Rhinosurgery: Rhinosurgery in RP is based on a limited number of case reports only. Rapini refused reconstruction of saddle nose deformities in RP because of the relapsing character of the disease. By contrast, Haug and coworkers successfully reconstructed a saddle nose using iliac crest. A bony implant was chosen because the authors considered it more resistant and less resorpable compared with cartilage. Torossian and coworkers reconstructed a saddle nose deformity in a young patient using calvarial bone graft. The operation was performed 12 months after establishing the diagnosis of RP and in a state of stable remission. Experiences reported by Bell and coworkers argue against a theoretical superiority of bone implants. Usage of cartilage implants is possible in RP if relapsing episodes of nasal chondritis are prevented or reduced to a minimum by immunosuppressive therapy. Bell and coworkers used autologous costal cartilage to reconstruct a saddle nose in 14 years old patient. Although two more episodes of chondritis occurred no signs of resorption of the cartilaginous implant was observed one year after the operation.
Epidemiology/pathogenesis: Systemic Lupus erythematosus (SLE) is an autoimmune disorder with potential manifestations at nearly all organs and tissues of the human body. The disease predominantly involves females. An annual incidence of 5/100,000 in Germany has been reported. Oral and nasopharyngeal ulceration are considered as diagnostic criteria for SLE.
Nasal symptoms: Nasal symptoms are generally unspecific such as mild episodes of epistaxis or local tenderness or facial pain. The nasal mucosa may appear diffusely reddish and edematous or as atrophic rhinitis. Less than 70 cases of nasal septal perforation have been reported so far worldwide. Nasal septal perforation is even more rarely observed as a first symptom. In a retrospective study of the lupus clinic of the University of Toronto nasal septal perforation was discovered in 40/885 (4.6%) patients with SLE. These perforations usually remain asymptomatic since they are detected after a course of 6.1 years in the mean. In addition, nasal septal perforations occur during exacerbation of the disease or in the context of vasculitis. Oral ulceration often precedes nasal septal perforation. 
Epidemiology/pathogenesis: Crohn's disease (CD) is an inflammatory bowel disease and can involve all parts of the gastrointestinal tract. An annual incidence of 5.2/100,000 in Germany has been reported. Histopathology reveals non-caseating granuloma as a valuable diagnostic sign that however is detectable in 10% of the cases only. Extraintestinal spread has been observed. Aside from the oral cavity, the nose and the larynx may be involved in the head and neck region,,. Nasal involvement is extremely rare since less than 15 cases have been reported throughout the world so far,,,,,,,,.
Nasal symptoms: The spectrum of nasal symptoms consists of nasal obstruction, rhinorrhea, hyposmia, epistaxis and complaints of sinusitis. The nasal mucosa may appear diffusely reddish and hypertrophic and easily starts bleeding. Erosion, ulceration and necrosis of the septum or inferior turbinates may be visible,,,,. In addition, nasal septal perforation and saddle nose deformity have been reported,,. It is worth to mention that nasal symptoms may precede gastrointestinal symptoms. An association with WG and RP has been observed. Book and coworkers retrospectively analyzed 160 patients suffering from inflammatory bowel disease. They observed chronic sinusitis particularly in those CD patients (23%) who suffered from obstructive bowel complications, whereas rhinosinusitis was observed only in 7% of patients without obstructive bowel complications.
Rhinosurgery: To our knowledge reconstruction of saddle nose deformities or closure of nasal septal perforations has not been discussed in the literature so far. Partial turbinate resection has been reported. Sinus surgery may become necessary to reduce sinonasal symptoms and to control orbital complications.
Epidemiology/pathogenesis: Aside from CD ulcerative colitis (UC) belongs to the group of inflammatory bowel disease. An annual incidence of 6/100,000 in Germany has been reported. Although aetiology of UC still remains unclear, an autoimmunepathogenesis has been suggested. An association of UC with chronic sinusitis has been hypothesized in some patients.
Nasal symptoms: Nasal septal perforation is a rarity.
Epidemiology/pathogenesis: Heriditary hemorrhagic telangiectasia (HHT, Osler's disease) is an autosomal dominant-disease of angiogenesis. The underlying genetic aberrations are associated with formation of endothelial-lined vascular lakes and dilatated vessels. Because the vessel's wall structure is abnormal due to the lack of muscular or elastic support rupture of these vessels already occurs by small trauma. This can lead to substantial bleeding although coagulation factors and platelets are normal because pathologic vessels do not retract or undergo vasospasm. Currently, an incidence of 1/10,000 has been reported.
Nasal symptoms: Quality of life is of HHT patients is mainly determined by recurrent episodes of epistaxis,, although arteriovenous malformations of the gastrointestinal tract, lungs, liver and brain are often associated with higher morbidity and mortality. A variety of treatment options to control epistaxis in HHT patients are available. These include topical therapies, hormonal therapies with estrogens, laser coagulation as well as argon-plasma coagulation,,,,. Argon-plasma coagulation, bipolare coagulation as well as anterior and/or posterior nasal packing represent procedures to control acute episodes of epistaxis. Severe epistaxis can be controlled by interventional radiological embolization, and surgical arterial ligation.
Rhinosurgery: Recently, new data have been published analyzing long-term results and quality of life in HHT patients treated by septodermoplasty or nasal closure.
The main principle of septodermoplasty (SDP) is resection of diseased mucosa of septal areas II and III and implantation of an epidermal graft. Elimination of easily bleeding mucosal areas leads to a reduction of the frequency of bleeding within the first two years. After two years however, new formation of telangiectatic vessels dorsal to the epidermal graft is often observed. As a consequence, the frequency of bleeding increases again. Moreover, access to the bleeding vessel then is usually difficult. These arguments have been considered as major points of criticism of this method.
By contrast, recently published studies emphasize the benefit of this method. Levine and coworkers retrospectively analyzed (mean follow-up 3.75 years) 50 HHT patients and found that 86% of patients reported increased quality of life following septodermoplasty although they also complained about impaired sense of smell (78%), crusting (72%), hyposmia (58%) and symptoms of sinusitis (30%). Harvey and coworkers reported on a collective of 131 HHT patients who were followed up for 60 months. Overall 268 KTP laser procedures were performed. In addition, 33 patients underwent SDP, which was significantly associated with a reduced number of laser procedures postoperatively. Fiorella and coworkers analyzed retrospectively 67 patients who received SDP. Again, a majority of patients (57/67) reported a higher quality of life. In addition, the authors found a significant reduction of blood transfusions with 12 months postoperatively compared with the 12 months period before the operation. Recently, a modification of SDP has been performed by Lesnik. He resected the remaining nasal septum and then performed SDP in nine patients. An increase of quality of life was observed as well as a reduction of the need of blood transfusion.
Taylor and Young introduced the method of permanent closure of the nasal nostrils in 1961. The aim of this procedure is to prevent dryness and traumata of the nasal mucosa thereby reducing the frequency of bleeding. Young himself reported about the problem of permanent breathing through the mouth and re-opened the nasal cavity in five of his patients. Nevertheless, permanent nasal closure may be an option in refractory cases as suggested by several authors,,. Hitchings and coworkers performed a modified Young's procedure in eight patients with sever bleeding episodes. Complete cessation of bleeding was observed in 88% of patients (mean follow-up 22 months). A detailed analysis revealed that reduction of episodes of bleeding was associated with increased quality of life which was considered even more important than permanent nasal obstruction.
Overall, SDP and Young's procedure are options for HHT patients with recurrent severe episodes of epistaxis refractory to local therapies and laser coagulation since in these patients reduction of bleeding obviously determines quality of life more importantly than nasal obstruction.
Rhinosurgery in patients with systemic diseases may become necessary to reconstruct saddle nose deformities and nasal septal perforations. Sinus surgery plays a role in patients with orbital and intracranial complications. In addition, sinus surgery may be useful in some patients to reduce sinonasal symptoms thereby improving quality of life (Table 1 (Tab. 1)).
Extended diagnostic is mandatory if an underlying systemic disease is suspected and in cases of aetiological unclear saddle nose deformity or nasal septal perforation to prevent failure of rhinosurgery in such patients. Furthermore, non-reflected rhinosurgery may camouflage the symptoms saddle nose or nasal septal perforation as manifestations of a yet localized systemic disease. Thereby, the chance to prevent a potential fatal systemic disease by early treatment could be missed or delayed.
Because of the rarity of numerous systemic diseases recommendations on the treatment of nasal deformities caused by systemic diseases are based on single case reports or case series only. Therefore, no specific rhinosurgical techniques have been established so far. However, according the experiences published cartilage and bone grafts haven been implanted with success in stable remission in patients suffering from tuberculosis, leprosy,, Wegener's granulomatosis,,,, sarcoidosis and relapsing polychondritis,.
Experiences gained from these patients suggest that traditionally-proven surgical techniques of rhinoplasty,, can also be applied in sytemic diseases as far as the underlying disease has been successfully treated previously. Rhinosurgery is performed ideally in a state of complete remission meaning during complete absence of clinical, serologic and radiologic signs of disease. Nevertheless revision surgery has to be expected more frequently compared to a normal collective of rhinoplasties. The question whether cartilage or bone grafts are associated with equal outcomes has not been answered so far since outcome also depends several factors including stage and severity of the underlying disease.
In WG costal cartilage grafts were associated with a better transplant success rate (83%) compared with bone grafts (calvarial bone graft; transplant success rate 75%). However, Shipchandler and coworkers did not observe complications or resorption in four cases of WG and in one case of sarcoidosis associated saddle nose deformity treated by implantation of a split calvarial bone L-shaped strut. Bone grafts were advocated because of greater availability and less resorption which, however, is not corroborated by the studies of Congdon and coworkers.
In general, infection and resorption is rarely observed following implantation of autologous cartilage,. In addition, cartilage grafts display favourable biomechanical properties particularly if used in the tip area. Although alloplastic materials are available in almost unlimited amounts, usage is associated with a high risk of infection and extrusion,,. Therefore, autologous cartilage is the material of first choice in the reconstruction of saddle nose deformities,,,,. According to the data presented in this review this statement seems to be valid for some systemic diseases as well,,,,.
In the case of nasal septal perforation caused by systemic diseases recommendations on closure of perforations are only theoretical due to missing clinical data. In general, nasal septal perforation occurs as complication following septal surgery, trauma or because of other factors,,,,,. Aside from these factors systemic diseases can be associated with nasal septal perforation. In many cases series and reviews retrospectively analyzing the outcome of different techniques for closure of nasal septal perforation, included patients were always free of any systemic disease. According to such a selection of patients it can be assumed that active systemic disease is a contraindication for closure of nasal septal perforation.
Closure of asymptomatic nasal perforations is not indicated. In the case of active systemic disease that cannot be controlled implantation of a septal button is an alternative option,. In the case of surgical closure the operation is only performed if the mucosa has been fully rehabilitated,.
Since implanation of autologous cartilage or bone grafts is also an important step in most multilayer techniques for closure of nasal septal perforation, experiences gained from reconstructive surgery of saddle nose deformities should be considered. It is essential to identify, eliminate or at least diminish the aetiological factor causing a nasal septal perforation,,. Provided that there are optimal conditions the same surgical techniques that have been proven successful in the closure of iatrogenic or traumatic nasal septal perforations can be applied in systemic diseases. According to our personal concept surgical approach and technique are dependent on the localization and the size of a perforation (Figure 6 (Fig. 6)). Small perforations (type A) can be closed by exchange of cartilage as demonstrated in HIV positive patient (Figure 2 (Fig. 2)). Type B perforations can be closed using bipedicled gingivobuccal flaps (Figure 7 (Fig. 7)). Larger perforations (type C) can be closed by cartilage implant and bilateral inferior turbinate flaps as demonstrated in a patient with WG in remission (Figure 4 (Fig. 4)).
In general, successful closure of nasal septal perforation has been observed if a multilayer technique was applied. These techniques have the following steps in common: a) extensive subperichondrial/periosteal dissection and mobilization of the nasal mucosa from the remaining septum, nasal floor and root, b) tension-free closure of mucosal borders, c) interposition of a connective tissue graft such as temporal muscle fascia, periosteum, cartilage grafts with perichondreum or a combination thereof,,,,,.
Overall, reconstruction of nasal deformities and closure of nasal septal perforation associated with systemic diseases has to be always decided in consideration of the type of the disease, severity of the disease, frequency of recurrences and prognosis. Ideally, patients should be in complete remission supported by excellent compliance and long-term medication.