Saddle-nose deformities in the rheumatology clinic.

Saddle-nose deformity can occur as a result of trauma to the nose, but it has also been well described in the setting of infections such as leprosy and syphilis and idiopathic inflammatory conditions such as granulomatosis with polyangiitis (formerly known as Wegener granulomatosis) and relapsing polychondritis. Since these deformities may also arise without an evident precipitating cause, they can pose a diagnostic conundrum. We review 2 cases of saddle-nose deformity that were treated at Northwick Park Hospital in Middlesex, England. The first patient was a 53-year-old woman who presented with epistaxis and deviation of the nasal septum. She subsequently developed a saddle-nose deformity and a septal ulcer. An autoimmune screen was negative, and histologic findings were nonspecific. She underwent successful reconstruction with a polyethylene implant. The second patient was a 21-year-old woman who presented with nasal obstruction and a nasal septal deviation. Two years later, she was diagnosed with Crohn disease and treatment with azathioprine was commenced. Eventually, the cartilaginous dorsum of her nose collapsed. A biopsy of the area revealed nonspecific, active, chronic inflammation. A polyethylene implant was placed to correct the deformity, but part of the implant became dislodged, and revision surgery was not successful. A subsequent revision was performed, and the early results were encouraging. Saddle-nose deformity may be a manifestation of underlying connective tissue disease, so it is important to detect and treat any such condition before embarking on surgical repair of the deformity. Our 2 cases indicate that this very deforming condition is poorly understood and treatment can be unsatisfactory.

[Nasal surgery in patients with systemic disorders]. / Rhinochirurgie bei Systemerkrankungen.

Systemic disorders represent a heterogenous group of diseases which can primarily manifest at the nose and sinuses as limited disease or secondarily as part of the systemic involvement. Rhinologists therefore play an important role in the diagnostic but also therapeutic process. Although therapy of systemic disorders is primary systemic, additional nasal surgery may become necessary. Surgical procedures include sinus surgery for the treatment of complications of the orbit and the lacrimal duct, septorhinoplasty due to saddle nose deformity and closure of septal perforation. Since many systemic disorders represent very rare diseases, recommendations are based on the analysis of single case reports and studies with a limited number of patients. Even though data is still limited, experiences published so far have shown that autogenous cartilage or bone transplants can be used in nasal reconstruction of deformities caused by tuberculosis, leprosy, wegener's granulomatosis, sarcoidosis and relapsing polychondritis. Experiences gained from these diseases support our observation that well-established techniques of septorhinoplasty can be used in systemic disorders as well. However, reaching a state of remission is an essential condition before considering any rhinosurgery in these patients. Under these circumstances revision surgery has to be expected more frequently compared to the typical collective of patients undergoing septorhinoplasty. These observations in part may also be useful for the treatment of nasal septal perforations since implantation of cartilage- or bone grafts represents an essential step in the closure of septal perforations. Apart from the treatment of orbital complications, sinus surgery has been proven beneficial in reducing nasal symptoms and increasing quality of life in patients refractory to systemic treatment.

Reconstructive surgery of the leprosy nose: a new approach.

There has still been no reduction in the detection rate worldwide for leprosy, despite supervised multi-drug therapy. In time, leprosy can result in a severe saddle-nose deformity leading to functional problems, disfiguration and stigmatization. In severe cases, only the nasal skin tissue and the lower lateral cartilages are preserved. In such cases, the ideal would be to restore the cartilaginous skeleton but, by contrast with other causes of saddle-nose deformities, this is complicated by the quantity and the poor quality of the remaining nasal mucosa. Leprosy-related saddle-nose deformities are therefore challenging and difficult to reconstruct with the techniques that have been proposed in the past. In this study, 24 patients underwent rhinoplastic surgery involving the use of autogenous costal and/or auricular cartilage or composite grafts. The nasal septum, the upper laterals and the anterior nasal spine were reconstructed with a dorsal onlay attached to a columellar strut with an extension on the proximal side. Before surgery, the saddle-nose deformities were classified according to severity with a new system based on clinical symptoms and signs. Postoperative evaluation was performed at least two years after surgery (N=17). Functional and aesthetic improvement, resorption rate, warping, infection and extrusion were analysed. Functional and aesthetic improvements were achieved in 15/17 patients. None of the patients developed an infection and extrusion or warping of the implants was not observed. The resorption rate depended on the localization and the type of cartilage implant. In general, auricular conchal cartilage implant grafts resulted in less resorption than costal cartilage. Least resorption (4/17 patients) was observed in the dorsal onlay grafts of both conchal (1/6) and costal cartilage grafts (3/11). Resorption of columellar strut implants and shield grafts was observed in 7/17 patients. No resorption was seen of composite grafts (0/4) and alar battens (0/7). Autogenous cartilage implants can be used to reconstruct saddle-nose deformities in leprosy with a minimum risk of complications. The preoperative grade of severity was used as a basis for the development of guidelines for optimal long-term functional and aesthetic outcome.

A rational approach to nasal reconstruction in leprosy.

Destruction of the nasal septum and nasal bones by Mycobacterium leprae and subsequent infection is still seen regularly in leprosy endemic areas. The social stigma associated with this deformity is significant. Many different procedures have been developed to reconstruct the nose. Patients operated on at Anandaban Hospital and the Green Pastures Hospital and Rehabilitation Center between 1986 and 2001 were reviewed. There were 48 patients with an average age of 47 years. Five deformities were mild, 22 were moderate, 13 were severe, and eight were not graded. Bone grafting with nasolabial skin flaps was performed in 14 cases, bone grafting alone was performed in 10 cases, flaps alone were performed in seven cases, and cartilage grafting was performed in 10 cases. In three patients, a prosthesis was inserted, and in three patients a gull-wing forehead flap was performed. Overall, excellent or good cosmetic results were obtained in 83 percent of cases. Grafting with conchal cartilage was associated with the best cosmetic results and had minimal complications. Bone grafting with or without nasolabial flaps was associated with a 50 percent complication rate of infection or graft resorption. In mild to moderate deformities, cartilage grafting is recommended; for more severe deformities, bone grafting with bony fixation and skin flaps is recommended. Perioperative antibiotics must be used, and these procedures should be performed by an experienced surgeon. In very severe cases with skin deficiency, reconstruction with a forehead flap gives good results.

Saddle nose: our approach to the problem.

Saddle nose is usually caused by a trauma or by excessive resection of the septal cartilage. Nevertheless, there are other, less frequent causes of injury, such as congenital, syphilis, leishmaniosis, and leprosy. Within this context, it is very likely to see widening of the bony bridge and dropping of the tip of the nose. For this clinical status, we found extremely satisfactory a therapy in which we use a dorsum cartilage graft, followed by narrowing of the nasal bridge and shortening of the nose. To achieve this aim, different kinds of materials were employed. The authors usually prefer rehydrated (0.9% saline solution) human costal cartilage. This material was used in a study of a series of patients with saddle nose in which we used open rhinoplasty and cartilage homografts.

[Nasal reconstruction with reference to the nose in leprosy]. / Nasenrekonstruktion unter Berücksichtigung der Lepra-Nase.

For the reconstruction of the nostril a nasolabial flap is recommended. According to the method of Pers (1967), the upper part of the flap is used for lining and the lower part for the outside coverage of the defect. In order to avoid lateral traction on the nostril, the author recommends that a small triangular flap with an inferior pedicle is left between the nasolabial flap and the nostril. For total reconstruction of the nose, a frontal flap with a primary cantilever bone graft as described by Millard (1966) is suggested. We advise to take one half of forehead skin. This gives a less obvious donor site and enough length in the diagonal direction for the dorsum of the nose and the columella. For the leprotic nose it is emphasized that no skin loss is present, there is only a loss of lining and support. In all advanced cases a large septal defect is encountered. The reliable postnasal inlay of Gillies is mentioned but the draw back to this method is that the care of the postnasal prosthesis may be difficult for leprosy patients with disabled hands. Secondary bone grafting after this procedure has a high failure rate because of infection. For these reasons the reconstruction of lining by two nasolabial flaps according to Farina (1957) is described. The author has regularly used this method with a primary cantilever graft. A modification is again suggested. A small triangular skin flap is raised with the ala, thus avoiding lateral traction on the nostrils after closure of the donor site.

Reconstructive surgery of facial deformities in leprosy.

Surgical techniques for correction of the facial deformities of leprosy are discussed. It is urged that surgery be undertaken only on patients who have had a negative smear for at least two years.