Juvenile angiofibroma of the skull base presentation. Symptoms of angiofibroma of the nasopharynx and its treatment
93. Foreign bodies of the esophagus.
Foreign bodies of the esophagus.A wide variety of objects can get stuck in the lumen of the esophagus - fish and meat bones, coins, badges, pins, sewing needles, buttons, removable dentures, etc. Most often, foreign bodies occur in children and elderly people wearing dentures, covering the hard palate, the receptors of the mucous membrane of which control the readiness of the bolus of food for swallowing. The reasons for foreign bodies entering the esophagus are hasty eating, lack of teeth, diseases of the masticatory apparatus, and the habit of holding various objects in the mouth. Conversation and laughter while eating contribute to the entry of foreign bodies. Round foreign bodies are less dangerous than sharp-angled ones or those with fixing needles (for example, badges), as well as removable dentures, since their hooks, when infected, damage the wall of the esophagus and can cause esophagitis. In 80% of cases, foreign bodies get stuck in the first physiological narrowing of the esophagus (its cervical part), equipped with striated muscles capable of making sharp and strong contractions to swallow a bolus of food into the lower parts of the esophagus. That is why, if a foreign body, especially with sharp edges, has penetrated into the lumen of the initial section of the esophagus, then under the influence of irritation of the receptors of the mucous membrane, muscle contraction, spasm of the upper esophagus and even stronger fixation of the foreign body occur. If a foreign body is pushed lower, it can become wedged in the area of the second physiological narrowing (thoracic region) or in the area of the third physiological narrowing (at the entrance to the stomach) of the esophagus. Clinic Spontaneous pain in the neck and chest, difficulty and sharply painful swallowing, pain when swallowing radiates (gives) to the arm, back, between the shoulder blades; salivation. When examining the laryngopharynx (hypopharyngoscopy), the abundance of saliva in the pyriform sinuses is determined. X-ray examination allows us to identify the presence and location of a foreign body. Radiopaque foreign bodies are easily identified. Non-contrast foreign bodies are detected by swallowing barium mass. A sip of water does not wash away the barium that has accumulated near the foreign body. Diagnosis. Established on the basis of anamnestic, clinical and radiological data. Treatment. Produced by a qualified specialist. After a mandatory preliminary X-ray examination and clarification of the location of the foreign body, esophagoscopy is performed (under anesthesia or local anesthesia) and the foreign body is removed with special forceps. Recently, esophagoscopy for diagnostic purposes and to remove a foreign body is performed using a fibroesophagoscope. However, large foreign bodies wedged into the walls of the esophagus are safer to remove using a rigid esophagoscope.
94. Juvenile fibroma of the nasopharynx
Juvenile fibroma of the nasopharynx- a fibrous tumor of dense consistency, characterized by significant bleeding, which is why it is called angiofibroma. The place of origin of the tumor is most often the nasopharynx, its pharyngeal base. Etiology unknown. There are “theories” of disembryogenesis and endocrine dysfunction. Pat. anatomy. Nasopharyngeal fibroma is a very dense tumor sitting on a wide base, closely fused to the periosteum. The strength of the fusion with the periosteum is so great that in other cases, when a tumor is removed by tearing it out, fragments of the underlying bone are also removed along with it. The surface of the tumor is covered with smooth papillary formations of pale pink or reddish light, depending on the degree of vascularization of the tumor. The tumor has a pronounced extensive growth, dislocating and destroying all tissues and filling all neighboring cavities located along this path (choanae, nasal cavity, orbit, sphenoid sinus, lower parts of the nasopharynx, paranasal sinuses, zygomatic and temporal fossae, etc.) . The tumor is covered with epithelium, which in the nasopharynx has the character of a single-layer epithelium, and in the nasal cavity - a cylindrical ciliated epithelium - a fact explained by the fact that the tumor arises in the zone of transition of squamous epithelium to cylindrical. The vascular content of the tumor, especially with the venous plexuses, is very large. Symptoms. Symptoms of nasopharyngeal fibroma develop slowly and are divided into subjective and objective. In the initial stages of tumor development, there is a gradually increasing difficulty in nasal breathing, sometimes causeless headaches and dull pain in the base of the nose, high mental and physical fatigue, due to which children begin to lag behind in mastering the school curriculum, in physical development, and often get sick colds. Nasal discharge is mucopurulent in nature. Complaints appear, first of minor ones, then of ever-increasing nosebleeds, due to which the child develops anemia. Subsequently, the tumor fills the nasal cavity, causing increasing difficulty in nasal breathing, up to its complete absence. The child's mouth is constantly open, his speech becomes nasal, obstruction of the auditory tube causes hearing loss, and at the same time there is a loss of smell and a decrease in taste sensitivity. When the tumor comes into contact with the soft palate, patients complain of impaired swallowing function and frequent choking. Compression of sensitive nerve trunks causes neuralgic eye and facial pain. With objective Upon examination, the doctor sees a pale child or young man with a constantly open mouth, blue stains under the eyes and a swollen base of the nose. In the nasal passages there is mucopurulent discharge, which the patient cannot remove by blowing his nose due to obstruction of the choanae (expiratory valve) by the tumor. After removing these secretions, a thickened, hyperemic mucous membrane and enlarged bluish-red nasal turbinates appear. After lubricating the mucous membrane of the nose with adrenaline and contracting the turbinates, the tumor itself becomes visible in the nasal passages in the form of a sedentary, smooth, grayish-pink or reddish formation that bleeds heavily when touched with a sharp instrument. During posterior rhinoscopy a typical picture in which the choanae, vomer and even the posterior ends of the nasal conchae are clearly visible is not determined. Instead, a massive bluish-red tumor is detected in the nasopharynx, completely filling it and sharply different in appearance from banal adenoid vegetations. During digital examination nasopharynx, which should be carried out carefully so as not to cause bleeding, a dense, immobile solitary tumor is determined. Associated objective symptoms may be lacrimation, exophthalmos, and dilatation of the nasal root. With the bilateral manifestation of these signs, the patient’s face takes on a peculiar appearance, which is called “frog face” in foreign literature. During oropharyngoscopy A bulging of the soft palate may be detected, due to the presence of a tumor in the middle parts of the pharynx.
The most serious complication is perforation of the cribriform plate by the tumor and its penetration into the anterior cranial fossa Diagnosis Nasopharyngeal fibroids are determined mainly by the clinical picture, the fact of the occurrence of nasopharyngeal fibroids in males in childhood and adolescence. The extent of the tumor is determined using X-ray or MRI or CT examination, as well as using angiography. Nasopharyngeal fibroma differentiate from adenoids, cysts, fibromyxoma, cancer and sarcoma of the nasopharynx. The main distinguishing feature of fibromas is their early and frequent bleeding, which is not observed at all in all other benign tumors of this localization, and in malignant tumors bleeding is observed only at an advanced clinical and morphological stage. Treatment. surgical. Denker's operation is a preliminary surgical intervention to create wide access to the internal pathological formations of the nasal cavity and, in particular, to the maxillary sinus, the middle and posterior parts of the nasal cavity and the base of the skull (nasopharynx, sphenoid sinus). In advanced cases, when the tumor grows into the zygomatic region, paranasal sinuses, orbit or retromaxillary region, it is removed after creating preliminary approaches. According to A.G. Likhachev (1939), the most advantageous access to the tumor is transmaxillary according to Denker. Non-operative treatment methods as independent means do not always give a positive result; It is advisable to carry them out as preoperative preparation or as an attempt at non-operative treatment. For small tumors it can be used diathermocoagulation with the endonasal approach in persons 18-20 years old with the expectation that they will soon complete puberty and will no longer experience a relapse. Main hormonal agent in the treatment nasopharyngeal fibroids is testosterone, which has androgenic, antitumor and anabolic effects. It regulates the development of male genital organs and secondary sexual characteristics, accelerates puberty in males, and also performs a number of other important biological functions in the developing organism. It is used in various dosage forms (capsules, tablets, intramuscular and subcutaneous implants, oil solutions of individual esters or combinations thereof). For nasopharyngeal fibroma, use 25 to 50 mg/week of the drug for 5-6 weeks. Treatment is carried out under the control of the content of 17-ketosteroids in the urine - normally, the excretion of 17-ketosteroids in men averages (12.83±0.8) mg/day (from 6.6 to 23.4 mg/day), in women - (10.61±0.66) mg/day (6.4-18.02 mg/day) and can be repeated with normal content of this excretion in the urine. An overdose of testosterone can lead to testicular atrophy, early appearance of secondary sexual characteristics in children, disorders of osteonesis and psyche, as well as an increase in the content of 17-ketosteroids in the urine.
The prognosis depends on the extent of the tumor, the timeliness and quality of treatment. With small tumors recognized at the very beginning of their occurrence and appropriate radical treatment, the prognosis is usually favorable.
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Juvenile angiofibroma of the nasopharynx- a benign tumor that grows in the inner cavity of the nose; occurs almost exclusively in boys during puberty. It is based on connective tissue (fibroma) and vascular tissue of varying maturity (angioma).
Juvenile angiofibroma is one of the most common tumors of the nasopharynx in adolescents: 1 in 50–60 thousand ENT patients or 0.5% of all head and neck tumors.
Angiofibroma, formally being a benign neoplasm, has the features of malignant tumors due to its localization, tendency to relapse and germination into structures bordering the nasopharynx and nasal cavity (pterygopalatine fossa, anterior and middle cranial fossa, orbit).
Intracranial (intracranial) growth is observed in 10-20% of cases. The most dangerous and prognostically unfavorable is the spread of the tumor around the intersection of the optic nerves and the pituitary gland, which makes it inoperable.
Types of angiofibromas depending on the source of tumor growth:
1. Sphenoethmoidal (begins to grow from the sphenoid bone, ethmoid bone, pharyngeal-basic fascia).
2. Basal (nasopharyngeal vault) - most common.
3. Pterygomaxillary (pterygoid process of the sphenoid bone).
Classification of juvenile angiofibroma (Fisch, 1983; Andrews, 1989)
This classification is used by almost all modern authors who publish articles on this problem.
Stage I – the tumor is limited to the nasal cavity;
Stage II – spread to the pterygopalatine fossa or maxillary, ethmoid, or sphenoid sinuses;
Stage IIIa – spread to the orbit or infratemporal fossa without intracranial (intracranial) growth;
IIIb – stage IIIa with extradural (without involvement of the dura mater) spread;
Stage IVa – intradural spread without involvement of the cavernous sinus, pituitary gland or optic chiasm;
Stage IVb – involvement of the cavernous sinus, pituitary gland or optic chiasm.
Symptoms and course of juvenile angiofibroma of the nasopharynx
The earliest and most common signs are:
Recurrent nosebleeds;
Unilateral nasal congestion;
Increasing difficulty in nasal breathing;
Hyposmia and anosmia (decrease and disappearance of the sense of smell);
Nasality;
Hearing loss in one (usually) or both ears;
Increasing headaches;
Deformation of the facial skeleton (in later stages leads to displacement of surrounding tissues).
Depending on the direction of growth of angiofibromas, the following may occur:
Facial asymmetry;
Deformation of the soft and bone tissues surrounding the tumor;
Impaired blood supply to the brain;
Compression of nerve endings;
Visual impairment (decreased visual acuity, exophthalmos (displacement of the eyeball forward (bulging eyes), sometimes with a shift to the side), limited mobility of the eyeballs, double vision (diplopia), etc.).
If the tumor grows aggressively, it can grow into the cranial cavity, which can cause death.
Diagnosis of juvenile angiofibroma of the nasopharynx:
1. Collection of complaints and medical history.
2. Rhinoscopy.
3. Endoscopy of the nasal cavity and nasopharynx.
4. Radiography
5. CT, MRI.
6. Bilateral carotid angiography.
7. Biopsy. A cytological test of the biopsy is necessary to clarify the diagnosis and type of tumor.
8. Examination by an ophthalmologist, neurologist, neurosurgeon and oncologist.
They try to carry out tissue biopsy analysis only in a hospital (in the operating room), because after taking a biopsy, bleeding from the vessels of the nose may occur.
Differential diagnosis of juvenile angiofibroma of the nasopharynx with diseases such as:
Adenoids in children;
Brain tumor;
Choanal polyps;
Sarcoma;
Papilloma.
Treatment of angiofibroma of the nasopharynx
Treatment is surgical only! To reduce trauma, angiofibroma is removed using endoscopic methods. The disease can progress over several years, but in some cases it manifests itself as a malignant neoplasm, that is, it grows rapidly. Therefore, if angiofibroma of the nasopharynx is suspected and the diagnosis is established, surgery is performed as early as possible.
Types of operations:
Surgery using natural approaches to the tumor (through the nose or mouth);
An operation using gentle access through the maxillary sinus and nasal cavity (with an incision under the lip) - Denker operation;
Operation using expanded access through the maxillary sinus and nasal cavity (with a facial incision) - Moore operation, Weber-Fergusson operation;
Operation using access through the palate - Owens operation.
During surgery, there is usually severe bleeding, which requires massive blood transfusions. To reduce blood loss, ligation of the external carotid artery is often performed before tumor removal.
In the postoperative period, the ENT doctor prescribes:
Antibiotics (protection against possible infection);
Transfusion of solutions to replenish blood loss;
Medicines that promote blood clotting.
Juvenile angiofibroma of the nasopharynx does not require preventive measures - tumor formation cannot be prevented. However, in order not to start the course of the onset of the disease, you should immediately contact an ENT doctor at the first characteristic manifestations.
Prognosis for juvenile angiofibroma of the nasopharynx
The prognosis of the disease is usually favorable. Timely surgery in combination with radiation therapy leads to a complete cure of the cancer patient.
In rare cases, a negative result of antitumor treatment is observed in the form of relapse or malignancy (malignancy) of the tumor. Therefore, operated patients are recommended to undergo annual examinations by an otolaryngologist.
Take care of yourself and, if necessary, contact an ENT surgeon on time.
Angiofibroma is a rare disease; it is formed from blood vessels and connective tissue. In oncology, angiofibroma is often considered in combination with dermatofibroma.
More about angiofibroma
The locations of this tumor are most often the nasopharynx and skin, although other locations are also found - tendons, mammary glands, torso, neck, face, ovaries, uterus, lungs, vocal cords.
Pay attention! Typically, cutaneous angiofibroma is diagnosed in males after 40 years of age, but can also form in women. This type of neoplasm is associated with photoaging of the dermis, so people of the older generation are considered at risk.
In children during puberty, juvenile-type angiophyroma (youthful) can be diagnosed, which can go away on its own with age.
Remember! Typically, juvenile angiofibroma is diagnosed in the nasopharynx area - this is the so-called mutation of the nasopharyngeal space.
Leading clinics in Israel
Causes
The exact cause of angiofibromas has not been established, but it is generally accepted that abnormal development of the embryo plays a significant role here.
In general, there are several theories about the occurrence of this disease:
- genetic. It is one of the most common, since the vast majority of patients diagnosed with angiofibroma have chromosomal abnormalities;
- hormonal. Frequent diagnosis of one of the types of angiofibroma in adolescents during puberty gives reason to assume the cause of angiofibroma formation is hormonal imbalance;
- age. The risk of getting this disease increases with age, given the natural aging process of the body.
In addition to the above theories, there are assumptions regarding the effects of some other factors:
One of the main factors provoking the disease is long-term exposure to sunlight (photoaging), which causes mutations in cells located in the deep layers of the skin. In the process of mutation, they begin to rapidly divide and form a focus with pathologically developed blood vessels.
Sometimes multiple angiofibromas (angiofibromatosis) occur, which can be the result of hereditary diseases - neurofibromatosis and tuberous sclerosis.
Symptoms of the disease
Symptoms of the disease include manifestations that depend on the location of the angiofibroma:
- in most cases (80-90%) in the early stages of the disease, chronic nasal congestion occurs (with angiofibroma in the nasopharynx);
- Almost half of the cases (45%) may experience nosebleeds. More often they are unilateral;
- due to congestion of the paranasal sinuses, frequent headaches may occur;
- if juvenile angiofibroma has a significant spread, then it can provoke disturbances in visual and auditory functions;
- when localized in the face area, swelling may occur and facial asymmetry may be observed.
In some cases, no symptoms are observed (with kidney damage).
Externally, this neoplasm may look like a single node (up to 3 mm in size) rising above the skin. Its color can range from almost indistinguishable from skin color to dark brown, and the top layer of the formation is transparent and makes it possible to see the capillary pattern of small vessels. The knot itself is slightly denser than the surrounding skin, but is quite elastic. Although the node itself is painless, it is accompanied by mild itching. The tumor develops slowly and does not cause changes in surrounding tissues.
Types of tumor
Depending on the microscopic structure, cutaneous angiofibromas can be divided into:
- hypercellular. They consist of a large number of fibroblasts - immature connective tissue cells;
- typical (angiofibrolipoma). These types of tumors are composed of foam cells that contain lipids;
- pigmented. Such a tumor contains many melanin pigments and is often mistaken for a mole;
- pleomorphic. This type contains nuclei of different shapes and sizes, and this sometimes leads to an incorrect diagnosis - a malignant skin tumor is determined - sarcoma;
- granular (angiogranuloma). The internal environment of such cells has granules, which is why they can be confused with malignant ones.
Based on location, this disease can be divided into:
Depending on the clinical and anatomical features, angiofibroma can be distinguished:
- basally widespread;
- intracranial-extended.
If we consider angiofibroma of the nasopharynx in more detail, then Several stages of its development can be distinguished:
- Stage 1 – the tumor does not extend beyond the nasopharynx;
- 2 – pathological tissues spread to the pterygopalatine fossa, to some sinuses (maxillary, sphenoid, ethmoid);
- Stage 3 can occur in two variants: 1 – the pathology begins to spread to the orbital area and the subtemporal region, 2 – the dura mater of the brain is involved in the process;
- Stage 4 can also occur in two variants: the first variant is characterized by damage to the dura mater, but without involving the pituitary gland, optic chiasm or cavernous sinus in the pathological process, the second variant is characterized by the spread of the tumor to all of the above areas.
Diagnosis of the disease
Pathological proliferation of skin tissue can be diagnosed based on an external examination, which is carried out in conjunction with. The final diagnosis is made based on the results of histology. To perform a biopsy, a small area of the pathological lesion is removed and a laboratory analysis of the biopsy is done.
In addition to these diagnostic methods, the following are carried out: palpation of suspicious areas of the body, MRI (it identifies the boundaries of the formation, localization and spread of the tumor), anterior and posterior rhinoscopy, radiography, ultrasonography, CT, fiberoscopy, angiography. Various laboratory tests are also carried out: blood, blood biochemistry, hormonal tests.
Important! If angiofibroma is suspected, it is first necessary to exclude cancer. They also perform differential diagnosis with diseases such as: hemangiomas, lipomas, nevi, melanoma, etc. In children, angiofibroma is differentiated from polypous growth, nasopharyngeal cancer and sinusitis.
Treatment of angiofibroma
Therapy for angiofibroma of the nasopharynx can be carried out using the following methods:
- hormone therapy. Hormone treatments include the use of testosterone, which blocks tumor growth and causes tumors to shrink by 44%;
- radiotherapy. This type of treatment has some limitations due to the high rate of radiological complications. Doctors often advise using a stereotactic technique, which uses high-precision and dosed use of radiation;
- surgical intervention. Due to the presence of a dense network of blood vessels, removal surgery is not always possible. Recently, intranasal endoscopic surgery has been used more often, when the surgeon excises the tumor with minimal damage to nearby normal tissues.
After removal, the tumor almost never recurs.
*Only upon receipt of data on the patient’s disease, a representative of the clinic will be able to calculate an accurate estimate for treatment.
Consequences after surgery
Sometimes after surgery, relapses of the disease, surgical bleeding and damage to adjacent tissues are possible.
After radiotherapy, the following complications may occur:
Disease prognosis and prevention
The prognosis of the disease is favorable. Timely surgery in combination with radiotherapy gives a high chance of curing the patient. In exceptional cases, relapse of the disease or malignancy of the tumor may occur. Statistics say that after removal of an angiofibroma, it may undergo cancerous degeneration in 2-3 years of the rehabilitation period. For timely diagnosis of the disease, it is necessary to undergo annual preventive examinations with an otolaryngologist.
- maintaining a healthy lifestyle;
- proper nutrition;
- giving up bad habits;
- avoiding nervous and physical stress.
According to the International Histological Classification of Neoplasms, juvenile nasopharyngeal angiofibroma (JAN) belongs to the group of mesenchymal tumors and has a benign histological structure. It is found exclusively in males, mainly during puberty (aged 7 to 21 years). According to some authors, JAN occurs in 50% of cases among patients with benign tumors of the nasopharynx or in less than 0.05% of cases with tumors of the head and neck.
Possessing the property of an expansive form of growth, the tumor, regardless of its original location (the fornix of the nasopharynx, the edge of the pterygopalatine notch, the medial plate of the pterygoid process of the sphenoid bone, etc.), almost always occupies the nasopharynx, spreads to certain anatomical areas of the facial skeleton, to base of the skull, and sometimes penetrates into the cranial cavity. Intracranial spread of the tumor accounts for 17 to 36% of all patients with JAN and poses an even greater threat to the patient’s life than tumors spreading at the base of the skull.
When removing JAN, various operations are used, among which the following have become widespread: operations using access through natural routes; surgery using gentle access through the maxillary sinus and nasal cavity (with an incision under the lip or Denker surgery); surgery using extended access through the maxillary sinus and nasal cavity (with a facial incision or Moore operation), as well as surgery using access through the palate.
Numerous works have been devoted to the issue of removal of SAN by access through natural routes. The general principle of surgery when removing tumors using natural approaches is that the tumor is separated from the surrounding tissues both through the mouth and through the nose, or through the mouth and nose at the same time. When accessing the tumor through natural pathways, the authors usually use auxiliary manipulations or preliminary operations. Thus, when using access through the oral cavity, the soft palate is first pulled back using rubber reins inserted through one or both nasal cavities. As a result of the displacement of the soft palate anteriorly, the lumen of the nasopharynx (from the side of the oral cavity) expands, which makes it easier to isolate the tumor and remove it through the oral cavity. During an operation using access through the nasal cavity, the nasal septum is initially temporarily shifted to the side. It is achieved by crossing the nasal septum along its entire length (at the base) and/or resection of the posterior part of the vomer. This, according to the authors, allows free separation of the tumor and its removal through the nasal cavity.
Most supporters of operations performed using natural approaches consider it justified to use these approaches when removing the so-called “small” JAN, when the tumor occupies the nasopharynx, nasal cavity and sphenoid sinuses. In their opinion, the operation is accompanied by minimal trauma to surrounding tissues and preserves the architecture of the nasal cavity.
It should be emphasized that access through natural routes also has its negative sides. Thus, when access is made through the oral cavity, even when the soft palate is displaced anteriorly due to its insufficient mobility, the vault of the nasopharynx and choanae, which may be the original sites of the tumor, remain inaccessible for viewing. This naturally makes it difficult to visualize the tumor, which leads to its removal “blindly”. At the same time, access through the nasal cavity is also not adequate, since when the transected nasal septum is displaced in the opposite direction from the tumor, only the volume of the nasal cavity increases, and the entrance to the nasopharynx remains limited by the size of the choanae. This, in turn, creates additional difficulties in isolating the tumor from the above anatomical areas and thereby jeopardizes the radicality of surgical intervention.
In contrast to supporters of the use of access through natural routes, most researchers, when removing “small” JAN (in particular, tumors spreading into the nasopharynx and nasal cavity), prefer surgery using access through the palate.
When removing tumors of the nasopharynx using access through the palate, the Owens operation became the most widespread. The operation begins with making a horseshoe-shaped incision in the mucous membrane of the hard palate along the edge of the gum. After exposing the bone surfaces, part of the hard palate is resected on the side of the predominant tumor location. If necessary, access can be expanded by resection of the posterior part of the vomer.
Access to the nasopharynx through the palate has a number of advantages and disadvantages, well formulated G. Spigno and G. Zampano . Thus, the advantage of access is the absence of an external scar and a good view of the nasopharynx, and the disadvantage is the danger of the formation of a fistula of the hard palate, the relative limitation of the surgical field, as well as the inability to control the tumor if it spreads beyond the nasopharynx. According to J. J. Pressman , the main disadvantage of access through the palate (especially when removing JAN) is that this access allows you to reach only the distal part of the tumor and its process freely lying in the nasal cavity, which, as usual, are not fused with the surrounding tissues. In this case, the separation of the proximal part of the tumor from the surrounding tissues is performed “blindly,” which, in the author’s opinion, leaves much to be desired.
Many authors, when removing “small” tumors, perform an operation using gentle access through the maxillary sinus and nasal cavity (with an incision under the lip), known as the “Denker operation”. This operation, according to the authors, provides access to both the nasopharyngeal vault, the nasal cavity and the maxillary sinus.
When performing the Denker operation, an incision is first made in the mucous membrane of the upper lip, then the tissue is separated and the edge of the pyriform opening is released. Next, the pyriform opening is expanded, during which the anterior and medial walls of the maxillary sinus are resected (the inferior turbinate is also resected), the anterior edge of the frontal process of the maxilla and the nasal bone. Some supporters of the use of this operation also consider it justified when removing “medium” JAN, in particular tumors occupying the pterygopalatine fossa and orbit.
Surgical intervention performed through an extended approach through the maxillary sinus and nasal cavity (with a facial incision) is known in otolaryngology as the “Moore operation.” Supporters of the use of this operation consider it justified when removing both “small” and “medium” SANs, i.e. in cases of tumor spread into the nasal cavity, ethmoid sinuses, sphenoid sinuses and orbit.
When performing this operation, a skin incision is made, starting from the medial end of the eyebrow to the upper lip, which runs along the lateral slope of the nose, followed by bordering its wing. After dissecting the dissected soft tissues to the sides, sequential resection of the frontal process of the upper jaw (not reaching the pyriform foramen), nasal and lacrimal bones, as well as the orbital plate of the ethmoid bone is performed.
Among the numerous modifications of this operation, the most widespread is the “Weber-Fergusson” operation.
In contrast to the classic Moore operation, when performing modified operations, the upper lip is additionally dissected, the anterior and medial walls of the maxillary sinus and/or the posterior edge of the vomer are resected. As independent operations, they are also used when the tumor has spread to the pterygopalatine and infratemporal fossa or to the lateral part of the base of the skull. When removing “large” or intracranially spreading tumors, some authors use these modified operations in combination with neurosurgical surgery.
According to some authors, an operation performed using expanded access through the maxillary sinus and nasal cavity (with a facial incision) has a number of advantages and disadvantages. The advantage of this access is the provision of a fairly wide approach to the paranasal sinuses and the pterygopalatine fossa, as well as the possibility of ligating the maxillary artery, but the disadvantage is the extensive destruction of the bones of the facial skeleton and the formation of a postoperative scar on the face.
A comparative assessment of the operations used by various authors shows that most authors, when removing “small” or “medium” JAN, prefer surgery using access through the palate or surgery through the maxillary sinus and nasal cavity than surgery through natural pathways or combined surgery.
Along with the use of a number of well-known operations, some authors support the use of unconventional operations when removing the Yuan. So, if some authors perform a pharyngotomy when removing “medium” tumors, others prefer the so-called lateral nasopharyngotomy for this purpose. Lastcarried out by using access through the infratemporal fossa. During this operation, an arcuate skin incision is made, which passes through the temporal and parotid regions. The indication for the use of this operation, according to the authors, is the spread of JAN into the retromandibular fossa.
From our point of view, an operation performed by performing a pharyngotomy cannot be justified as an adequate surgical intervention for the removal of JAN, since the resulting relatively large distance to the tumor, as well as the inability to control its processes, reduce the merits of this operation.
Some proponents of the use of unconventional operations when removing “medium” tumors perform surgery using palatoalveolar access, while others use osteotomy type Le Fort 1.
Among the various operations used to remove the “middle” JAN, over the past 20 years, operations using access through the middle part of the face, known as “ Midfacial degloving ”, which translated means “removing the skin of the middle part of the face, like a glove.”
When performing this operation, a circular vestibular incision is first made in the nasal cavity, then the nasal septum is dissected, and then an incision is made along the transitional fold of the upper lip. After connecting the incisions, the bridge of the nose is exposed. This allows for complete separation of the soft tissues of the face down to the lower and inner edges of the orbit and upward to the frontonasal suture, without making an external incision. According to J. Trotoux et al , this operation provides wide access to the nasal cavity and paranasal sinuses up to the clivus, and therefore, if the tumor spreads to the base of the skull, it can also be combined with neurosurgical surgery. The combined use of this operation has been reported J. D. Browne and A. N. Massner . Thus, using a combined operation, the authors carried out complete removal of some intracranial spreading JAN. Unlike them L. G. Close et al . when removing intracranially spreading tumors, this operation was used in combination with an operation performed through the palate.
To summarize, regarding some aspects of surgical treatment of patients with JAN, it should be noted that, despite the use of various operations, the number of tumor relapses currently remains high. The main reason for the development of relapses, apparently, is the continued growth of the tumor remaining as a result of its incomplete removal. At the same time, complete removal of the tumor largely depends on the differentiated use of adequate surgery, taking into account the degree of tumor spread.
The works of most authors concerning the issue of surgical treatment of patients with intracranial spreading JAN deserve special attention.
For the first time, complete removal of intracranial spreading JAN was carried out by E.A. Krekorian and L.G. Kempe. The authors used a combined neuro- and rhinosurgical operation, which included bifrontal craniotomy (osteoplastic trepanation of the frontotemporal region) and the Weber-Fergusson operation. The principle of the neurosurgical operation was to open the subarachnoid space (intracranial and transdural approach) and free the tumor from the blood vessels feeding it inside the skull. To isolate the tumor from the blood supply, both from the internal carotid artery itself and from its intracranial branches (extradural branching of the intracavernous part of the internal carotid artery, ophthalmic artery, middle artery of the dura mater), in all cases, after appropriate preparation of the patient, occlusion of the internal carotid artery was carried out in two places . So, first, the proximal part of the artery was ligated on the neck, then its distal part was clipped inside the skull. Occlusion of the distal part of the internal carotid artery leads to the cessation of retrograde blood flow into the blood vessel feeding the tumor, which in turn eliminates the threat of bleeding during tumor separation. Only after this the authors carried out complete removal of the tumor through rhinosurgery.
Considering that among patients with intracranial spreading JAN, in most cases the intracranial process of the tumor is localized extradurally, the use of a combined operation cannot be justified in all cases, since opening the subarachnoid space, which is included in the surgical plan, is in itself unsafe. One of the life-threatening complications of the patient is the threat of infection of the wound inside the skull and possible leakage of cerebrospinal fluid, which can lead to serious consequences.
In contrast to the above-mentioned combined operation, in order to carry out radical removal of intracranial spreading JAN, U. Fisch developed an alternative operation - tumor removal using access through the infratemporal fossa (intracranial and extradural access). This operation does not have the disadvantages that occur during a combined operation, and allowed the author to completely remove the tumor without opening the subarachnoid space in some patients with intracranial spreading JAN. Only when the tumor spreads to the cavernous sinus, given the risk of developing ophthalmoplegia, the author resorts to subtotal resection of the tumor, and recommends removal of the residual tumor using neurosurgical surgery in the future. A number of other authors also report the successful use of this operation when removing “large” JAN.
Undoubtedly, the Fisch operation is justified if we are talking about saving the patient’s life, but at the same time it is not without its drawbacks. One of these disadvantages is conductive deafness, which developed on the side of the operation as a result of a planned extended mastectomy.
G.A. Gates using the basic principle of the operation proposed by U. Fisch, namely the access modified by him through the infratemporal fossa, developed an alternative version of the combined operation, which allowed the author to completely remove intracranially spreading JAN (with tumor invasion of the cavernous sinus), while avoiding damage to the hearing organ.
Assessing the merits of the operation performed, G .A . Gates points out some of its advantages and disadvantages. Thus, the advantage of the operation is adequate access to the tumor, visual control of vital structures inside the skull, preservation of the anatomical continuity of the facial nerve and the function of the lower jaw, as well as an excellent aesthetic result. Among the disadvantages of the operation, the author notes planned damage to the second branch of the trigeminal nerve and the possibility of trismus of the masticatory muscle.
Analysis of literature data shows that, despite the numerous available information concerning the issue of surgical treatment of patients with JAN, the information contained in this information is very difficult to reduce to a “common denominator”. Carrying out various operations even with an adequate form of tumor growth indicates the lack of a rational classification of JAN, which would unify the extent of tumor spread and provide a differentiated approach to the use of surgical intervention in each individual case. Many of the existing SAN classifications are essentially duplicative, and some of them are overly detailed. The lack of a unified classification, however, does not allow for a comparative assessment of the clinical observations of various authors, as well as to evaluate the merits of a particular operation for the removal of JAN.
Thus, the constant interest in the problem of treating patients with JAN allows us to hope that the development of rational tactics for surgical treatment of patients, taking into account the optimization of surgical methods, can significantly improve the results of treatment of patients with this pathology.
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Currently, the number of people with diagnosed neoplasms does not cease to grow, and cases of morbidity have often begun to be recorded among adolescents. For example, among ENT patients, the most common people are diagnosed with formations in the nasopharynx, called angiofibromas.
Angiofibroma (juvenile) of the nasopharynx is a benign tumor that occurs mainly in male adolescents due to hormonal imbalance. Morphologically, it consists of connective and vascular tissues. The tumor is fed by the external carotid artery.
Despite its benign structure, the clinical course of this pathology is considered as malignant, which is associated with rapid progression and frequent exacerbations of the disease, as well as destructive damage to surrounding tissues.
The reasons why angiofibroma of the nasopharynx could occur are not fully understood. There are suggestions that this disease is formed as a result of a disruption in the development of embryonic tissue in the nasopharynx, which was the reason for the creation of the embryonic theory of the occurrence of pathology. In addition to it, there are also hormonal (decrease in androgens, excess testosterone), pituitary, traumatic, mesenchymal, inflammatory and genetic theories of development.
However, a lot of additional research is needed to prove their impact.
Classification
The identification of different forms of juvenile angiofibroma is carried out depending on the anatomical location and direction of growth of the neoplasm. According to localization, the tumor is distinguished at the base of the sphenoid bone, in the region of the posterior wall of the ethmoid bone, and in the pharyngeal fascia. The development of education can occur in various directions:
- From the vault of the nasopharynx to the base of the skull.
- From the base of the sphenoid bone - into the nasal sinuses, the labyrinth of the ethmoid bone and the orbit.
- Into the pterygopalatine fossae and nasal cavity.
The direction of development determines the degree of deformation of adjacent tissues. Its germination can lead to many different complications: blurred vision, limited movement of the eyeballs, their protrusion and many other pathologies. Cases of tumor growth around the optic nerves (in the area of their decussation) and the pituitary gland lead to serious consequences, including death, and are not subject to surgical treatment.
When making a diagnosis, the attending physician must determine the prevalence of the process:
- Stage 1 – tumor without spreading to other parts.
- Stage 2 – there is growth in the area of the pterygopalatine fossa or nasal sinuses.
- Stage 3A – germination into the eye sockets or into the area of the infratemporal fossa.
- Stage 3B – the lesion spreads outward from the dura mater.
- Stage 4A – tumor growth under the dura mater without involving other structures.
- Stage 4B – damage to the cavernous sinus, pituitary gland, optic nerves in the chiasm area.
These data must be taken into account by the attending physician when determining further tactics for managing the patient.
Symptoms of the disease
Angiofibroma of the nasopharynx at the initial stages of its development does not have specific clinical manifestations. But thanks to its rapid growth, a person begins to be bothered by the following symptoms:
- Difficulty in nasal breathing.
- Nasal voice.
- Deterioration of hearing and smell.
- Intense headaches.
Nosebleeds may often occur, which tend to intensify as the size of the tumor increases.
When a tumor grows in a person, visible changes appear in the form of deformation of the facial skeleton and facial asymmetry.
In case of damage to the maxillary sinus, symptoms of dental diseases are observed. With intracranial tumor growth, signs of neurological symptoms appear: increased periosteal and decreased skin reflexes, nystagmus, pain along the course of the trigeminal nerve.
Since the disease does not have early specific manifestations, and the deterioration develops quite quickly, special attention when studying the symptoms should be paid to the runny nose, which cannot be cured by conservative methods.
Diagnostics
When a person with the above complaints seeks medical help, the otolaryngologist, to confirm the alleged diagnosis, prescribes a list of additional tests. Thus, juvenile angiofibroma is detected in the presence of characteristic signs of the disease on x-rays, CT and MRI studies, as well as during angiography and biopsy. The diagnostic features are as follows:
- CT, in addition to visualizing the formation itself, determines the extent of damage to other structures. It is also carried out to evaluate the effectiveness of surgical treatment.
- The advantages of MRI in this case lie in its ability to assess the degree of blood supply to the tumor. Also, unlike CT, it allows for more detailed visualization of low-density formations.
- Angiography also makes it possible to identify the sources of vascularization of the formation, which is taken into account during surgical intervention.
- A biopsy definitively confirms the diagnosis, as it allows one to visualize the characteristic morphological structure of the tumor.
All of the above methods are quite informative, but you should not undergo certain examinations yourself, since only an experienced specialist can correctly interpret the results obtained.
Therapeutic measures
The main goal of therapy when identifying angiofibroma of the nasopharynx is the complete removal of the formation and the prevention of its further relapses. Since surgery can be complicated by massive bleeding (depending on the degree of vascularization of the tumor), it is important to carefully carry out preoperative preparation.
The type of surgery depends on the location, size and growth of the tumor. To reduce the risk of disease relapse after surgical removal of the tumor, specialists in some cases prescribe additional courses of radiation therapy.
If the tumor grows into vital anatomical structures, surgical intervention is strictly contraindicated. In such cases, the patient undergoes only courses of radiation therapy. It is very important to identify the disease at the initial stages of its development, therefore, at the first appearance of alarming symptoms, you must immediately consult your doctor and not self-medicate.
