Neurofibromatose tipo 1 com acometimento do nervo infraorbital: relato de caso / Neurofibromatosis type 1 with infraorbital nerve involvement: a case report

A neurofibromatose tipo 1 é uma doença autossômica dominante rara, com manifestações clínicas diversas. Sua apresentação mais marcante é a presença de neurofibromas (tumores da bainha neural) cutâneos ou internos, que também podem ocorrer de forma esporádica, associados a outras manifestações sistêmicas, como manchas café com leite e lesões oculares. Por serem tumores da bainha de mielina, os neurofibromas podem acometer diversos nervos periféricos, incluindo nervos da face. Apresentamos o caso de um paciente de 1 ano, portador de neurofibromatose tipo 1, com neurofibroma em nervo infraorbital direito, com o acesso proposto para tratamento cirúrgico que fornecesse ampla visualização e acesso a lesão, sem comprometimento estético importante, permitindo preservação de partes moles e adequado crescimento facial.

Neurofibromatosis type 1 (NF1) is a rare autosomal dominant disease with multiple clinical manifestations. Its most significant presentation is cutaneous or subcutaneous neurofibromas (myelin sheath tumors), which may be associated with other systemic manifestations such as caféau- lait spots and eye involvement. Neurofibromas can affect several peripheral nerves, including the facial nerves. This report presents a case of a 1-year-old patient with NF1 with right infraorbital nerve neurofibroma in which the proposed access for surgical treatment allowed adequate visualization of the tumor with good aesthetic results, preservation of the soft tissues, and normal facial growth.

Retalho interpolado de sulco nasogeniano em único estágio para reconstrução de defeitos nasais e de canto interno da órbita / Single-stage nasolabial interpolation flap for reconstructing defects of the nose and inner corner of the eye

Introdução: Os retalhos interpolados são opções cirúrgicas eficazes para reconstruções de defeitos cutâneos em várias áreas do corpo, inclusive na face. O retalho proposto dispensa cuidados pós-operatórios com o pedículo exposto e pode ser realizado em tempo único. O objetivo é avaliar a utilidade do retalho interpolado de sulco nasogeniano (RISN) em ilha, na reconstrução de segmentos nasais e do canto interno da órbita, bem como discutir refinamentos em seu design e execução. Métodos: Estudo retrospectivo de prontuários de pacientes com defeitos nasais ou de canto interno da órbita, e que foram reparados com retalho interpolado do sulco nasogeniano. Todos os retalhos foram confeccionados de maneira randômica, realizando-se túnel subcutâneo para evitar pedículo exposto e cicatriz que comunicasse a área doadora e o defeito. Resultados: cinco pacientes foram incluídos no estudo, com idade entre 30 e 92 anos. Em todos os casos foi realizada biópsia de congelação intraoperatória que revelou margens livres de doença, orientando a extensão da ressecção. O CBC foi encontrado em 4 pacientes e o CEC em um paciente. Não houve complicações como sangramento pós-operatório ou necrose. Bons resultados funcionais e estéticos foram alcançados em todos os pacientes. Discussão: Vale ressaltar a versatilidade do retalho nasogeniano interpolado, sendo capaz de auxiliar na reconstrução de defeitos extensos não apenas de asa, ponta e columela nasais, mas também de dorso e canto medial do olho. Destaca-se também o aspecto estético mais favorável do pedículo do retalho interpolado em ilha comparado ao de transposição. Conclusão: O RISN interpolado em único estágio é uma opção confiável na reconstrução de segmentos faciais. Apresenta boa vascularização, possibilidade se ser realizado em único tempo e pode ser utilizado para cobertura nos locais onde há poucas opções reconstrutivas disponíveis.

Introduction: Interpolation flaps are effective surgical options for reconstructing skin defects in various areas of the body, including the face. The proposed flap does not require postoperative care with the pedicle exposed and can be performed in a single surgery. The objective is to evaluate the usefulness of the nasolabial interpolation island flap (NIF) for reconstructing nasal segments and the inner corner of the eye, as well as discuss improvements in its design and performance. Methods: In this retrospective study, medical records of patients with nasal defects that were repaired with a nasolabial interpolation flap were reviewed. All flaps were created with a subcutaneous tunnel to avoid pedicle exposure and prevent scar connection with the donor area and the defect. Results: Five patients aged 30­92 years were included. In all cases, intraoperative frozen biopsy revealed disease-free margins, indicating the extent of the resection. Basal cell carcinoma was found in four patients and squamous cell carcinoma in one. There were no complications such as postoperative bleeding or necrosis. Good functional and aesthetic results were achieved. Discussion: The NIF can help in the reconstruction of extensive defects of the nasal ala, tip, columella, and medial dorsum as well as the corner of the eye. We also highlight the more favorable aesthetic aspect of the pedicle in the interpolation island versus transposition flap. Conclusion: The single-stage NIF flap is a reliable option for reconstructing facial segments as it has good vascularization, can be performed in a single surgery, and can be used to cover places where few other reconstructive options are available.

Eye socket reconstruction in patients with severe depressed eye socket combined anophthalmos / 中华医学美学美容杂志

Objective To summarize the effect of eye socket reconstruction in patients with severe depressed eye socket combined anophthalmos and to assess the methods of eye socket reconstruction.Methods Forty patients of severe depressed eye socket combined anophthahnos,from Oct,2001 to Mar,2014,underwent eye socket reconstruction in Beijing Tongren Hospital.Thirty four eye sockets were reconstructed with free flap,the scapular flap in 2 cases,the forearm flap in 17 cases,the lateral arm flap in 15 cases.The reversed submental island flap was utilized in 2 patients.The other 4 cases were treated by implant-retained orbital prosthesis.Results All the patients were followed up for more than 2 years.The flaps survived.The artificial eye could be fitted satisfactorily and the appearance of the ill eye socket was improved significantly.The implant-bodies in orbital bone and the prosthesis were stable without peri-implantitis.Conclusions The flap transfer is effective for eye socket reconstruction in patient with severe depressed eye socket combined anophthalmos.The implant-retained orbital prosthesis is also alternative.The treatment choice must be based on the patient 's own conditions.

Reconstruction of severe atresic eye socket with lateral arm free flap / 中华显微外科杂志

Objective To study the method and effect of lateral arm free flap in reconstruction of severe atresic eye socket.Methods Forteen cases of severe atresic eye socket,from June,2011 to June,2013,were repaired by lateral arm free flap.The flaps were designed and harvested as drop shape with size about 6 cm × 10 cm and then were removed epidermis except distal 6 cm × 6 cm area which were transferred to orbit for eye socket reconstruction.The remaining fascia and dermis were filled to augment temporal defect.Superficial temporal artery was anastomosed with posterior branch of radial collateral artery in 14 cases and superficial temporal vein was anstomosed with radial collateral vein in 11 cases,with middle temporal vein in 3 cases.Results All 14 cases lateral arm free flaps survived with no donor site morbidity.Followed up for 1 year to 3 years,artificial eye could be fitted satisfactorily and temporal contour improved.Conclusion Lateral arm free flap is a recommendable option for severe atresic eye socket reconstruction because of concealed donor site scar,proper volume,matched vascular caliber and minor donor site morbidity.

Correction of Orbital Hypoplasia Using A Radial Forearm Osteocutaneous Free Flap

Exenteraion in early infancy by a tumor and radiotherapy caused the contracted eye socket and Orbital hypoplasia. This result is because of the abscence of the conjunctival fornix and in severe contraction of orbital fat and developmental bone abnormalities. The authors corrected a contracted eye socket and orbital hypoplasia simultaneously, secondary to previous surgery and radiotherapy. A one-stage surgical reconstruction was undertaken using a radial osteocutaneous flap, which were transfered for eye socket reconstruction and orbital hypoplasia. Adipofascia and fragment of radius inserted as an hypoplastic orbit rim susbtitute to enlarge the orbital region. We report that we have experienced a case of eye socket reconstruction with a radial forearm osteocutaneous free flap.

Versatile Use of Buccinator Myomucosal Flap for Oral Cavity and Orbital Reconstruction

This paper represents the versatility of buccinator myomucosal flap for intraoral and orbital reconstruction of mild to moderate defect and we introduce recontructive methods are introduced along with our clinical experience. Buccal artery from internal maxillary artery is the main arterial pedicle, and buccal branch from facial artery also can supply blood for the buccinator muscle. The abundant blood flow from interconnected pedicles supports the reliable circulation of the buccal mucosa. So we could reconstruct the eye socket using reversed island buccinator myomucosal flap based on the angular vessel. The motor innervation of the buccinator muscle comes from the facial nerve. The buccinator muscle is considered to be a part of the sphincteric muscular system involving the functions of sucking, whistling, propelling food during mastication and voiding the buccal cavity. From 1990 to 1999, the flap was utilized in 8 patients to reconstruct the small to moderate intraoral defect and 1 patient for orbital mucosal defect. All flaps survived completely. There has been no flap necrosis and fistula. The results have been functionally and esthetically satisfactory. It is very reliable and safe flap with a minimal morbidity of donor site. It doesn't require microsurgical technique. Rapid healing may be achieved with its mucosal nature. We conclude that buccinator myomucosal flap can be widely used with a lot of advantages for intraoral and orbital defect because of its many advantages.