Facial reanimation surgery in Möbius syndrome: Experience from 76 cases from a tertiary referral hospital in Latin America.

INTRODUCTION: Möbius syndrome is defined as a combined congenital bilateral facial and abducens nerve palsies. The main goal of treatment is to provide facial reanimation by means of a dynamic surgical procedure. The microneurovascular transfer of a free muscle transplant is the procedure of choice for facial animation in a child with facial paralysis. OBSERVATION: Between January 2008 and January 2017, 124 patients with the syndrome have been approached at our institution. Distribution according to Möbius Syndrome classification presents as follows: Complete Möbius syndrome (n=88), Incomplete Möbius syndrome (n=28), Möbius-Like syndrome (n=8). Seventy-nine female and 45 male patients. Sixty-one percent have undergone a microsurgical procedure (n=76), in all of them, a free gracilis flap transfer was performed. DISCUSSION: Our proposed treatment protocol for complete Möbius syndrome is determined by the available donor nerves. We prefer to use the masseteric nerve as first choice, however, if this nerve is not available, then our second choice is the spinal accesory nerve. For this purpose, all patients have an electromyography performed preoperatively. Overall, dynamic facial reanimation obtained through the microvascular transfer of the gracilis muscle have proved to improve notoriously oral comissure excursion and speech intelligibility. CONCLUSION: The free gracilis flap transfer is a reproducible procedure for patients with Möbius syndrome. It is of utmost importance to select the best motor nerve possible, based on an individualized preoperative clinical and electromyographic evaluation. To our best knowledge, this is the largest series of patients with Möbius syndrome globally, treated at a single-institution.

Quiste subaracnoideo y displasia del ala mayor del esfenoides en neurofibromatosis / Arachnoid cyst and dysplasia of the greater wing of the sphenoid in neurofibromatosis

Los quistes subaracnoideos representan menos del 1% de los tumores de ocupación intracraneal. Hay pocas publicaciones acerca de quistes subaracnoideos del lóbulo temporal con remodelación de la cavidad orbitaria, pero no existen en relación al tratamiento reconstructivo de la cavidad orbitaria en esta patología. Presentamos el caso clínico de un varón de 44 años de edad con diagnóstico de neurofibromatosis tipo 1, aumento progresivo de volumen del ojo y párpado izquierdos de 40años de evolución y dolor retro-ocular intermitente desde hacía 10 años, al que se le diagnostica un quiste subaracnoideo del lóbulo temporal que invade la cavidad orbitaria con displasia del ala mayor del esfenoides. Realizamos drenaje y cistocisternostomía del quiste subaracnoideo, con reconstrucción de paredes orbitarias con malla de titanio y cobertura con un colgajo de pericráneo así como elevación y reposicionamiento del globoocular mediante cantopexias, seguida de una osteotomía de la órbita para reducir el volumen de la misma (AU)

Arachnoid cysts represent less than 1% of brain tumors. There are few reports of subarachnoid cysts of the temporal lobe with subsequent remodeling of the orbit, however there are no reports regarding the reconstructive management of the orbital cavity in this pathology. We report the case of a man of 44 years with diagnostic of neurofibromatosis type 1 who presented progressive increase in volume of the left eye of 40 years of evolution, with intermittent retro-ocular pain. A temporal lobe arachnoid cyst was diagnosed invading the orbital cavity and dysplasia of the greater wing of the sphenoid. Drainage and cystocisternostomy of arachnoid cyst was performed, with orbital wall reconstruction with titanium mesh and covered with a flap of pericranium as well as the lifting and repositioning of the eyeball by canthopexies and orbital osteotomy to decrease the volume of the cavity (AU)

Hemangioma esclerosante pulmonar / No disponible

El hemangioma esclerosante pulmonar es un tumor benigno pulmonar que debe ser considerado en el diagnóstico diferencial del nódulo pulmonar solitario. Presentamos a continuación un caso de un paciente varón de 30 años con antecedente de tuberculosis pulmonar hace 10 años, y quien acudió a consulta por disnea. En estudios de imagen fue identificada una lesión en lóbulo superior izquierdo. Debido a la sospecha de malignidad, se realizó intervenir quirúrgicamente. Se realizó lobectomía de manera satisfactoria. El diagnóstico final fue hemangioma esclerosante pulmonar y a 6 meses de seguimiento, se encuentra asintomático. El pronóstico para el hemangioma esclerosante pulmonar es en términos generales bueno; no obstante, el seguimiento continuo a largo plazo en este tipo de pacientes es mandatorio

Pulmonary sclerosing hemangioma is a benign tumor that should be considered in the differential diagnosis of pulmonary solitary nodule. We present the case of a 30 year old male patient with history of pulmonary tuberculosis 10 years before. He was refered to our service because of dyspnea. CT-Scan demonstrated a suspicious image in left superior lobe. Due to the high suspicion of malignancy, we decided to perform surgery. Lobectomy was successfully performed. Final diagnosis reported a pulmonary sclerosing hemangioma. At six month follow up, he remains asymptomatic. Prognosis in pulmonary sclerosing hemangioma is good in general terms, however, long-term follow up is mandatory in these patients

Sarcoma del timo asociado a síndrome de vena cava superior / No disponible

No disponible

Resección de tumor de células germinales extragonadal gigante en mediastino / No disponible

Los tumores germinales extragonadales representan menos del 1% de las neoplasias en términos generales. Constituyen aproximadamente el 10% de los tumores de mediastino. La incidencia es mayor en varones de 15 a 30 años. Presentamos a continuación un caso de un varón de tercera década de la vida con un tumor no seminomatoso resecado satisfactoriamente. El tratamiento de este tipo de pacientes es multidisciplinario y requiere, entre otros, del manejo conjunto entre especialistas de patología respiratoria, cirugía de tórax y oncología (AU)

Extragonadal germ cell tumors represent less than 1% of neoplasms in general terms. They account for approximately 10% of mediastinal tumors. The incidence is greater in male from 15 to 30 years. We herein present the case of a third decade patient with a non seminomatous tumor surgically removed successfully. The treatment of these patients is multidisciplinary and demand the management of specialists in respiratory pathology, thorax surgery and oncology (AU)

Leiomiomatosis benigna pulmonar metastatizante / Pulmonary benign metastasising leiomyomatosis

La leiomiomatosis benigna metastatizante se caracteriza por la presencia de lesiones leiomiomatosas pulmonares, en ausencia de criterios clínicos, radiológicos e histopatológicos de malignidad. Nos centraremos en una paciente con antecedente de leiomiomatosis uterina. Se trata de una enfermedad poco común que debe ser considerada en el diagnóstico diferencial de tumores benignos pulmonares. Debido a que el abordaje en el manejo de este tipo de pacientes constituye un reto para el médico responsable de atender patología respiratoria, presentamos a continuación un caso de una mujer con antecedente de histerectomía secundaria a miomatosis uterina y a quien se realizó el diagnóstico de leiomiomatosis benigna metastatizante siendo tratada satisfactoriamente (AU)

Pulmonary benign metastasising leiomyomatosis is an uncommon disease characterized by the presence of pulmonary leiomyomas, in the absence of malignancy criteria in a patient with previous uterin leiomyomatosis. The disease is very uncommon and should be considered amongst the differential diagnosis of pulmonary benign tumors. Due to the challenge that represents to health care providers specialized in respiratory pathology in the diagnosis and management of these type of patients, we present the case of a patient with hysterectomy for uterin myomatosis in which the diagnosis of pulmonary benign metastasising leiomyomatosis was established and treated successfully (AU)

Infiltrating cellular pattern in kidney graft biopsies translates into forkhead box protein 3 up-regulation and p16INK4α senescence protein down-regulation in patients treated with belatacept compared to cyclosporin A.

Renal allograft survival is related directly to cell senescence. In the transplantation scenario many cellular events - participating as immunological and non-immunological factors - could contribute to accelerate this biological process, responsible for the ultimate fate of the graft. Mechanisms concerned in tolerance versus rejection are paramount in this outcome. For this reason, immunosuppressive treatment constitutes an extremely important decision to prevent organ dysfunction and, finally, graft loss. This study was conducted to document the proportion of CD4(+) /interleukin (IL)-17A(+) -, CD16(+) /indoleamine 2, 3-dioxygenase (IDO(+) )-, forkhead box protein P3 (FoxP3(+))-expressing cells, senescent cells (p16(INK) (4α)) and the percentage of interstitial fibrosis (IF) in graft biopsies of kidney transplant recipients participating in the BENEFIT (Bristol-Myers Squibb IM103008) study. CD4(+) /IL-17A(+) , CD16(+) /IDO(+), FoxP3(+) and p16(INK) (4α+) cells were evaluated by immunohistochemistry, and the percentage of IF by morphometry on graft biopsies obtained at time 0 (pre-implantation) and at 12 months post-transplant. Senescent cells and CD4(+) /IL-17A(+) cells were increased among graft biopsies in subjects receiving cyclosporin A (CsA) compared to those under belatacept treatment. Meanwhile, CD16(+) /IDO(+) and FoxP3(+) -expressing cells were lower in biopsies from CsA treatment compared to patients treated with Belatacept. Histological morphometric analyses disclosed more IF in 12-month CsA-treated patients in comparison to pre-implantation biopsy findings. Summing up, renal biopsies from patients receiving belatacept showed greater amounts of FoxP3(+) cells and lower amounts of CD4(+) /IL-17A(+) and senescent cells compared to patients under CsA treatment. Along with these findings, an increase in IF in annual CsA-treated-patients biopsies compared to pre-implantation and belatacept-treated patients were observed.

Role of major histocompatibility complex and ethnicity in acute renal graft rejection.

BACKGROUND: The major histocompatibility complex (MHC) plays a main role in antigen presentation. Class I, II, and III genes form defined "blocks" of conserved DNA sequences (conserved extended haplotypes) that are useful to follow the ancestry of a population. Each variant encodes a specific peptide that determines a particular individual's immune response. In addition, differential expression of HLA antigens in certain physiological and pathological conditions may participate in the pathogenesis of allograft rejection versus tolerance. OBJECTIVES: The aim of this study was to determine whether the specific HLA ancestry was associated with acute renal graft rejection among the Mexican mestizo population. MATERIALS AND METHODS: We studied 544 Mexican mestizo renal donors and their respective recipients for their serologically determined HLA and based on antigens haplotype assignments. The acute rejection group was compared with the nonrejection group among donors and recipients, correspondingly. RESULTS: Frequent Mexican alleles were observed in this study. Moreover, HLA-B*61/-DR*04, HLA-A*35/-DR*06 (Amerindian ancestry), HLA-A*68/-DR*01, HLA-A*28/-B*65/-DR*01 (African ancestry), and HLA-A*33/-B*65 (Caucasian ancestry) in donors were associated with acute renal graft rejection episodes. CONCLUSION: Knowing the ancestry of a donor's HLA molecules may help to individualize immunosuppressive therapy for posttransplant surveillance, because they may be more membrane-exposed in parenchymal cells, making them more susceptible of being recognized by the recipient's immune system.