Unusual presentation of Loeys-Dietz syndrome: A case report of clinical findings and treatment challenges.

BACKGROUND: Loeys-Dietz syndrome (LDS) is a rare autosomal dominant syndrome characterized by heterozygous mutations causing multisystemic alterations. It was recently described in 2005, and today at least six different subtypes have been identified. Classically presenting with aortic root enlargement or aneurysms and craniofacial and skeletal abnormalities, with specific arterial tortuosity at any site. The differential diagnosis of LDS includes atypical Marfan syndrome, vascular Ehlers-Danlos syndrome, Shprintzen-Goldberg craniosynostosis, and familial aortic aneurysm and dissection syndrome. CASE SUMMARY: We present a case study of a 35-year-old female who came to the emergency department due to lower gastrointestinal bleeding and severe abdominal pain. Computed tomography revealed vascular tortuosity in almost every abdominal vein. CONCLUSION: This case report will help us analyze the infrequent presentation of LDS type 4 and the numerous complications that it implies, underlying the importance of publishing more cases in order to expand our knowledge and offer better treatment for these patients. Differential diagnosis, clinical presentation and treatment options for this syndrome are discussed in this article.

Conversion from mini bypass to laparoscopic Roux en Y gastric bypass in an emergency setting: Case report and literature review.

INTRODUCTION: It has been demonstrated that certain technique endpoints are key to the success for the OAGB and RYGB procedures but only a few texts in which post-operative complications are documented. PRESENTATION OF CASE: 42-year-old male patient admitted to the emergency department for presenting abdominal pain located in the epigastrium for 4 days, melenic evacuations and syncope on one occasion. Two years prior to admission, the patient underwent a single anastomosis bypass for grade III obesity.Gastric bypass mini revision surgery was performed an antecolic and antegastric gastrointestinal anastomosis was made with a 3 cm latero-lateral anastomosis; an intestinal-intestinal anastomosis was performed 60 cm from the gastric anastomosis. The length of the biliopancreatic loop (120 cm) and the feeding loop (60 cm) are reviewed. DISCUSSION: Performing an "en bloc" resection of the anastomosis is essential since bile reflux is one of the irritation mechanisms of the anastomosis but not the only one. The size of the gastric pouch directly influences the frequency of marginal ulcers, so during the OAGBP revision, the gastro-jejunal junction must be resected to remodel it, reducing the size of the gastric reservoir that allows to perform the new anastomosis in less inflamed tissue. Roux-en-Y reconstruction should be performed once the length of the biliopancreatic loop is verified and it does not exceed 150 cm and a short alimentary loop to avoid nutritional complications.Complications arising from bariatric procedures are varied, infrequent in well-trained surgeons, but severe in inexpert hands, leading to an increase in mortality rates. CONCLUSIONS: We propose the laparoscopic conversion of OAGB to RYGB as a safe method, and feasible in hemodynamically unstable patients.

Laparoscopic T-tube choledochotomy for biliary lithiasis.

T-tube choledochotomy has been an established practice in common bile duct exploration for many years. Although bile leaks, biliary peritonitis, and long-term postoperative strictures have been reported and are directly associated with the placement or removal of the T-tube, the severity of these complications may often be underestimated by surgeons. We present the case of a 31-year-old male patient who developed biliary peritonitis and septic shock after removal of a T-tube and illustrate one of the catastrophic events that may follow such procedures. Literature shows that these complications may occur more frequently and have higher morbidity and mortality than other less invasive procedures. This article reviews the advances in laparoscopic and endoscopic techniques, which provide alternative therapeutic approaches to choledocholithiasis and allow the surgeon to avoid having to perform a choledochotomy with T-tube drainage.

[Capsule endoscopy: The evolution in the diagnosis of small bowel diseases]. / La capsula endoscópica: la evolución en el diagnóstico de las enfermedades del intestino delgado.

Wireless capsule endoscopy is a diagnostic procedure to study the pathology of the small intestine physiologically and painlessly. The capsule dimensions are 11 x 26 mm, and takes 2 picture per second whilst 8 hours. Unexplained occult gastrointestinal tract bleeding is the main indication, but everyday new indications for its use come to the literature. Our objective were to review our experience about the clinical usefulness and impact in our clinic. We included 45 cases, excluding 3 because of technical problems. There were 24 women and 18 men, with an average age of 54 years old (18 to 86 years old). Indications for the study were: Gastrointestinal bleeding of obscure origin in 24 cases, anemia in 6 cases, chronic diarrhea in 8 cases, chronic abdominal pain in 2 cases and Crohn's disease in 2 cases. The source of bleeding in the first group was identified in 18 patients (75%), where jejunal and ileal angiodysplasias were found in 11 patients, in 4 cases there were ulcers or erosions, in one case a Meckel diverticulum was found and, in the last one an hammartomatous lesion with an active bleeding was found. In chronic diarrhea patients a lesion was found in 5 cases (62.5%), where mucosal atrophy were found in two patients who responded to a free gluten diet, and in 3 patients acute inflammations with ulcers were treated as Crohn's disease. In the patients with anemia a lesion was found in 2 cases (33%), where a submucosal tumor and a jejunal ulcer were the findings. No lesions were found in the patients with chronic abdominal pain. Finally in the patients with Crohn's disease we were able to know the extent and one patient presented two stenotic lesions. In conclusion, wireless capsule endoscopy is a useful diagnostic tool that let us study easily the small intestine and should be integrated to different study protocols as gastrointestinal bleeding of obscure origin, chronic diarrhea and evaluation of Crohn's disease. It is not useful for abdominal pain, nevertheless we just studied two patients.

[Endoscopic ultrasound guided fine needle aspiration biopsy: first experience in Mexico]. / Biopsia por aspiración con aguja delgada guiada por ultrasonido endoscópico: primera experiencia en México.

BACKGROUND: Endoscopic ultrasound (EUS) -guided fine-needle aspiration (FNA) is a recently described diagnostic method that has demonstrated its usefulness in certain clinical conditions. MATERIAL AND METHODS: Descriptive and retrospective analysis of the initial experience with EUS-guided FNA at the Hospital General "Dr Manuel Gea González", during the period between March, 1998 and December, 1999. A Pentax FG 32UA linear echoendoscope was used for all cases, as well as two different types of needles. Procedures were performed under sedation and a cytopathologist was not present during the FNA. The biopsy site, quality of the material obtained, cytological as well as final diagnosis and complications are described. RESULTS: A total of 40 procedures were done. FNA was performed on the pancreas, esophagus, stomach, duodenum, mediastinum, rectum and lymph nodes. A diagnosis was obtained in 75%. In the remaining cases, the material obtained was either inadequate or insufficient for diagnosis. FNA results were confirmed histologically in all cases that underwent surgery. Complication presented in 2.5%, and consisted of one case of self-limited and clinically irrelevant bleeding. The best results were obtained in lymph nodes, mediastinum, liver and pancreatic tumors. CONCLUSIONS: This study demonstrated the utility of EUS-guided FNA to obtain cytologic material for diagnosis in a high percentage of cases and with minimal complications. Some factors that could lead to better results were also identified.

Experiencia del uso de Enbucrilato, para obliteración de várices fúndicas, en el Hospital General "Dr. Manuel Gea González" / Experience with of enbucrilate to fundic varices in the Hospital General \"Dr. Manuel Gea Gonzalez\"

Siendo el sangrado variceal una causa importante de muerte, numerosos métodos se han introducido para el control de la hemorragia aguda variceal, como la escleroterapia transendoscópica con diversidad de agentes esclerosantes, la aplicación de ligaduras y actualmente el enbucrilato, el cual ha demostrado resultados adecuados en la obliteración de las várices, especialmente las fúndicas, que tienen un alto número de fracaso terapéutico con otros métodos. Presentamos a 17 pacientes, diez hombres y siete mujeres con promedio de edad de 53 años, incluidos del 25 de febrero de 1997 al 15 de junio de 1998 con aplicación de enbucrilato en várices fúndicas, revisando tanto resultados inmediatos, seguimiento endoscópico y comprobación de la ausencia de flujo por medio del USG endoscópico doppler color. No se observó ninguna complicación y a la mayoría de los pacientes les fue suficiente de una a dos aplicaciones para su obliteración total

Características del comportamiento del reflujo en pacientes con esófago de Barrett y enfermedad por reflujo gastroesofágico no complicada / Characteristics of patients with reflux esophagitis and Barrett's epithelium

La casi invariable asociación entre el reflujo gastroesofágico y epitelio de Barrett apoya la teoría de ser condición adquirida, varios estudios han reportado una mayor exposición de la mucosa esofágica al ácido en estos pacientes al compararlos con pacientes con enfermedad por reflujo no complicada. El objetivo de este trabajo fue valorar el grado de exposición de la mucosa esofágica al ácido en pacientes con esofágo de Barrett y compararlo con aquellos con enfermedad por reflujo gastroesofágico no complicada. Se evaluaron 51 pacientes de ambos sexos divididos en tres grupos. Grupo I, pacientes con esofágo de Barrett (metaplasia intestinal), Grupo II, 22 pacientes con enfermedad por reflujo gastroesofágico no complicada y Grupo III, (15 voluntarios sanos con endoscopia, manometría y monitoreo de pH normales). El grado de exposición de la mucosa esofágica al ácido se valoró mediante monitoreo ambulatorio del pH con los siguientes resultados: los pacientes con esofágo de Barrett mostraron un mayor número de episodios de reflujo tanto en la posición de pie como en la supina, así como episodios de mayor duración al compararlos con aquellos con enfermedad por reflujo no complicada, quienes mostraron también tener una exposición anormal, pero con periodos más cortos y menos numerosos sugiriendo que la magnitud del reflujo gastroesofágico es un factor importante en la patogénesis del esófago de Barrett