Nasal septum is an important anatomical structure supporting the nose (1). Septum consists of three parts:

– membranous – limiting the entrance to the nasal vestibule, consisting of double skin layer,

– cartilaginous – cartilage of the nasal septum,

– bone – made of vomer bone and the perpendicular plate of the ethmoid bone (2).

Nasal cartilage is connected with perpendicular plate of the ethmoid bone through the posterior-superior margin. The cartilage is also connected with vomer and nasal crest of the maxillary bones through the posterior-inferior margin. The posterior-superior and posterior-inferior margin together form the posterior process (sphenoidal process), which is situated between vomer and perpendicular plate of the ethmoid bone. Sphenoidal process is characterized by individual variability and has a key role in the development of nasal septum deviations (3).

Nasal septum perforation is a defect of the nasal septum (4). The resulting perforation is responsible for the airflow between the two parts of the nasal cavity (5). Perforation is a defect of the nasal septum manifested by the disruption of mucosa in the cartillaginous or bone part of the nasal septum or in both of the parts at the same time (1, 4).

Nasal septum perforations not only cause the desintegration of the septum, but also impair the nasal physiology (4). Perforations are a cause of breathing disorders and impaired nasal airflow (6). In addition, large anterior perforations may result in the loss of dorsal support and deformation of the external nose (7).

Normal airflow in the nasal cavity is of laminar nature. In nasal septum perforations, laminar flow is disturbed and substituted with turbulent flow. The turbulent flow is the main cause of crusting, epistaxis, and wheezing (4). The severity of air turbulence is proportional to the size of perforation (7).

Nasal septum perforations occur relatively often. Reports from Sweden estimate the prevalence of perforations in the general population to be 0.9% (8). Other sources indicate that perforations are present in about 1% of ENT patients (9). The prevalence of nasal septum perforations is probably higher than 1%, and the underestimation may be a result of the fact that close to two thirds of the patients remain asymptomatic and never visit a medical professional (1). Nasal septum perforations that remain untreated for a long time may contribute to the destruction of the ciliated epithelium, and thus contribute to dryness of the nasal mucosa (10).Nasal septum perforations are rare in pediatric population. While the symptoms, etiology, and treatment methods of nasal septum perforation in adults are well described in the literature, reports concerning pediatric population are still limited to a small number of cases, and scientific papers consist mainly of case reports (10).

Perforations are classified according to their size, type, and localization (anterior, mid septal, posterior) (8). Anterior perforations are the most common (92%), and less than 10% of perforations are located posteriorly. Posterior perforations are usually asymptomatic and caused by systemic diseases (11). Perforations may have different shapes: from linear to round or oval. Depending on the size, perforations can be divided into small (< 1 cm diameter), medium (1–2 cm), and large (> 2 cm) (12).

Data concerning large perforations is limited. In 2007, Døsen i Haye assessed 197 patients treated for septal perforations in the years 1981–-2005 (100 men and 97 women). The authors confirmed the presence of large perforations in 11% of the studied patients (12). Islam et al. (7) introduced a classification of perforations depending on its height. A perforation that is lower than one third of septal height (0–8 mm) is classified as small, perforations ranging between one third and two thirds of the septal height (9–18 mm) – are classified as medium, and perforations larger than two thirds of septal height – as large. These proportions are changed in patients with a large nasal hump or a collapsed nasal bridge (7). Sapmaz et al. (6) measured the longest vertical dimension of the perforation and vertical length of the septum in the plane in which the perforation is largest. By comparing these two measurements, they created a classification consisting of two groups. The authors believe that the new classification may help surgeons determine the exact size of perforation before the procedure, which will result in a better choice of method of treatment and selection of an appropriate surgical approach, as well as help estimate the number of expected surgical procedures (6).

Perforations contribute to the occurrence of the turbulent airflow in the nasal cavity, which causes dryness to the nasal mucosa. This leads to many symptoms, including crusting, dryness, recurrent epistaxis, nasal congestion, discharge of an unpleasant odor, headache, and wheezing (1).

The symptoms of septal perforation depend on the localization, size, and cause of perforation. Small perforations located posteriorly may be asymptomatic. Anterior perforations are usually associated with wheezing (7). Wheezing occurs more frequently in patients with smaller perforations, whereas epistaxis and crusting are more common in patients with larger defects. The more anterior is the perforation located, the more severe the symptoms (13). Up to 39% of patients with perforation may remain asymptomatic (9).

Chang et al. conducted a study on a group of children with septal perforation, the majority of whom complained of crusting (73%). Crusting was mainly due to dryness of the nasal mucosa secondary to turbulent airflow. In more than half of the patients, epistaxis was observed, and one third of the children complained of nasal congestion. The opposite situation usually occurs in adults, in whom epistaxis is initially observed (in 58%), and crusting follows (43%) (10).

The pathogenesis of nasal septum perforations consists of four stages:

– in the first stage, there is a local inflammatory reaction resulting in mucosal damage, erythema, edema, and crusting;

– in the second stage, a reduction in vascularization along with infiltration with inflammatory cells is observed, which leads to cartilage ischemia and loss of mucosa and submucosa;

– the third stage involves cartilage ulceration and necrosis;in the last, fourth stage, perforation borders are covered with atrophic epithelium prone to bleeding and crusting (11).

There are many causes for nasal septum perforation: trauma, surgery, tumors, coexistence of inflammatory, infectious, degenerative, and autoimmune diseases. What is more, nasal decongestants and cocaine abuse may also contribute to the formation of a perforation (4, 8).

Other causes of septal perforation include complications after embolization, foreign bodies in the nasal cavity, chronic use of nasal cannulas, and systemic diseases: sarcoidosis, systemic lupus erythematosis, tuberculosis, AIDS, Crohn’s disease, leishmaniasis, celiac disease, as well as invasive fungal sinusitis, renal failure, nicotinism, and targeted biological therapies used for treatment of malignant and non-malignant tumors (bevacizumab, methotrexate) (8). The perforation of nasal septum is most often caused by surgery, mainly septoplasty, blunt trauma, and cocaine abuse (12). In about 39% of patients, the cause of the perforation are nose and face injuries (11). Septal perforation occurs when the blood supply to the nasal cartilage is closed on both sides of the septum in approximately the same localization (9).

Damage to nasal mucosa leads to inflammation and further disintegration of the mucosa and aggravation of the infection. The final destruction of the mucosa is responsible for the loss of cartilage blood supply, which results in a perforation. Health care professionals should be aware of the risks of mucosal damage associated with medical procedures within the nose, such as cauterization. It is suspected that bilateral cauterization is more traumatic (10). Cocaine abuse may result in vasculitis, skin necrosis, lung diseases, and other conditions. Long-term cocaine abuse is also responsible for intranasal damage, including damage to the lateral wall of the nasal cavity and/or damage to the hard palate (11). The prevalence of nasal septum perforation in cocaine addicts is 4.8%. The damaging effects of cocaine on intranasal structures may result from an increased local cellular apoptosis, which is time- and dosage-dependent. In addition, the blockade of catecholamine reuptake causes vasoconstriction. Vasoconstriction may lead to mucosal damage and ulceration. Long-term ischemia causes necrosis of the nasal mucosa and septum (11). The influence of vasoconstrictive drugs on nasal septum perforation has not been sufficiently documented. Experiments on animal models using high doses of oxymetazoline showed a significant increase in the prevalence of septal perforations. Docuyuku et al. (11) observed that rats treated with high doses of oxymetazoline showed a significant increase in the incidence of ischemic lesions, hyperemia, arterial thrombosis, necrosis, and ulcerations when compared with rats receiving saline solution. These results have not been confirmed in humans.

Harmful working conditions may also contribute to the occurrence of nasal septum perforations. The prevalence of septal perforations resulting from occupational exposure to chemical substances is often underestimated. The most frequently reported damage are related to chemicals with caustic properties. Perforations have also been reported in workers exposed to toxic heavy metals, such as chromic acid. The prevalence of nasal septum perforation is this groups varies between 20 and 30% (11). Nasal septum perforation can also be caused by syphilis. During three stages of syphilis, nasal cavity may be involved to a different extent: primary syphilis is usually characterized by few nasal symptoms,in the secondary syphilis, acute rhinitis accompanied by profuse nasal secretion and irritation of nasal mucosa is observed, tertiary syphilis may manifest with an advanced nasal involvement with condylomas, septal perforations, and deformation of the nasal bridge (11).

A few cases of nasal septum perforation in patients with AIDS have been reported. Acute and chronic rhinitis may be the first symptom of infection with human immunodeficiency virus (HIV). The exacerbation of allergic rhinitis is very common in this disease. Opportunistic infections may also occur. Nasal polyps and tumors, including lymphomas and Kaposi’s sarcoma, are quite common in HIV-positive patients (11). There is a relationship between fungal sinusitis and nasal septum perforation. Fungal sinusitis usually occurs in immunocompromised patients. The disease is characterised by a progressive infiltration of blood vessels and thrombosis, followed by necrosis and tissue destruction. Patients often complain of epistaxis, facial pain, edema, and fever. The disease progresses rapidly and may result in ulceration of the mucous membrane, perforations and gangrene of the nasal septum with cranial nerve palsy, vision loss, and proptosis (11). In 60 to 90% of patients with systemic vasculitis, respiratory symptoms are observed. Over 25% of all patients report symptoms such as: crusting (69%), chronic sinusitis (61%), nasal congestion (58%), mucosal discharge (52%), epistaxis, and nasalgia. Untreated, this condition may later contribute to the destruction in the middle line of the nose. Bone destruction initially affects only nasal septum, but may gradually spread to conchae, sinuses, and other structures. Patients with a locally aggressive form of the disease may have symptoms of nasal septum necrosis, which results in the loss of support of the septum and collapse of the nasal cartilage. In 23% of patients, nasal bridge deformation occurs (11).

Among autoimmune diseases characterized by nasal symptoms, granulomatosis with polyangiitis (Wegener’s granulomatosis) seems to be the main reason for nasal septum perforations (48% of all cases of autoimmune diseases) (11). The occurrence of nasal septum perforation is common in some neoplasms, especially tumors associated with nasal cavity. Monoclonal antibody inhibitors prevent angiogenesis in the nasal septum cartilage, which may contribute to the formation of a septal perforation (11). In children, trauma and iatrogenic factors together cause over 50% of all septal perforations. Trauma usually occurs as a result of children picking their nose. Using suction devices during infections and nasogastric tubes may also contribute to the occurrence of septal perforation (10). If the cause of nasal septum perforation cannot be identified, neoplastic and autoimmune causes should be taken into account, as they are present in 20% of pediatric patients with septal perforations. The most common malignant tumors include lymphomas and leukemias. No tumors of the nasal area are observed in children (10).

There are reports in the literature concerning rare but serious complications following septoplasty, including toxic shock syndrome, endocarditis, osteomyelitis, meningitis, and cavernous sinus thrombosis. Prophylactic antibiotics are usually sufficient to prevent post-operative infections. Sometimes, however, they are ineffective. The majority of the infections are caused by Staphylococcus aureus, which is part of the normal bacterial flora of the nasal cavity in about 50% of the population. There are also reports of patients with perforations caused by other pathogens, e.g. a case report of a patient who had undergone septoplasty complicated by tissue necrosis and perforation of the nasal septum caused by Enterobacter cloacae (14). It is speculated that the frequent occurrence of S. aureus in patients with nasal septum perforation may result from the transfer of bacteria from healthcare professionals. However, this seems unlikely, as the prevalence of S. aureus among hospital staff is similar to that observed in the general population. The higher prevalence of S. aureus in patients with nasal septum perforation may be a result of a greater susceptibility of these patients to bacterial colonization and a greater tendency to the chronicity of the infection (15). The use of prophylactic antibiotics in nasal surgery is preferred by most medical doctors. However, recent studies have shown that there is still no evidence of the need for administration of antibiotics before every surgical procedure. Some researchers have not observed a significant difference between groups of patients who had or had not received antibiotics post-operatively. Therefore, they suggest that nasal surgery may not require antibiotic prophylaxis due to a low risk of infection (14). C. pseudodiphthericum and C. propioniquum are species that are frequently part of human oropharyngeal flora. Although these bacteria are characterized by a low virulence, they may contribute to poor clinical outcomes, especially in patients with immunodeficiencies and endocarditis. An inverse relationship between the prevalence of Corynebacterium and S. aureus have also been described, as severe colonization with Corynebacterium prevents colonization with S. aureus (16). In patients who inhale cocaine nasally, nasal septum perforations with colonization with anaerobic bacteria is most often caused by ideal environmental conditions, which combine a reduced amount of oxygen, inflammation, irritation, and open wound. Staphylococcus aureus is a common bacterium occurring in this group of patients (17).

Conducting the diagnostic process in patients with nasal septum perforation is necessary for detecting causes of the problem (8). Patients usually report to a specialist when the first symptoms occur (18).

The assessment of a patient with nasal septum perforation includes:

– detailed medical history,

– physical examination,

– diagnostic and laboratory tests (11).

Collecting medical interview aims to gather information on previous surgeries and nasal intubations, nasal injuries, nose picking habits, irritants in the work environment, the use of intranasal drugs, and the co-occurrence of systemic diseases or neoplasms. Physical examination of the nose starts with an assessment of the external nose (8). Physical examination should include a full assessment, including endoscopic examination of the sinuses (11). Performing endoscopic examination may be helpful in the visual assessment of nasal septum perforation, as well as in assessing the irregularity of the mucosal structure (8). It is also advised to evaluate and measure the remains of cartilaginous and bone support (11).

Imaging examinations are an extremely important element of the diagnostic process and are performed both before and after surgery to obtain as much information about the structure of the nose as possible, as well as to determine the degree of damage to bone and mucosa. This is necessary for planning the reconstruction surgery and maximizing the diagnostic and therapeutic efficacy (8).