Introduction:
Cushing syndrome is a hyper-cortisol state in which signs and symptoms are produced due to the excessive and prolonged secretion of corticosteroids. The basic cause of Cushing syndrome is based on the ACTH (Adrenocorticotropic hormone is a polypeptide hormone synthesized and secreted by the anterior pituitary gland).
ACTH hormone-dependent is the most common cause, mainly seen in females.
The causes are:
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Pituitary microadenoma or macroadenoma.
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The nonendocrine tumor produces ectopic ACTH.
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ACTH therapy.
The ACTH-independent cause is based on the adrenal gland.
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Adrenal adenoma.
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Adrenal carcinoma.
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Glucocorticoid therapy.
How Should One Decide That a Surgical Procedure Is Needed to Treat Cushing Syndrome?
Based on the root cause of Cushing syndrome, surgical intervention is undertaken.
The transsphenoidal surgical approach is considered the treatment of choice in most patients with Cushing's disease. The neurosurgeons investigate and build the diagnosis based on the clinical features.
Further investigation is carried out using a dexamethasone suppression test to assess the cortical level after the medication. The root cause is determined by scanning the pathology with computerized tomography (CT) and magnetic resonance (MR) imaging. Introducing the operative microscope and fluoroscopy imaging during surgery permits detailed identification of normal and pathological (abnormal) tissue within the sella turcica (part of the cranial fossa where the pituitary gland is located).
What Are the Surgical Options for Cushing Syndrome?
There are two types of operations available for pituitary adenomas. The most frequently used route suitable for most patients with Cushing's disease is the transsphenoidal (transnasal) route.
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Transsphenoidal Surgery:
Neurosurgeons prefer to operate on a patient in the supine or semi-sitting position with the head slightly extended. Radiofluoroscopic control is always the most commonly used; some still prefer navigation systems. The procedure can be achieved with or without dissection of the septal mucosa. Either a sublabial or medial nasal incision is used. The medial nasal mucosa is separated unilaterally from the cartilaginous and bony nasal septum.
A nasal speculum is inserted to maintain mucosal tunnel patency, or a direct endonasal approach to the sphenoid sinus is used. The vomer (an anatomical landmark in the cranium), which acts as an ideal midline orientation, is revealed and outstretched with forceps and drills. The detached sphenoid sinus is resected, visualizing the sellar floor through the sphenoid sinus. Once the sella floor is removed, the basal dura of the pituitary fossa may be incised, and the gland and adenoma may be envisioned.
The sella tunica's content can be adequately pictured via the transsphenoidal approach. The sectioning of the gland, adenomectomy (removal of the pituitary adenoma), or a variant of hypophysectomy can be completed afterward. The operating microscope and the endoscope permit an adequate visualization of the intrasellar content and help to properly locate and detail the content.
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Selective Adenomectomy:
In microadenomas, the tumor is mainly entrenched within the pituitary. Therefore, it can be selectively released from the normal pituitary gland, whereas in larger adenomas, the dural opening permits the tumor to protrude via this opening. The normal pituitary gland is determined by its yellowish color, firm consistency, and vascular surface structure.
To preserve, only the pathological gland is resected with the 2 mm healthy margin to prevent adenoma recurrences. The extent and radicality of tumor resection can be evaluated by inspection and palpation of the tumor niche, visualization of the cavernous sinus (dural venous sinuses of the brain associated with neuro-vasculature) bilaterally, and in larger tumors with suprasellar extensions determined by the arachnoid that dives into the intrasellar space. Using curettes and microforceps for ease, the tumor is resected. The demanding criteria in adenomectomy, which requires technical expertise, are the vascularization of the basal dura and the proximity of the cavernous sinus and carotid arteries.
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Hypophysectomy:
The ACTH-secreting pituitary cells are in the medial portion of the gland, and evidential reports suggest that many tiny tumors lie in the midline. In the event of not identifying a distinct microadenoma intraoperatively, it is suggested that half the pituitary is resected at the side with the more elevated ACTH concentration. Hypophysectomy is the ultimate option, resecting the entire gland in one attempt.
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Transcranial Surgery:
The factors indicative of transcranial surgery are more restrictive. However, transcranial surgery is still being performed on a suprasellar tumor with only a minor or no intrasellar segment. The sub-frontal approach is mostly used. The front-lateral or frontotemporal craniotomies are usually preferred. The brain is protected by a basal bone flap, followed by CSF drainage. The visual pathways and the major arteries of the anterior cerebral circulation are dissected. The tumor is then removed medially between the optic nerves or laterally between the optic nerve and carotid arteries.
What Are the Complications After Surgical Intervention for Cushing Syndrome?
Cushing's disease patients are more prone to complications from pituitary surgery than patients with other diagnoses. As a result, the mortality rate ranges from 0.9 to 1.9 %.
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Venous Thrombosis: Blockage of the vein caused by a blood clot (thrombus). The most common is deep vein thrombosis, where the blood clot in the lower limb causes reduced blood flow and necrosis or gangrene formation.
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Pulmonary Embolism: A blood clot or thrombus in the lungs' arteries that causes reduced blood flow to the lungs.
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Gastrointestinal Bleeding: It is related to peptic ulceration due to elevated acid levels in the gastrointestinal tract.
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Infections:
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Meningitis: It is inflammation of the brain's protective covering surface mainly caused by a bacterial, viral, or fungal infection.
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Pneumonia: It is an infectious disease that causes inflammation of the air sacs, which are filled with fluid or pus (purulent material) in one or both lungs. Thus, symptoms like cough with sputum (phlegm) or pus, fever, chills, and difficulty breathing are mainly caused by bacteria, viruses, and fungi.
What Is the Self-Care That Can Be Done to Recover From Cushing Syndrome?
Recovering from Cushing syndrome is a slow process, but certain lifestyle changes can help the recovery process, such as
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Increasing Activity at a Slower Pace:
Performing comfortable exercise regularly without overloading. Avoid high-impact exercise, which can cause injury.
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Proper Intake of Food:
Nutritious food plays a major role during recovery. It will also aid in losing weight gained due to Cushing syndrome. A proper amount of calcium and vitamin D should be taken, which help to strengthen the bones and compensate for the bone loss due to Cushing syndrome.
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Good Mental Health:
Depression is one of the main side effects of Cushing syndrome. Depression should not be ignored, and immediate help needs to be taken.
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Gently Sooth Aches:
Hot baths and massages can help eliminate some muscle and joint aches that occur during the recovery phase of Cushing syndrome.
Conclusion:
Every disorder or disease has its own pathophysiology and principle elements that cause an imbalance between the natural physiological progress and the pathology of the syndrome. Therefore, the principal treatment is ruling out and treating the cause. Thus, ACTH (adrenocorticotropic hormone) based imbalance Cushing syndrome needs surgical intervention.
