Successful initiation of hemodialysis for a hemophilia A patient with factor VIII inhibitor: a case report and literature review.

We report the first case of hemophilia A with factor VIII (FVIII) inhibitor who received hemodialysis via an arteriovenous (AV) fistula. Hemophilia A is a congenital deficiency of blood coagulation FVIII that is characterized by prolonged bleeding. Approximately 30% of patients with hemophilia develop allogeneic antibodies of FVIII. The inhibitors decrease the hemostatic effect of replacement therapy; thus, the prophylaxis strategy should be well designed. Prophylactic treatment with invasive procedures is needed to prevent excessive bleeding in patients with hemophilia undergoing hemodialysis. On the contrary, hemodialysis requires attention to the development of intracircuit coagulation during dialysis. Peritoneal dialysis or hemodialysis with a long-term tunneled central venous catheter has mainly been selected as the dialysis modality for patients with hemophilia and end-stage renal disease requiring renal replacement therapy because hemodialysis with an arteriovenous fistula may result in bleeding from the puncture site after each hemodialysis session. In our patient, hemodialysis was safely performed without any anticoagulant agents, and replacement therapy with FVIII concentrates prevented bleeding after puncture of the AV fistula.

Intraoperative application of triamcinolone - a risk factor for delayed CSF fistula formation?

PURPOSE: Cerebrospinal fluid (CSF) leaks are a well-known complication in spinal surgery, caused mostly by incidental durotomy (ID). However, delayed pseudomeningocele formation has been described in patients following an unremarkable surgery - without ID. Intraoperative and epidural triamcinolone application has been suspected to be a potential risk factor. This study was conducted to evaluate the management of ID and identify further risk factors for secondary CSF fistula formation. METHODS: After obtaining approval from the institutional ethics committee, a total of about 5512 patients, who underwent spine surgery between January 2014 and December 2017, were retrospectively reviewed. Of those, 139 cases with intraoperative ID and 15 with delayed pseudomeningocele formation were extracted and analyzed to identify potential risk factors for a late presenting dural injury (LPDI). RESULTS: The incidence of delayed CSF fistulas was 0.27%, with 15 patients presenting with a secondary symptomatic CSF fistula following an unremarkable surgery. Triamcinolone was identified as a risk factor (p<0.001) for pseudomeningocele formation with an OR of 11.5, as it was applied in 80.0% (n=12) of these cases. Revision surgery was performed at a mean period of 6 weeks after initial surgery. CONCLUSION: In our retrospective analysis, intraoperative application of triamcinolone was significantly associated with a high rate of delayed CSF fistulas. It should therefore be used with caution and only after weighing in potential negative side effects.

Clinical Characteristics Associated With Lenvatinib-induced Fistula and Tumor-related Bleeding in Patients With Thyroid Cancer.

BACKGROUND: Lenvatinib has become an important treatment option for advanced thyroid cancer. Fistula and tumor-related bleeding are life-threatening adverse effects that are triggered by tumor shrinkage. The aim of this study was to evaluate basic parameters, such as time and tumor shrinkage level, and analyze patient characteristics that might be related to onset of complications. PATIENTS AND METHODS: A retrospective study of 16 patients who received lenvatinib for thyroid cancer treatment was performed. RESULTS: Fistula was observed in two patients (12.5%), while tumor-related bleeding was observed in one (6.3%). Complications were found to appear at 10.4 weeks from initiation and with tumor decrease of 19.2%. Risk factors for complications were identified as anaplastic histological type, tumor invasion, and leukocytopenia induced by lenvatinib. CONCLUSION: Individual dose adjustment is needed with respect to these features in order to manage severe adverse effects induced by lenvatinib.

Nivolumab therapy for lung cancer with tracheo-parenchymal fistula: A case report.

RATIONALE: Tracheobronchial fistulas are rare complications in lung cancer patients. These lesions are associated with a high rate of mortality caused by infection and bleeding, and there is no consensus on a definitive optimal therapy. PATIENT CONCERNS AND DIAGNOSES: The patient was a 59-year-old man with a right lung mass showing mediastinal invasion and tracheal compression, diagnosed with adenocarcinoma, cT4N0M0, stage IIIA. He was treated with concurrent chemoradiotherapy with carboplatin and paclitaxel, and the lesion markedly shrunk. Eleven months later, the lesion showed regrowth, and he underwent repeated chemotherapy for stabilization of the lesion. Thirty-six months after the first regrowth, the tumor showed regrowth again. The patient was then administered docetaxel and bevacizumab as fifth-line therapy. After 11 cycles of docetaxel and bevacizumab therapy, a tracheo-parenchymal fistula appeared. INTERVENTIONS AND OUTCOMES: Docetaxel and bevacizumab therapy was stopped, and nivolumab therapy was initiated. Subsequently, the fistula and cavity became stable with slight shrinkage. To date, the patient is alive with no complaints and no disease progression and has continued nivolumab for a total of 28 months. LESSONS: Immune-checkpoint inhibitor therapy involving nivolumab therapy might be a useful alternative for the treatment of lung cancer involving a tracheobronchial fistula.

Crizotinib-induced Rectal Perforation with Abscess.

An 86-year-old Japanese man was diagnosed with stage IV lung adenocarcinoma. The patient was treated with crizotinib after echinoderm microtubule-associated protein-like 4 (EML4)-anaplastic lymphoma kinase (ALK) rearrangement was detected from his pleural effusion. He subsequently developed abdominal pain and rebound tenderness in the right lower abdomen. Contrast-enhanced abdominal CT showed a low-density area in the abdominal cavity. The size of the abscess was decreased by drainage and the administration of antibiotics. Fistulography revealed a fistula from the rectum to the abscess, and a diagnosis of lower intestinal tract perforation with abscess formation was made. Crizotinib was discontinued and treatment with alectinib was initiated. The patient remains under treatment as an outpatient at our department without adverse effects.

Gastro-splenic fistula as a complication of chemotherapy for large B cell lymphoma.

AIM: Gastro-splenic fistula is a rare entity in which malignant tumors are the primary cause, followed by perforated peptic ulcers and Crohn's disease. CASE REPORT: A 66 years old patient undergoing chemotherapy for gastric large cells B lymphoma presented fever, fatigue and worsening of general conditions. A CT scan showed the presence of an abdominal abscess resulting from a pathological communication between stomach and spleen. RESULTS: En - bloc splenectomy and gastric wedge resection was performed; gastric wall was sutured with a linear stapler. Postoperative stay was uneventful; alimentation was restarted 5 days after the surgical procedure, and the patient was discharged 2 days later CONCLUSION: We have described an unusual case of gastric fistula complicating chemotherapy early diagnosed and successfully treated. KEY WORDS: Chemothera Gastrosplenic fistula, Lymphoma, Surgery.

Double pylorus

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Nicorandil: from ulcer to fistula into adjacent organs.

Nicorandil is an original vasodilatator used to control angina by decreasing cardiac preload and afterload. Since 1997, many reports of single or multiple nicorandil-induced ulcerations have been published. To date, eight cases of nicorandil-induced fistula into adjacent organs have been described. The pathogeneses of nicorandil-induced ulceration and fistula into adjacent organs are not yet elucidated. The two main hepatic biotransformation pathways of nicorandil are denitration and reduction of the alkyl chain leading to nicotinamide and niconitic acid which merge into the endogenous pool of nicotinamide adenine dinucleotide/phosphate. This merging which is known as saturable, may contribute to a slow and abnormal distribution of nicotinamide and nicotinic acid out of the endogenous pool. Under these special conditions, providing these two molecules in situ, nicotinic acid associated with nicotinamide may ulcerate rather recent or maintained trauma. Ulcers and fistulae induced by nicorandil heal after withdrawal. Surgical intervention is unnecessary and inappropriate as it is ineffective and exacerbates morbidity. All practitioners should be correctly informed about these serious but preventable nicorandil side effects, which mostly occur in the elderly and fragile population. In the absence of corrective measures, withdrawal of this original and active drug should be considered.

Doble fístula coronaria como causa de angina inestable / Dual coronary artery fistulas as a cause of unstable angina

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Establishment of a rescue program for anorectal malformations induced by retinoic acid in mice.

AIMS OF STUDY: Retinoid-mediated signal transduction plays a crucial role in the embryogenesis of various organs. We previously reported the successful induction of anorectal malformations in mice using retinoic acid (RA). Retinoic acid controls the expression of essential target genes for cell differentiation, morphogenesis, and apoptosis through a complicated interaction in which RA receptors form heterodimers with retinoid X receptors. In the present study, we investigated whether the retinoid antagonist, LE135, could prevent the induction of anorectal malformations (ARMs) in mice. METHODS: Retinoic acid was intraperitoneally administered as 100 mg/kg of all-trans RA on E9; and then the retinoid antagonist, LE135, was intraperitoneally administered to pregnant ICR strain mice on the eighth gestational day (E8), 1 day before administration of RA (group B) or on E9, simultaneously (group C) with RA administration. All of the embryos were obtained from the uteri on E18. Frozen sections were evaluated for concentric layers around the endodermal epithelium by hematoxylin and eosin staining. RESULTS: In group A, all of the embryos demonstrated ARM with rectoprostatic urethral fistula, or rectocloacal fistula, and all of the embryos showed the absence of a tail. In group B, 36% of the embryos could be rescued from ARM. However, all of the rescued embryos had a short tail that was shorter than their hind limb. The ARM rescue rates in group B were significantly improved compared to those in group A (P < .01). In group C, 45% of the embryos were rescued from ARM, but all of the rescued embryos had short tail. The ARM rescue rate in group C was significantly improved compared to that in group A (P < .01). However, there was no significant difference in the ARM rescue rate between group B and Group C. CONCLUSION: The present study provides evidence that in the hindgut region, RAR selective retinoid antagonist, LE135, could rescue embryos from ARM. However, the disturbance of all-trans RA acid was limited to the caudal region. Further study to establish an appropriate rescue program for ARM in a mouse model might suggest a step toward protection against human ARM in the future.

Tumor fistulization associated with targeted therapy: computed tomographic findings and clinical consequences.

PURPOSE: To describe the computed tomographic (CT) appearances and clinical consequences of tumor fistulization as a complication of targeted therapy for cancer. METHODS: The committee on human research approved this Health Insurance Portability and Accountability Act-compliant study and waived written informed consent. Based on the records of the senior author and our multidisciplinary Tumor Boards, we retrospectively identified 4 patients (1 man and 3 women with a mean age of 55.25 years; range, 47 to 64 years) who developed tumor fistulization while being treated with targeted therapy consisting of sunitinib (n = 2); bevacizumab (n = 1); and XL184, an investigational c-Met inhibitor (n = 1). All available clinical, imaging, and histopathological records were reviewed, with particular emphasis on treatment administered, CT findings, and clinical course. RESULTS: All 4 patients developed fistulae from large metastatic deposits in the abdomen (mean size before treatment, 10.55 cm; range, 7.4-13.4 cm) to the gastrointestinal tract, and one patient also developed fistulae from a lung metastasis of undetermined size to the bronchial tree. All fistulae manifested as the appearance of air within a pre-existing tumor mass. At the time of fistula detection, disease at other sites in the 4 patients showed signs of regression (n = 1), progression (n = 2), or stability (n = 1). Currently, one patient is alive without evidence of disease, and the 3 other patients are deceased. CONCLUSIONS: Targeted therapy can be associated with tumor fistulization to the gastrointestinal tract or tracheobronchial tree; familiarity with the CT findings should facilitate the diagnosis of this complication, which seems to be of variable and patient-specific prognostic significance.

Comparación del abordaje sublabial transesfenoidal microquirúrgico frente al abordaje endonasal transesfenoidal endoscópico. Estudio prospectivo de 50 pacientes / Prospective comparative study on 50 patients between microsurgical sublabial transsphenoidal approach and endoscopic endonasal transsphenoidal approach

Objetivos. Comparar el abordaje transesfenoidal endonasal endoscópico con el abordaje transesfenoidal sublabial microquirúrgico. Valoración del grado de resección tumoral, grado de invasividad tumoral, complicaciones y estancia postoperatoria de ambos grupos. Material y métodos. Estudio prospectivo no randomizado de 50 pacientes intervenidos en nuestro centro entre 2002 y 2006 de lesiones en la región selar con diferentes grados de invasión del seno cavernoso según la clasificación de Knosp. Entre las variables a estudio se incluyeron los grados de invasión, los grados de resección postoperatoria (total, subtotal y parcial), lesión del nervio óptico, panhipopituitarismo postoperatorio, fístula de LCR, déficit de pares craneales, epistaxis, meningitis, diabetes insípida y lesión de arteria caró- tida. Nuestra serie consta de 27 hombres y 23 mujeres, con una edad media de 48 años (19-80 años). En 23 casos se utilizó una vía transesfenoidal sublabial microquirúrgica (2 pacientes fueron excluidos) y en 25 casos se realizó un abordaje transesfenoidal endoscópico. El seguimiento medio fue de 12 meses. Resultados. En nuestra experiencia la técnica endoscópica presenta un porcentaje de exéresis completa superior al de la técnica clásica (60% frente a 34,8%) que también se hace evidente en el caso de la resección subtotal (32% frente 26%) existiendo una diferencia estadísticamente significativa (p=0,033). En contraposición no encontramos diferencias en cuanto a complicaciones. También hemos evidenciado que existe una diferencia estadísticamente significativa en cuanto a la estancia postoperatoria (p=0,001), reduciéndose ésta a la mitad (3 días) con la técnica endoscópica. Si bien no hemos encontrado diferencias significativas en cuanto al grado de invasividad tumoral y al grado de resección, un mayor grado de invasividad aumenta en 3,59 veces el riesgo de poco éxito de la cirugía. Discusión y conclusiones. En nuestra experiencia con la técnica endoscópica obtenemos un mayor grado de resección quirúrgica y una estancia postoperatoria menor. No hemos observado diferencias en cuanto a complicaciones (AU)

Objective. Compare the standard transsphenoidal sublabial microscopic approach with the endoscopic transsphenoidal approach concerning the tumoral invasiveness and resection, complications of the approaches and time of post operative hospitalisation. Material and methods. We realized a prospective, non randomised study with 50 patients. They were operated between 2002 and 2006. All the patients had sellar lesions with different grades of invasiveness of the cavernous sinus as classified by Knosp. The variables included in our study were tumoral invasiveness and operative resection (total, subtotal and partial), optic nerve lesion, postoperative panhypopituitarism, CSF fistula, cranial nerves deficits, epistaxis, meningitis, diabetes insipidus and carotid artery lesion. Our series included 27 males and 23 females ranging from 19 to 80 years old (48 mean). In 23 patients we used the standard sublabial microscopic approach (two patients were excluded) and for 25 patients we used the endoscopic approach. The mean follow up was of 12 months. Results. In our experience the endoscopic technique presents a higher percentage of total resection comparing to the sublabial microscopic approach (60% versus 34,8%) and higher percentage of subtotal resections (32% versus 26%) with a statistical significan difference (p=0,033). The time of hospitalisation was significant shorter for the endoscopic approach group (p=0,001), diminishing by half of the time (3 days) of the microscopic approach group. Concerning the tumoral invasiveness and complications we did not appreciate any significant dissimilarity. We appreciated that a higher grade of invasiveness augments by 3,59 the risk of an unsuccessful surgery. Discussion and conclusion. In our experience the endoscopic technique may favour a better tumoral resection and shorter time of hospitalisation. We did not appreciate differences concerning the complications (AU)

Fístulas mandibulares en una mujer de 75 años / Jaw fistulas in 75 years-old woman

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