Laparoscopic Swenson procedure - An optimal approach for both primary and secondary pull-through for Hirschsprung's disease

Background/Purpose: Several pull-through procedures are available for the surgical management of Hirschsprung's disease (HD) in children. The authors have adopted a laparoscopic approach since 1995, including laparoscopic Swenson procedure (LSw), both for one-stage primary and 2-stage secondary procedures. The aim of this study was to examine the role of LSw in children with HD in both primary and secondary procedures. Methods: From January 1995 to December 2001, 42 children with biopsy-proven HD underwent laparoscopic pull-through procedure for HD. This group included 29 children who underwent LSw, a detailed analysis of which forms the basis of this report. Results: Sixteen children underwent a single-stage neonatal LSw; the median weight of this group at the time of surgery was 3.2 kg and the median age was 5 days. Secondary LSw was performed in the remaining 13 children, which included 3 children with total colonic HD who underwent laparoscopic total colectomy and LSw. The median operating time was 105 minutes (range, 66 to 175 minutes). The median time to commence full diet was 48 hours (range, 24 to 86 hours), and median time to return to normal play and activity was 72 hours (range, 48 hours to 5 days). There was no difference in operating time between primary and secondary pull-through procedures. There were no intraoperative complications, and no patient required open conversion. Postoperative ileus was noted in 3 children and enterocolitis in 2. The median hospital stay was 4 days (range, 2 to 6 days). Follow-up was between 6 months to 7 years with a median follow-up of 2.2 years. At follow-up, 2 children required laparoscopic antegrade continence enema procedure. A satisfactory continence was noted in 15 of the 19 children who were older than 3 years at the time of last follow-up. Conclusions: LSw seems to be a suitable procedure for laparoscopic management of HD in children. LSw is safe and effective, both for primary and secondary type of pull-through procedures, with good short-term results.

Syringomatous adenoma of the nipple

Infiltrating syringomatous adenoma (SA) of the nipple is a rare but distinct benign clinical entity affecting the breast. It needs to be included in the differential diagnosis of patients who present with a lump in the nipple/areola complex. It is similar histologically to a syringoma, a benign tumour originating in the ducts of the dermal sweat glands, and importantly needs to be distinguished from a tubular carcinoma. SA of the nipple is locally infiltrating but is not known to metastasise. It often presents as a subareolar lesion with clinical, mammographic and ultrasound findings suspicious for malignancy. Whilst it may be possible to suspect the diagnosis on fine needle cytology, core biopsy or excisional biopsy is usually required to establish the diagnosis. There is a tendency to recurrence if excision is incomplete. The following is a case report, literature review and discussion of the surgical management options available in this unusual condition. (C) 2004 Elsevier Ltd. All rights reserved.

New era: Prophylactic surgery for patients with multiple endocrine neoplasia-2A

Background: The surgical management of patients with multiple endocrine neoplasia-2A (MEN-2A) continues to evolve with specific genotype-phenotype correlations allowing for a more tailored approach. In this study, we report the surgical management of one of the largest MEN-2A families with a rearranged during transfection (RET) codon 804 mutation. Method: This is a cohort study comprising all at-risk kindred within a single known MEN-2A family. Prophylactic total thyroidectomy with lymph node dissection was recommended to all mutation carriers aged 5 years and older. Results: There were a total of 48 at-risk individuals in the MEN-2A kindred, with 22 patients undergoing thyroidectomy after appropriate preoperative evaluation. A total of 9 patients had medullary thyroid cancer including 5 with a normal preoperative calcitonin level. A total of 11 patients had C-cell hyperplasia and 7 showed histological evidence of parathyroid disease. Only the index case had a phaeochromocytoma. Conclusion: Genetic testing for germline mutations in the RET proto-oncogene has allowed precise identification of affected RET carriers and provided the opportunity for prophylactic or 'preclinical' surgery to treat and in fact to prevent medullary thyroid cancer. This concept of prophylactic surgery based on a genetic test is likely to be applied more widely as the tools of molecular biology advance.

Calcaneus-navicular coalition associated to flatfoot in a child. A case report

Tarsal coalition (a congenital fibrous, cartilaginous or bony connection between two bones) often leads to a flatfoot deformity in children. Usually it presents with recurrent ankle sprains or insidious onset of a painful rigid flatfoot and movement limitation of midtarsal and subtalar joints. Clinical diagnosis is confirmed by X-rays, computed axial tomography and nuclear magnetic resonance. The anteater nose sign is caused by a tubular elongation of the anterior process of the calcaneus that approaches or overlaps the tarsal scaphoid (navicular) and resembles the nose of an anteater on a lateral foot or ankle radiograph. The treatment of this union is primarily symptomatic but if the pain persists must be surgical .

Hallux rigidus: Arthroplasty or arthrodesis. What technique produces better results?

Hallux rígidus (HR) affects the first metatarsophalangeal joint (MTPJ) between 35% and 60% of the population over 65 years and there are multiple ways of treatment. Depending on the radiological stage where you find the deformity determines the procedure to be performed; in the early stages cheilectomy techniques and corrective osteotomy is performed while the more advanced ratings, the surgeon chooses destructive techniques considered as arthrodesis and arthroplasty. This final of degree project aims to focus on 1 MTPJ destructive techniques to clarify which of the procedures generates better results by a number of parameters; outcomes of the American Orthopaedic Foot scale and Ankle Society Hallux metatarsophalangeal Interphalangeal-scale (AOFAS), range of motion (ROM) of the 1ºAMTF, radiological classification. As for the implant arthroplasty technique, this article offers information on material and design that generates better relating to patient characteristics such as age, inflammatory joint diseases, viability and durability of the implant results. The conclusion from this review is that the values obtained in the arthrodesis according AOFAS decrease due to loss of mobility, but both techniques have similar values of effectiveness and concludes with the decision that the technique used is determined taking into account various factors and patient characteristics. Keywords: Hallux rígidus; (Hallux Rígidus) and surgery treatment; Hallux Rígidus arthrodesis; Hallux Rígidus arthroplasty; Hallux Rígidus (arthroplasty and arthrodesis).

Dermoscopic variability of basal cell carcinoma according to clinical type and anatomic location

Background Correctly diagnosing basal cell carcinoma (BCC) clinical type is crucial for the therapeutic management. A systematic description of the variability of all reported BCC dermoscopic features according to clinical type and anatomic location is lacking. Objectives To describe the dermoscopic variability of BCC according to clinical type and anatomic location and to test the hypothesis of a clinical/dermoscopic continuum across superficial BCCs (sBCCs) with increasing palpability. Methods Clinical/dermoscopic images of nodular BCCs (nBCCs) and sBCCs with different degrees of palpability were retrospectively evaluated for the presence of dermoscopic criteria including degree of pigmentation, BCC-associated patterns, diverse vascular patterns, melanocytic patterns and polarized light patterns. Results We examined 501 histopathologically proven BCCs (66.9% sBCCs; 33.1% nBCCs), mainly located on trunk (46.7%; mostly sBCCs) and face (30.5%; mostly nBCCs). Short fine telangiectasias, leaf-like areas, spoke-wheel areas, small erosions and concentric structures were significantly associated with sBCC, whereas arborizing telangiectasias, blue-white veil-like structures, white shiny areas and rainbow pattern with nBCCs. Short fine telangiectasia, spoke-wheel areas and small erosions were independently associated with trunk location, whereas arborizing telangiectasias with facial location. Scalp BCCs had significantly more pigmentation and melanocytic criteria than BCCs located elsewhere. Multiple clinical/dermoscopic parameters displayed a significant linear trend across increasingly palpable sBCCs. Conclusions Particular dermoscopic criteria are independently associated with clinical type and anatomic location of BCC. Heavily pigmented, scalp BCCs are the most challenging to diagnose. A clinical/dermoscopic continuum across increasingly palpable sBCCs was detected and could be potentially important for the non-surgical management of the disease.

Vacuum-assisted closure treatment of leg skin necrosis after angiographic embolization of a giant plexiform neurofibroma

Type 1 neurofibromatosis is a relatively common inherited disease of the nervous system, with a frequency of almost 1 in 3000. It is associated with neurofibromas of various sites. Our case report is about the surgical management of a giant neurofibroma of the right gluteal fold in a 46-year-old male with NF1. The patient presented with increasing edema and accelerated growth of the mass; he underwent percutaneous embolization of lesion vessels that induced necrosis of the neurofibroma. The patient was taken to the operating room, where surgical resection of the bulk of the lesion was undertaken. The postoperative course was complicated by delayed wound closure managed with antibiotics and vacuum-assisted wound closure. Giant neurofibromas similar to this tumor require complex preoperative, intraoperative and postoperative management strategies. Surgical debulk is best managed with preoperative percutaneous embolization that help to avoid surgical bleeding. Postoperative delayed wound closure was managed with the application of negative pressure in a closed environment that triggers granulation and tissue formation.

Endovascular treatment of multiple anomalous splenic artery aneurysms in a Jehovah witness

Purpose. The present report describes a full endovascular treatment of a multiple anomalous (Splenic artery aneurysms) SAA with combination of coils embolization and proximal occlusion of the splenic artery with the Amplatzer vascular plug. Case report. A 53-year-old Jehovah witness woman presented with multiple aneurysms arising from an anomalous splenic artery. An endovascular treatment was performed by implantation of multiple coils and an Amplatzer Vascular Plug. A CT scan 2 months after the procedure showed complete thrombosis of the aneurysms. Discussion. Aneurysms involving an anomalous or aberrant splenic artery are rarely reported in the literature. Their surgical treatment involves potential difficulties as a consequence of anatomical position and vascular anomalies. A fully endovascular technique can be much more attractive compared to any surgical management, providing an effective and minimally invasive option. Splenic artery - Aberrant splenic artery - Aneurysm - Endovascular treatment - Jehovah witness.

Tumores do espaço parafaríngeo: experiência de 10 anos do IPO-LFG

Introdução: As neoplasias do espaço parafaríngeo são raras, representando apenas 0,5% dos tumores da cabeça e pescoço. A maioria são benignas, mas uma ampla variedade de patologias benignas e malignas podem ser encontradas neste espaço, o que cria desafios complexos de diagnóstico e tratamento. Objetivo: Descrever e analisar uma série de casos de neoplasias primárias do espaço parafaríngeo tratadas no Instituto Português de Oncologia de Lisboa Francisco Gentil (IPOLFG). Material e métodos: Estudo retrospetivo, com recolha e análise dos dados dos processos clínicos de tumores primários do espaço parafaríngeo, que foram diagnosticados ou referenciados ao IPOLFG entre 1 de Janeiro de 2003 e 31 de Dezembro de 2013. Resultados: Foram incluídos 38 doentes. A idade mediana foi de 52 anos (Âmbito Interquartil: 40-63 anos). Dez (26,3%) doentes eram assintomáticos. O sintoma mais comum à apresentação foi a sensação de corpo estranho orofaríngeo (23,7%) e o achado mais frequente foi um abaulamento orofaríngeo (78,4%). Todos os doentes fizeram exames de imagem pré-operatórios: 94,7% tomografia computorizada e 68,4% ressonância magnética. A citologia aspirativa foi realizada em 39,5%. 31 tumores eram benignos (81,6%), sendo os mais frequentes os adenomas pleomórficos (58,1%). 7 eram malignos (18,4%), com os carcinomas exadenomas pleomórficos (28,6%) e os linfomas (28,6%) sendo os mais comuns. 36 doentes (94,7%) foram submetidos a tratamento cirúrgico primário; os outros 2 doentes (5,3%) receberam tratamento não cirúrgico, com quimioterapia e quimioradioterapia, respectivamente. A abordagem cervical foi a mais utilizada (80%). A mandibulotomia foi necessária em apenas 5,7%. A complicação mais frequente foi a neuropatia de pares cranianos de novo, identificada em 22,2%. Destes, 75% foram sequela da resseção de tumores neurogénicos. Todas as neuropatias que resultaram da resseção de tumores não neurogénicos foram transitórias. O follow-up mediano foi de 6,5 anos. A taxa de recorrência foi de 13,5%. Conclusões: Os tumores do espaço parafaríngeo requerem um elevado índice de suspeição para serem diagnosticados num estadio precoce. A resseção cirúrgica completa é o principal tratamento. A abordagem cirúrgica deve ser selecionada caso a caso, mas a cervical fornece um excelente acesso à maioria dos tumores deste espaço

Trombose dos seios laterais como complicação de otite média : do diagnóstico ao tratamento

Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2014

Phyllodes tumor of the breast: a clinic-pathologic study of 77 cases in a Hispanic cohort

Introduction: Breast Phyllodes tumors are rare breast tumors present in less than 1% of new cases of breast cancer, usually occurring among middle-aged women (40-50 yrs). Objective: This study shows diagnostic experience, surgical management and follows up of patients with this disease during a period of ten years in a oncology referral center. Methods: Retrospectively, breast cancer registries at the institution were reviewed, identifying 77 patients with Phyllodes tumors between 2002 and 2012, who had been operated on at the Instituto de Cancerología – Clínica Las Américas, in Medellín (Colombia). Clinical and histopathological data belonging to these cases was captured and analyzed and descriptive statistics were used. Results: The follow up median was 22.5 months (IQR: 10.5-60.0), average age was 47.2 yrs (SD: 12.4), mean tumor size was 3.6 cm (SD: 4.6), 88.3% of the patients (68 cases) presented negative margins and none of them received adjuvant chemotherapy. Of the patients with Phyllodes tumors; 33.8% had benign, 31.2% had borderline and 35.0% had malignant tumor. Disease-free survival was 85.8% and overall survival was 94.5%. Discussion: Reported data in this article is in accordance with what has been reported in worldwide literature. In our cohort even the high mean size of the tumors, the risk of local relapse and metastatic disease is low than previously reported in literature. Trials with longer follow up and molecular trials in Phyllodes tumors are necessary to understand the behavior of these tumors in Hispanics population.

Caractérisation pharmacocinétique et pharmacodynamique de la lidocaïne avec ou sans adrénaline lors d’un bloc paravertébral du plexus brachial chez le chien

Au cours des vingt dernières années, l’anesthésie régionale est devenue, autant en médecine vétérinaire qu’humaine, un outil essentiel à l’élaboration de protocoles analgésiques péri-opératoires. Parmi l’éventail de techniques mises au point en anesthésie canine, le bloc paravertébral du plexus vertébral (PBPB) et sa version modifiée sont d’un grand intérêt pour toute procédure du membre thoracique, dans sa portion proximale. Toutefois, l’essentiel des données publiées à ce jour provient d’études colorimétriques, sans évaluation clinique, et peu d’information est disponible sur les techniques de localisation nerveuse envisageables à ce site. Notre étude visait à décrire une approche échoguidée du PBPB modifié, puis à caractériser ses paramètres pharmacocinétiques et pharmacodynamiques après administration de lidocaïne (LI) ou lidocaïne adrénalinée (LA). Huit chiens ont été inclus dans un protocole prospectif, randomisé, en aveugle et croisé, réparti sur trois périodes. L’impact pharmacodynamique du bloc effectué avec LI ou LA a été évalué régulièrement pour 180 min suivant son exécution. Le traitement à l’adrénaline n’a pas démontré d’impact significatif (P = 0,845) sur la durée du bloc sensitif, tel qu’évalué par un stimulus douloureux mécanique appliqué aux dermatomes ciblés. À l’opposé, l’atteinte proprioceptive évaluée par la démarche a été trouvée prolongée (P = 0,027) et le bloc moteur mesuré par le pic de force verticale (PVF) au trot sur la plaque de force s’est avéré plus marqué (PVF réduit; P = 0,007) sous LA. À l’arrêt comme au trot, le nadir de la courbe PVF-temps a été trouvé retardé (P < 0,005) et la pente ascendante de retour aux valeurs normales adoucie (P = 0,005). Parallèlement aux évaluations cliniques, des échantillons plasmatiques ont été collectés régulièrement afin de quantifier et décrire le devenir pharmacocinétique de la lidocaïne. Parmi les trois élaborés, un modèle bi-compartimental doté d’une double absorption asynchrone d’ordre zéro a finalement été sélectionné et appliqué aux données expérimentales. Sous LA, la Cmax a été trouvée significativement diminuée (P < 0,001), les phases d’absorption prolongées [P < 0,020 (Dur1) et P < 0,001 (Dur2)] et leurs constantes réduites [P = 0,046(k01) et P < 0,001 (k02)], le tout en concordance avec les effets proprioceptifs et moteurs rapportés. Bien que l’extrapolation du dosage soit maintenant théoriquement envisageable à partir du modèle mis en lumière ici, des études supplémentaires sont encore nécessaires afin d’établir un protocole de PBPB d’intérêt clinique. L’analyse sur plaque de force pourrait alors devenir un outil de choix pour évaluer l’efficacité du bloc dans un cadre expérimental.

Caractérisation pharmacocinétique et pharmacodynamique de la lidocaïne avec ou sans adrénaline lors d’un bloc paravertébral du plexus brachial chez le chien

Au cours des vingt dernières années, l’anesthésie régionale est devenue, autant en médecine vétérinaire qu’humaine, un outil essentiel à l’élaboration de protocoles analgésiques péri-opératoires. Parmi l’éventail de techniques mises au point en anesthésie canine, le bloc paravertébral du plexus vertébral (PBPB) et sa version modifiée sont d’un grand intérêt pour toute procédure du membre thoracique, dans sa portion proximale. Toutefois, l’essentiel des données publiées à ce jour provient d’études colorimétriques, sans évaluation clinique, et peu d’information est disponible sur les techniques de localisation nerveuse envisageables à ce site. Notre étude visait à décrire une approche échoguidée du PBPB modifié, puis à caractériser ses paramètres pharmacocinétiques et pharmacodynamiques après administration de lidocaïne (LI) ou lidocaïne adrénalinée (LA). Huit chiens ont été inclus dans un protocole prospectif, randomisé, en aveugle et croisé, réparti sur trois périodes. L’impact pharmacodynamique du bloc effectué avec LI ou LA a été évalué régulièrement pour 180 min suivant son exécution. Le traitement à l’adrénaline n’a pas démontré d’impact significatif (P = 0,845) sur la durée du bloc sensitif, tel qu’évalué par un stimulus douloureux mécanique appliqué aux dermatomes ciblés. À l’opposé, l’atteinte proprioceptive évaluée par la démarche a été trouvée prolongée (P = 0,027) et le bloc moteur mesuré par le pic de force verticale (PVF) au trot sur la plaque de force s’est avéré plus marqué (PVF réduit; P = 0,007) sous LA. À l’arrêt comme au trot, le nadir de la courbe PVF-temps a été trouvé retardé (P < 0,005) et la pente ascendante de retour aux valeurs normales adoucie (P = 0,005). Parallèlement aux évaluations cliniques, des échantillons plasmatiques ont été collectés régulièrement afin de quantifier et décrire le devenir pharmacocinétique de la lidocaïne. Parmi les trois élaborés, un modèle bi-compartimental doté d’une double absorption asynchrone d’ordre zéro a finalement été sélectionné et appliqué aux données expérimentales. Sous LA, la Cmax a été trouvée significativement diminuée (P < 0,001), les phases d’absorption prolongées [P < 0,020 (Dur1) et P < 0,001 (Dur2)] et leurs constantes réduites [P = 0,046(k01) et P < 0,001 (k02)], le tout en concordance avec les effets proprioceptifs et moteurs rapportés. Bien que l’extrapolation du dosage soit maintenant théoriquement envisageable à partir du modèle mis en lumière ici, des études supplémentaires sont encore nécessaires afin d’établir un protocole de PBPB d’intérêt clinique. L’analyse sur plaque de force pourrait alors devenir un outil de choix pour évaluer l’efficacité du bloc dans un cadre expérimental.

Desenlaces en pacientes embarazadas con patología quirúrgica abdominal 2011-2014

Estudio transversal realizado entre 2011 y 2014 en la Clínica de la Mujer con el fin de describir el comportamiento de la salud el binomio madre hijo en gestantes que requirieron manejo quirúrgico de patologías quirúrgicas abdominales no ginecobstétricas.