Paragangliomas are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia, small organs consisting mainly of neuroendocrine cells that are derived from the embryonic neural crest and have the ability to secrete catecholamines (1). They are closely related to pheochromocytomas, which are sometimes referred to as intra-adrenal paragangliomas (2). The majority appear to be sporadic, but approximately one-third to one-half are associated with an inherited syndrome (3, 4). The four genetic syndromes that are associated are: multiple endocrine neoplasia 2A and 2B, neurofibromatosis type 1, von Hippel Lindau, and the Carney-Stratakis dyad. The male-to-female ratio is approximately equal among patients with hereditary paraganglioma, …show more content…
The majority of sympathetic paragangliomas arise outside of the skull base and neck anywhere along the sympathetic chain (8). About 75 percent arise in the abdomen, most often at the junction of the inferior vena cava and left renal vein and about 10 percent arise in the thorax (9, 10). They excrete excess amounts of catecholamine (86 percent in one series) (7), usually almost always …show more content…
Patients can become hypertensive during induction, positioning, or tumor resection (12). Chronic catecholamine excess causes volume contraction and patients can become severely hypotensive, as in this case, if adequate volume resuscitation is not performed (12). It is prudent to evaluate for adverse events following hypertensive and hypotensive episodes. Serial neurological evaluations, CT brain, electrocardiograms, or serial cardiac enzymes may be warranted. Complications of surgery are primarily due to severe preoperative hypertension, high secretion tumors, or repeat intervention for recurrence (13). In one study, adverse perioperative events occurred in 32 percent of cases (14). The most common adverse event was sustained hypertension in 25 percent of the patients. There were no perioperative deaths, myocardial infarctions, or cerebrovascular events. Despite premedication of most patients with phenoxybenzamine and a beta-blocker, varying degrees of intraoperative hemodynamic lability occurred
The patient wakes up after few minutes of the procedure with some dizziness (anesthetic effects) and becomes alert after few hours and can resume normal activities.
The preanesthetic drug consisted of 0.1 to 0.2 mg / kg of midazolam administered orally 35 minutes before surgery. Anesthetic induction was performed with 0.2 to 0.3 mg / kg of etomidate, 3 to 5 μg / kg of fentanyl, 0.05 mg / kg midazolam and 0.1 mg / kg intravenous pancuronium. Maintenance was performed with isoflurane combined with a mixture of oxygen and compressed air, and fentanyl if necessary. During cardiopulmonary bypass, additional doses of midazolam and pancuronium were used on demand.
A week after initial admission, the patient is on the medical surgical floor recovering from his transverse colostomy five days ago. At 1200 vital signs are as follows, temperature 99.1; pulse 96; respirations 18; blood pressure 141/69; pulse ox is 94% on 1L NC in AM. The patient appears acutely ill and lays in bed with his eyes closed even when family comes into the room to check on him. He is alert and oriented to person, but not place or situation. He appears lethargic and is slow to respond to questioning, this appears to be due to recent administration of pain medication. Pupils are equal round and reactive to light and grips are week bilaterally in hands. Abdomen is firm, distended, and non-tender. Colostomy site appears to be
Malignant Hyperthermia is primarily thought to be an autosomal dominant genetic disorder that causes a hypermetabolic state after administration of volatile anesthetics. When a patient is under anesthesia, the muscles are usually relaxed, but when a patient is experiencing Malignant Hyperthermia crisis, certain IV anesthesia causes the opposite effect. Most inhaled anesthetics other than nitrous oxide, cause or trigger Malignant Hyperthermia. More specifically, the anesthetic agents: Halothane, Chloroform, and Succinylcholine. The genic condition of Malignant Hyperthermia only becomes apparent when a patient is exposed to certain anesthetics such as halothane, which causes muscle rigidity.
Pheochromocytoma is an adrenal gland tissue tumor. (PubMed) This growth is a result of excessive release of catecholamine’s (hormones). Norepinephrine and epinephrine are the primary hormones secreted while some can produce dopamine. (Medscape) These increased chemicals in the body can influence the persons “heart rate, blood pressure and metabolism”. (PubMed) If not treated these tumors affect the cardiovascular system and can lead to the development of “life threatening hypertension”. (Medscape) These tumors can be seen as a single mass or multiple tumors and can appear unilateral or bilateral in the body. (PubMed) Thirty percent of pheochromocytoma cases are found to be heredity where ten genes have been linked to the condition. Ten percent of pheochromocytomas are malignant and the most common sites of metastasis include “liver, bone and lymph nodes”. (Medscape)
Propofol is a hypnosis which is used to keep the patient sedated during the surgery this also used to keep patients sedated (Stonebridge, 2012). The most common physiological effects of Propofol are the local pain during the injection, eyes rolling back; coughing after anaesthesia, arching body spasms, muscle jerks, muscle twitching, slow heartbeat, hot flushes, increased breathing, hiccups, and periods of not breathing this is when the anaesthetist oxygenates the patient since the patient is sedated. Low blood pressure is also a side effect which shows on the monitor that the blood pressure levels have dropped once the Propofol has been injected (Stonebridge, 2012). Another common side effect is increased levels of fat in the blood, which
Perioperative hypothermia causes adverse effects in the recovery of a post-operative patient. These well known effects include delayed anaesthetic recovery, increased incidence and duration of postoperative ventilation, increased blood loss and transfusion requirements, increased cardiac events, increased surgical wound infection, pro-longed hospital length-of-stay (LOS) and higher hospital mortality.
Paraganglioma is a rare neoplasm of neuroendocrine origin which may develop at many different anatomical sites including head and neck, thorax, and abdomen. The occurrence of paraganglioma in lower spine is rare, but well established as one of the differential. The investigation of choice pre-operatively is magnetic resonance imaging4, which usually shows a very well circumscribed partially cystic mass which is iso- or hypointense to spinal cord on T1-weighted images, and hyperintense on T2-weighted images4. These MRI features are similar to other cauda equine tumors such as ependymoma2. However sometimes MRI may show serpentine congested ectatic blood vessels with “cap sign” (a low intensity signal rim) on T2-weighted images which are considered diagnostic for paragangliomas2,7.
Paraneoplastic syndromes are rare disorders triggered by a person's immune system in response to a cancerous tumor. Paraneoplastic syndromes can affect many areas of the body systems, including the nervous system, hormone (endocrine) system, skin (dermatologic) system, blood (hematologic) system, and joints (rheumatologic) system.
Both systems usually perform on the same organ and produce the same action potentials, yet their functions are diverse and they compete with one another (Appendix 3). The sympathetic nervous system prepares the body for emergency situations while the parasympathetic nervous system maintains conserving functions when the body is at rest. Parasympathetic ganglia occur individually in the tissues and organs, however sympathetic ganglia occur on either side of spinal cord as two lateral chains. Preganglionic fibers of sympathetic nervous system arise from thoracic and lumbar segments of spinal cord, whereas that of parasympathetic nervous system arises from the midbrain and sacral segments of the spinal cord. Overall effect of sympathetic nervous
Inherited pheochromocytomas may be isolated (inherited as an autosomal dominant trait) or a component of hereditary tumor syndromes – primarily multiple endocrine neoplasia (MEN) 2A and 2B and von Hippel-Lindau (VHL) disease, the familial paraganglioma syndromes and, in rare instances, MEN 1, neurofibromatosis (NF) type 1 and the tuberous sclerosis
Paraneoplastic syndrome is a set of signs that happens from cancer but is not due to the local presence of cancer cells.
A detailed history and clinical exam with a thorough neurological exam aid in the diagnosis. A CT scan of the head to visualize the tumour or a Gadolinium-enhanced MRI that has a higher sensitivity to visualize the cranial nerves could be performed to confirm diagnosis (Marzo, Zender, & Leonetti, 2009). Electrical testing of the facial nerve could assist in determining the severity and extent the tumour is impeding function (Marzo, Zender & Leonetti, 2009).
There is much deliberation concerning phenylephrine and vasopressin working on systolic blood pressure. Both constrict blood vessels, both can lead to bad complications if you give it into a blown IV and phenylephrine will cause pulmonary arterial vasoconstriction as well. Fluid resuscitation was continued while BP was supported with phenylephrine and vasopressin. Phenylephrine was changed
Take notice of figure.1 in the illustrated diagram that the sympathetic nervous system originates in the spinal cord. Specifically, the cell bodies of the first neuron are located in the thoracic and lumbar spinal cord. Axons from these neurons project to a chain of ganglia located near the spinal cord. In most cases, this neuron makes a synapse with another postganglionic neuron in the ganglion. A few preganglionic neurons go to other ganglia outside of the sympathetic chain and synapse there. The postganglionic neuron then projects to the "target" - either a muscle or a gland.