caso clínico reconstrucción de la deformidad nasal en

www.sgorl.org Acta nº13 - 2020168

Caso Clínico Reconstrucción de la deformidad nasal en

silla de montar con cartílago costal autólogo en la Granulomatosis con Poliangitis.

Saddle nose deformity reconstruction with autologous costal cartilage in Granulomatosis

with Polyangiitis.

Miguel Mayo-Yáñez* (1,2), Juan Cabrera-Sarmiento (1), Alejandro Martínez-Morán (1)


ISSN: 2340-3438

Edita: Sociedad Gallega de Otorrinolaringología

Periodicidad: continuada.

Web: www.sgorl.org/ACTA

Correo electrónico: [email protected]

Centro de trabajo

(1) Otorhinolaryngology – Head and Neck Surgery Department, Complexo Hospitalario Universitario A Coruña (CHUAC),

15006, A Coruña, Galicia, Spain.

(2) Clinical Research in Medicine, International Center for Doctorate and Advanced Studies (CIEDUS),

Universidade de Santiago de Compostela (USC), 15782, Santiago de Compostela, Galicia, Spain.

Correspondencia* Otorhinolaryngology – Head and Neck Surgery Department,

Complexo Hospitalario Universitario A Coruña (CHUAC). As Xubias 84, 15006, A Coruña, Spain.

[email protected]

Fecha de envío: 13/2/2020 Fecha de aceptación: 21/2/2020

Acta Otorrinolaringol. Gallega 2020; 13: 168-181


ResumenLa Granulomatosis con Poliangitis es una vasculitis necrotizante idiopática que afecta predominante-

mente al sistema respiratorio superior. La deformidad en la silla de montar ocurre en el 10% al 25% de los pacientes y la obstrucción del conducto nasolagrimal en el 7% de los pacientes. Presentamos un caso de un paciente con granulomatosis con poliangitis intervenida de septorrinoplastia y dacriocistorrinostomía endoscópica para corregir estas dos complicaciones, lo que generalmente requiere reemplazo del soporte dorsal y columelar y puede estar limitado por déficits en el revestimiento nasal. La reconstrucción nasal abierta y la cirugía endoscópica lagrimal en pacientes afectados por Granulomatosis con Poliangitis parece ser segura y efectiva si la enfermedad está en remisión antes de cualquier procedimiento.

Palabras clave

Granulomatosis con Poliangitis; Deformidad en silla de montar; Septorrinoplastia; Dacriocistitis



AbstractGranulomatosis with Polyangiitis is an idiopathic necrotizing vasculitis which predominantly affects the

upper respiratory system. Saddle nose deformity occurs in 10% to 25% of patients and obstruction of the nasolacrimal duct in 7% of patients. We present a case of a patient with Granulomatosis with Polyangiitis intervened of septorhinoplasty and endoscopic dacryocystorhinostomy to correct these two complications, what usually requires replacement of dorsal and columellar support and may be limited by deficits in the nasal lining. Open nasal reconstruction and lacrimal endoscopic surgery in patients affected by granulo-matosis with polyangiitis seems to be safe and effective if the disease is in remission before any procedure

Keywords

Granulomatosis with Polyangiitis; Saddle nose deformity; Septorhinoplasty; Dacryocystitis



IntroductionGranulomatosis with Polyangiitis (GP) is an idiopathic necrotizing vasculitis with an incidence of 50-

100 per million population in Europe and predominately affects the upper respiratory system, kidneys and lungs (1). Although not absolutely specific, severe nasal crusting, nasal septal perforation, chronic dacryo-cystitis, saddle nose deformity and/or subglottic stenosis are suggestive of GP.

Saddle nose deformity occurs in 10% to 25% of patients, quite rapidly and chiefly during the early phases of the disease (2). However, it is not pathognomonic for GP, it is a well-known stigma of patients affected by this disease. On the other hand, ophthalmic involvement in this disease is frequent, finding obs-truction of the nasolacrimal duct in 7% of patients (2,3).

The surgical treatment of saddle nose deformity or chronic dacryocistitis resulting from autoimmune conditions such as GP is technically challenging. We present a case of a patient with GP intervened of sep-torhinoplasty and endoscopic dacryocystorrinostomy to correct these two complications.



CaseA 44-year-old woman, diagnosed and in complete remission of GP, presented a stage 3 saddle nose

deformity (Figure 1A & B) accompanied by a chronic left nasolacrimal obstruction with recurrent dacryo-cystitis. The endoscopic surgery was complicated, since the nasal mucosa was very friable, with a tendency to bleeding and the different anatomical repairs were unstructured. Despite this, the tear duct was canalized and the silicon probe could be placed without added complications (figure 2).

For the restoration of the nasal frame an open rhinoplasty approach was chosen with a costal cartilage L-strut. Using an inverted V incision of the columella combined with bilateral marginal incisions, the dorsal skin was elevated and a space dissected to fit the long leg of the cartilage strut (figure 3). A residual carti-laginous septum was found and dissected to obtain local support for the distal part of the L-strut (figure 4). After these steps, the desired length and height of the strut was defined.

A slightly curved incision in the submammary fold provided access to the seventh rib. After incision of the fascia and splitting of the overlying rectus and serratus anterior muscle, the desired size and shape of the L-strut was outlined with a surgical marker, after which the cartilage was incised and extracted (figure 5A). The donor site was closed in layers, with careful approximation of the tissue to avoid obvious scarring.

The strut then was carved in multiple steps to generate 2 pieces that fit in the space provided (figure 5B). The distal part of the short leg of the L-strut was inserted into a pocket created close to the bony nasal spine and fixed with another 6-0 Prolene® suture to periosteum of the nasal spine. It was then sutured to the long part anchored to the nasal dorsum to complete the L-graft (figure 6A). The medial crura of the alar cartilage was fixed to the short leg of the L-strut using 4-0 Vycril U-sutures (figure 6B). An inter and intradomal su-tures were applied along with a cap graft to improve the projection of the nasal tip (figure 7A & B).

Finally, the skin was redraped, the cartilage was controlled for irregularities and diced gel cartilage te-chnique was used to correct residual sinking (figure 8). After closure of the nasal skin at the columella with 6-0 Prolene® and the mucosa with 5-0 Vicryl (figure 9), skin tapes and a plaster were applied. The Prolene stitches were removed on day 6 and the plaster after 14 days and the surgery was functional and aesthetica-lly successful, presenting no postoperative complications in either procedure. After one year of follow-up, the patient remains satisfied with the aesthetic and functional result (figure 10A & B), as well as without presenting exacerbations of her underlying disease at the naso-ophthalmic level.



DiscussionSinonasal involvement is the most common manifestation of GP in the head and neck, occurring in up to

85% of patients (4). Rhinitis with a granulomatous or “cobblestone” appearance is almost always present, accompanied by nasal dripping, obstruction, congestion, crusting and often epistaxis. Sinusitis, with sinus pain, is frequently erosive and/or leads to sinus atrophy over time. Anosmia or hyposmia is a frequent com-plaint of GP patients.

Nasolacrimal duct obstruction is a late finding and can be either secondary to inflammatory spread from adjacent sinonasal disease, or a direct result of focal GP inflammation. As a result of the nasolacrimal bloc-kage, dacryocystitis and epiphora can occur (3). Surgical management has limited effectiveness, as there is a possibility of relapse, although it is helpful in severe situations. In cases of nasolacrimal duct obstruction, dacryocystorhinostomy with the creation of a new outflow to bypass the obstruction may be required.

The saddle nose deformity is a well-known stigma of patients affected by GP, representing one of the most challenging nasal deformities for the surgeon. The patient affected has almost always psychological problems, including concerns related to their own identity. The surgeon should evaluate the patient from different points of view, including diagnostic, therapeutical, and psychological. The choice of the correct approach should be rational and based on the severity of the deformity. (5,6)

The treatment of saddle-nose deformity and its associated symptoms falls into two categories: sympto-matic and definitive. Conservatively, treatment for nasal crusting can be initiated with nasal saline rinses, topical steroids, mucolytics, and emollients which may be complemented with minor surgical operations including polypectomy and mucosal-sparing techniques. The only definitive treatment is surgical correction via nasal reconstruction (7). Reconstruction of these defects usually requires replacement of dorsal and co-lumellar support and may be limited by deficits in the nasal lining. The use of auricular, conchal or costal cartilage for grafting seems to be successful when performed with the disease in remission.

Open nasal reconstruction and lacrimal endoscopic surgery in patients affected by granulomatosis with polyangiitis seems to be safe and effective if the disease is in remission before any procedure. Therefore, nasal reconstruction could improve the physical appearance, as well as the psychological well-being and quality of life of these patients seems (7,8).



DisclosuresThere was no conflict of interest and authors have nothing to declare. Informed consent was obtained

from all individual participants included in the study.



References1. Pagnoux C, Wolter NE. Vasculitis of the upper airways. Swiss Med Wkly. 2012;142:w13541.

2. Cannady SB, Batra PS, Koening C, Lorenz RR, Citardi MJ, Langford C, et al. Sinonasal Wegener granulomatosis: a single-institution experience with 120 cases. Laryngoscope. 2009;119(4):757-61.

3. Sfiniadaki E, Tsiara I, Theodossiadis P, Chatziralli I. Ocular Manifestations of Granulomatosis with Polyangiitis: A Review of the Literature. Ophthalmol Ther. 2019;8(2):227-34.

4. Rasmussen N. Management of the ear, nose, and throat manifestations of Wegener granulomatosis: an otorhinolaryngologist’s perspective. Curr Opin Rheumatol. 2001;13(1):3-11.

5. Matteo MT. Saddle Nose: A Systematic Approach. Contemp Rhinoplasty - Whats New [Internet]. 10 de diciembre de 2018 [Accessed: October 2019; cited October 2019]; Available in: https://www.in-techopen.com/online-first/saddle-nose-a-systematic-approach

6. Durbec M, Disant F. Saddle nose: classification and therapeutic management. Eur Ann Otorhi-nolaryngol Head Neck Dis. 2014;131(2):99-106.

7. Erickson VR, Hwang PH. Wegener’s granulomatosis: current trends in diagnosis and management. Curr Opin Otolaryngol Head Neck Surg. 2007;15(3):170-6.

8. Vogt PM, Gohritz A, Haubitz M, Steiert A. Reconstruction of nasal deformity in Wegener’s granu-lomatosis: contraindication or benefit? Aesthetic Plast Surg. 2011;35(2):156-61.

9. Ezzat WH, Compton RA, Basa KC, Levi J. Reconstructive Techniques for the Saddle Nose Deform-ity in Granulomatosis With Polyangiitis: A Systematic Review. JAMA Otolaryngol. 2017;143(5):507-12.



Figures1. A) Frontal view of saddle nose deformity in stage 3. B) Lateral view of nasal deformity with complete

loss of the nasal dorsum and projection, and increase of the nasolabial angle.

2. Endoscopic vision of the left fossa in which the difficulty of the surgery can be seen, with minimal anatomical references and a blood-stained surgical field. The * marks the head of the middle turbinate rem-nant. The arrow indicates the silicone probe coming out of the area where the lacrimal sac would be.



3. Approach of open septorhinoplasty with elevation of nasal dorsum skin to region of nasal bones.

4. Subperichondrium approach of the septal region where existing remnants of cartilage can be seen.



5. A) Costal graft obtained. B) Carved rib graft.

6. A) Strut columelar between the cruras. B) L-strut shaped and fixed after placement of costal graft of the dorsum.



7. A) Domal suture to improve the projection. B) Cap graft

8. Diced gel cartilage for nasal dorsum corrections

9. Closure of inverted “V” and marginal incision.



10. A) Front view of the result after one year of follow-up. B) Lateral view of the result after one year of follow-up, where a clear improvement of the nasal dorsum can be seen.

caso clínico reconstrucción de la deformidad nasal en

Documents