly by an abnormal foot position (talipes), congenital heart defect small jaw, cleft soft palate and a tongue abnormality (glossoptosis)
Symptoms
The list of signs and symptoms mentioned in various sources for Pierre Robin sequence - congenital heart defect - talipes includes the 9 symptoms listed below: * Glossoptosis * Cleft soft palate * Congenital heart defect * Abnormal foot position * Auricular septal defect * Small jaw * Vascular anomalies * Low set ears * Undescended testes
Prevention
* limit duration of surgery *minimize exposure to open venous sinuses by careful surgical resection *limit the position of the irrigation bag to max. 60 cm above the surgical field in order to minimize hydrostatic pressure of the fluid *limit extent of bladder distension by frequent drainage of the bladder in order to avoid increased absorption through open venous sinuses *maintain adequate blood pressure and therefore normal periprostatic venous pressure in order to avoid increased absorption through open venous sinuses *check neuro-status (in patients with regional anesthesia), temperature, and laboratory measurements frequently
Treatment
* Treatment of TURP syndrome should start without delay. * The surgeon should be informed immediately about the possibility of a TURP syndrome and surgery should be stopped as quick as possible. * Send blood for measurements of electrolytes, blood gas analysis, hemoglobin and hematocrit, coagulation profile, glucose and ammonia level. * Switch irrigation to warm normal saline for continuous bladder irrigation in order to avoid further hypothermia and hemodilution with hypoosmolar solutions. * Continue ventilation or consider intubation (in awake, non-intubated patients) in cases will pulmonary edema and respiratory insufficiency. Administer furosemide. Anti-Trendelenburg positioning if hemodynamically tolerated. * Monitor for hyponatremia and treat only if clinical symptoms are obvious or very likely. Treatment with hypotonic saline is somewhat controversial as explained above in the case report. Monitor sodium levels frequently during replacement therapy. Avoid quick correction of hyponatremia. Administer maximal 50 to 100cc/hr of 3% saline until clinical signs of hyponatremia dissappear. Continue then with diuretic therapy and infusion of normal saline (0.9%). * Monitor for hyperkalemia due to hemolysis during hypoosmolar dilution and for hypokalemia during treatment with diuretics. Correct potassium imbalances. * Monitor for coagulopathy and treat disturbances accordingly. * Consider placement of an arterial catheter, central venous catheter/pulmonary artery catheter for better control of hemodynamics and fluid therapy.the * Treat complications such as seizures or arrhythmias while correcting their causes (hyponatremia, hyperkalemia, hypokalemia, hypoxia, hypothermia).