Skull Base Program

Approximately one in ten adults have an abnormality of the pituitary gland. Most are found incidentally, are benign, and can be monitored over time with no surgical intervention. One reason why a surgeon may offer a surgical procedure could be if the tumor is big enough to press on nearby structures and cause issues with vision or a decrease in normal pituitary function. Another reason for offering a surgical intervention would be if the tumor is causing an over secreting of a pituitary hormone that is in turn causing you harm. Over secretion of pituitary hormone ACTH or adrenocorticotropic hormone causes Cushing’s Disease which can lead to diabetes and heart disease and thereby shorten your lifespan. Over production of pituitary hormone IGF-1 or insulin-like growth factor 1 causes acromegaly in adults or gigantism in childhood. Acromegaly shortens lifespan with complications like high blood pressure, diabetes, and enlargement of soft tissues including the heart (cardiomyopathy).

While there are multiple ways to access the pituitary gland our Skull Base Program physicians specialize in accessing the anterior skull base or “anterior fossa” using the transnasal approach. This means that they use an endoscope or long camera and delicate instruments to go through the nasal passages and sinuses. There is no visible craniotomy scar.

Acoustic Neuromas and vestibular schwannomas are in essence two names for the same tumor. These tumors grow off the sheath or outer covering of the cranial nerves that assist with hearing and balance. Tumors in this region can happen on either the right or left side of the head, are usually benign, and typically cause mild to complete hearing loss and difficulty with balance due to vertigo and dizziness.

While watchful waiting with surveillance imaging may be an option for some tumors, others may require surgical intervention. Access to the lateral skull base or “middle fossa” can be gained through hearing sparing approaches like retrosigmoid or transtemporal craniotomy. Translabrynthine approach craniotomy does not spare hearing. These approaches all require a visible incision.

Enchephaloceles are described as an outpouching of the outer covering of the brain and sometimes brain tissue through a defect in the skull base. These defects can be due to trauma, individual anatomy, or high intracranial pressure. Sometime the defect in the skull base is sharp and may puncture the outer covering of the brain or the “dura” causing a CSF leak. CSF or cerebrospinal fluid is created by your brain and bathes the brain and spinal cord. Your brain creates about ½ quart of CSF daily and reabsorbs it but if there is a hole in the dura it can leak out. People with CSF leaks may notice that they have thin, clear, salty fluid leaking from their noses, their ears, or down the back of their throats. Depending on where the skull base defect is you may be at increased risk for seizures and infections like meningitis.

Treatment for CSF leaks and encephaloceles almost always requires surgical intervention. Access or approach depends on of the defect is in the anterior or lateral skull base. Your skull base specialists will need to patch the hole created in the outer covering of the brain and rid the defected bone of the sharp edges that created the puncture.

Meningiomas are a mostly benign type of tumor the grows from the outer covering of the brain and spinal cord. Small meningiomas may be left alone to watch over time and assess for growth if they are not putting pressure on any other important structures. If a meningioma is putting pressure on an important structure like the optic nerves or blood vessels or if it is growing at a rapid rate your surgeon may encourage surgery to remove it. If a meningioma grows large enough to cause swelling in the brain around the tumor or if it causes seizures your surgeon will strongly encourage surgery.

Meningiomas can occur anywhere on the outer covering of the brain. Therefore surgical treatment of meningiomas can involve numerous techniques to gain access to the tumor including transnasal or anterior, retrosigmoid or lateral, and suboccipital or posterior craniotomies among other non-skull base approaches.

Craniopharyngiomas are a type of benign, or non-cancerous, slow growing tumor of the pituitary gland.

They typically arise from remnants of Rathke’s cleft, a small pouch between the two lobes of the pituitary gland. Symptoms of craniopharyngiomas are typically due to pressure the tumor puts on surrounding structures like the optic nerves, the pituitary gland itself, and the hypothalamus which is responsible for coordinating many bodily functions to maintain your body’s homeostasis. Symptoms can include disruption of hormone production, problems with vision, or hydrocephalus. Hydrocephalus is the blockage of cerebrospinal fluid leading to increased pressure within the skull causing nausea, vomiting, and altered mental status.

Craniopharyngiomas are most often identified using MRI or magnetic resonance imaging. This identifies tumor location, size and pressure on surrounding structures. Bloodwork may also be used to assess hormone dysfunction.

First line of treatment for craniopharyngiomas is surgery. The goal of surgery is to remove as much of the tumor as is safely possible while avoiding damage to the surrounding structures. The Skull Base Program at Hartford Healthcare utilizes an endoscopic trans-nasal approach to these tumors. A long and camera and delicate instruments are inserted up the nose to get access to the base of the skull to remove the tumor. There are no visible incisions created during these procedures.

Due to the complexity of these surgeries a multidisciplinary team approach is utilized and includes Neurosurgery, Endocrinology, Ophthalmology, and Otolaryngology. Occasionally Radiation Oncology is involved for patients who require additional treatment after surgery to help control tumor growth if the tumor grows back

 Long term follow up and management is crucial for addressing hormonal imbalances and ensuring that any tumor recurrence is detected early.

Esthesioneuroblastoma, also called olfactory neuroblastoma, is a rare and aggressive cancer that arises from the neuroepithelial cells of the olfactory system in the upper part of the nasal cavity. The olfactory system is responsible for sense of smell. These tumors are significant due to their potential to spread to surrounding structures as well as their complex treatment needs.

The symptoms of esthesioneuroblastomas depends on the tumor’s size and location. Symptoms can often mimic conditions related to sinus or nasal issues. These tumors are diagnosed through a combination of imaging studies, clinical examination, and biopsy.

A multidisciplinary team, including surgeons, radiation oncologists, medical oncologists, and pathologists, is essential in providing the best possible care for these patients. The choice of treatment depends on the tumor’s size, location, and stage, as well as the patient's overall health.

Surgical resection is the cornerstone of treatment for esthesioneuroblastomas. The goal is to completely remove the tumor. There are different surgical approaches that can be used depending on the location of the tumor. For tumors confined to the nasal cavity or sinuses, an endoscopic approach is often preferred because it is minimally invasive. This is when the surgeon uses a long camera and long delicate instruments and approaches the tumor through the nose. For tumors involving the skull base or brain, a craniotomy may be required to access and remove the tumor and for the tumor invading the orbit or skull base. If the surgery is more extensive reconstructive surgery may be necessary afterward.

Patients may require a radiation oncologist in addition to their surgical team in cases where complete surgical resection is not possible or when there is high risk of recurrence. Chemotherapy may be used in cases where the tumor is advanced or has spread to distant sites. It is often combined with surgery and radiation therapy.

Regular follow-up with imaging (CT, MRI) and physical exams is essential to monitor tumor recurrence.

Rathke’s cyst is a benign, fluid-filled sac that typically forms in the pituitary gland, specifically in the region of the Rathke's pouch. Rathke's pouch is an embryonic structure that eventually forms the anterior part of the pituitary gland. When the pouch does not fully close during development, a cyst can form.

These cysts are usually asymptomatic but can cause symptoms if they grow large enough to compress nearby structures, such as the optic nerves or the pituitary gland itself. Symptoms may include headaches, vision problems, or hormonal imbalances.

Rathke's cysts are typically discovered incidentally during imaging studies (like MRI or CT scans) performed for other reasons. Treatment is generally not required unless the cyst causes significant symptoms. In those cases, options include observation, surgical drainage, or removal.

Surgery is typically reserved for cases where conservative management (such as observation and regular monitoring) does not relieve symptoms. The most common surgical approach for a Rathke's cyst is trans-nasal endoscopic surgery, which involves accessing the cyst through the nasal cavity. This technique avoids the need for an external incision and is less invasive compared to craniotomy (opening the skull).

At Hartford HealthCare, our neurosurgeons use a multidisciplinary approach to managing Rathke's cysts. Such an approach ensures comprehensive care, involving specialists from different fields to address all aspects of the patient's condition. We do collaboration between the following specialties: Neurosurgery, Endocrinology, Ophthalmology and Otolaryngology. This kind of team approach ensures that every aspect of the patient's care is addressed, from diagnosis and treatment to post-operative recovery and long-term monitoring.

Schwannomas are benign tumors that arise from Schwann cells, which are responsible for producing myelin (the protective sheath) around nerve fibers. These tumors typically occur on the cranial, spinal, or peripheral nerves. When they arise in the brain they are referred to as cranial schwannomas or vestibular schwannomas (if they develop on the vestibulocochlear nerve, which controls hearing and balance).

The symptoms of a schwannoma in the brain can vary depending on the location of the tumor and its size. Common symptoms include gradual hearing loss, ringing in the ears or tinnitus, dizziness, headaches, facial numbness or pain or weakness, vision changes if the tumor affects the optic nerve.

A diagnosis of a brain schwannoma is typically made using imaging techniques such as: CT scan in conjunction with MRI. Audiometry is also used to test the hearing to assess the extent of auditory nerve involvement.

Treatment for brain schwannomas depends on the size of the tumor, its location, symptoms, and whether it's growing or stable. There are several management strategies such as observation, surgical removal and radiation therapy. Surgery is usually recommended when the tumor causes significant symptoms (such as hearing loss, tinnitus, or balance issues), or if it is growing rapidly.

A multidisciplinary team approach is essential in the successful management of vestibular schwannomas because of the complex nature of the surgery and its potential impact on several critical functions (hearing, balance, facial movement). The team often includes neurosurgery, neurotology, audiology, and rehabilitation specialists (speech and language pathology, physical therapy and occupation therapy). In cases where the schwannoma has affected balance or hearing, rehabilitation therapies such as vestibular therapy may help improve balance and coordination. This collaborative approach maximizes outcomes and improves the patient's quality of life post-surgery.

The prognosis for patients with brain schwannomas is generally good, as most schwannomas are benign and slow growing. The outcome depends on factors such as tumor size, location, and how well the tumor can be surgically removed. Some patients may experience a complete recovery, while others might have persistent symptoms, especially if the tumor was large or located near critical structures. Patients with vestibular schwannomas may require long-term monitoring for any potential recurrence, but many people with schwannomas go on to live normal, healthy lives after treatment.

Glomus tumors are rare, benign tumors that arise from the glomus body, a specialized vascular structure involved in temperature regulation. These tumors can be found in various locations throughout the body, although the most common sites are in the head and neck region, particularly in areas like the ear (especially the middle ear), the carotid body, and the jugular bulb. They can also occur in the lungs, fingers, and toes.

The symptoms of glomus tumors depend on the location of the tumor and the structures involved. Treatment for glomus tumors depends on their location, size, and symptoms. Options include surgical removal, radiation, embolization or observation.

Surgery is the primary treatment for accessible glomus tumors. The goal is to remove the tumor completely while preserving surrounding structures. In some cases, particularly when the tumor is in a location that makes surgery difficult or risky, radiation therapy (such as stereotactic radiosurgery with Gamma Knife) may be used. This method can help shrink the tumor or prevent further growth. Embolization is offered in some cases, especially for glomus tumors with a rich blood supply. Preoperative embolization (blocking the blood vessels feeding the tumor) can help reduce blood loss during surgery and make the tumor easier to remove. Small, asymptomatic tumors that do not cause significant issues may be monitored over time with regular imaging.

With a thorough diagnosis and a tailored treatment plan, most patients with glomus tumors can expect a favorable outcome, though long-term monitoring is important to detect any recurrence or complications.