Case reports

CASE REPORTS

PULMONARY PARACOCCIDIOIDOMYCOSIS ASSOCIATED WITH SEPTIC SHOCK IN AN IMMUNOCOMPETENT PATIENT. CASE REPORT

ABSTRACT Introduction. Paracoccidioidomycosis (PCM) is a chronic granulomatous disease caused by the dimorphic fungus known as Paracoccidioides brasiliensis. This entity compromises mainly the lungs, but can spread to other organs, with particular trophism, through oral mucosa, adrenal glands, lymph nodes, among others. Case presentation. This paper reports the case of a male patient with pulmonary PCM treated at the Hospital Universitario de Santander. The patient was admitted with initial suspicion of active pulmonary tuberculosis due to the presence of multiple cavitations and nodules of random distribution in the lung parenchyma observed in the chest tomography, and subsequent isolation of yeasts compatible with Paracoccidioides. Amphotericin B deoxycholate was administered without favorable outcomes and development of septic shock by extended spectrum Klebsiella pneumoniae. In spite of multi-conjugate antibiotic management, the patient presented multiple organ failure syndrome with fatal outcome at 21 days of hospitalization. Conclusion. Pulmonary PCM is an endemic disease that leads to an inadequate immune response of the host that -along with risk factors such as smoking, alcohol abuse, malnutrition and low socioeconomic status- facilitates the onset of life-threatening infections or coexisting diseases. Timely diagnosis based on early clinical suspicion potentially influences the patient's survival.

SPLENIC RUPTURE ASSOCIATED WITH THROMBOCYTOPENIC PURPURA CAUSED BY INFECTIOUS MONONUCLEOSIS. CASE REPORT

ABSTRACT Introduction. Splenic rupture associated with thrombocytopenic purpura caused by infectious mononucleosis is extremely rare. The evolution of patients with infectious mononucleosis associated with Epstein-Barr virus is favorable, self-limiting and does not require specific therapeutic interventions. The symptoms are well tolerated and have a low frequency of complications. Case presentation. Female 12-year-old patient presenting with diffuse abdominal pain, distension, nausea, tegument pallor and un-quantified fever for two days. Upon admission to the emergency department, hemodynamic decompensation, purpuric lesions and ecchymosis in the limbs were observed. Laboratory and cabinet studies were carried out to confirm anemia, thrombocytopenia and splenic hematoma. Finally, an exploratory laparotomy was performed considering the possibility of hemoperitoneum. Results. The patient presented with splenomegaly, broken subcapsular hematoma, bleeding of 4000mL and accessory spleen lobe with splenic rupture. Conclusions. Spontaneous splenic rupture is a rare but possible complication of infectious diseases. However, its association with thrombocytopenic purpura is extremely rare.

RESUMEN Introducción. La rotura esplénica asociada a la presencia de purpura trombocitopénica causada por mononucleosis infecciosa es extremadamente rara; la evolución de los pacientes con mononucleosis infecciosa asociada al virus de Epstein-Barr es benigna y autolimitada y no requiere intervenciones terapéuticas específicas. El cuadro es bien tolerado y tiene una baja frecuencia de complicaciones. Presentación del caso. Paciente femenino de 12 años de edad con dos días de evolución de dolor abdominal difuso, distensión, náuseas, palidez de tegumentos y fiebre no cuantificada, quien a su ingreso al servicio de urgencias muestra datos de descompensación hemodinámica, lesiones purpúricas y manchas equimoticas en extremidades. Se realizan estudios de laboratorio y gabinete que confirman anemia, trombocitopenia y hematoma esplénico, por lo que se practica laparotomía exploradora ante la posibilidad de hemoperitoneo. Resultados. La paciente presenta esplenomegalia, hematoma subcapsular roto con sangrado de 4000mL y lóbulo accesorio de bazo con rotura esplénica. Conclusiones. La rotura espontánea del bazo es una complicación infrecuente pero posible en enfermedades infecciosas; sin embargo, su asociación a purpura trombocitopenica es extremadamente rara.

AGENESIS OF THE GALLBLADDER AND CHOLEDOCHOLITHIASIS. CASE REPORT

ABSTRACT Introduction. The most frequent elective surgery in General Surgery is the gallbladder surgery (cholecystectomy) in General Surgery in adults. There are many abnormalities of the gallbladder and the common bile duct. The most uncommon case is gallbladder agenesis. It could be difficult even for a experimented surgeon. It's the most erratic biliar duct malformation, and there are near 500 cases reported.(l) Case presentation. We present a case report of a 44 years old female patient, with abdominal pain in right superior quadrant, history of jaundice and acholia, with higher hepatic enzymes and direct bilirubin, with high probability of Choledocholithiasis. The images had not finding of the gallbladder (ultrasonography, Magnetic Resonance). The endoscopic retrograde colangiopancreatography (ERCP) was done, without removal of lithiasis and it used stent. Finally we did laparoscopy common bile duct exploration, and the surgery confirmation of agenesis of the gallbladder, with mecanic lithotripsy, and the success with total resolution of the patology in the posterior medical control. Conclusion. Agenesis of the gallbladder is a rare pathology that not many surgeons have the opportunity to treat. However, a surgeon must be prepared for any malformation and anatomical variant.

RESUMEN Introducción. Una de las cirugías electivas que más desarrolla el cirujano general en adultos, es la colecistectomía. Sin embargo, el cirujano debe estar preparado para múltiples hallazgos, entre ellas las malformaciones. El caso más exótico que puede encontrar el mismo, es la agenesia de la vesícula biliar, el cual puede desorientar completamente a un cirujano incluso experimentado, debido a que es la malformación con más baja incidencia de las vías biliares y sólo hay cerca de 500 casos reportados en la literatura.1 Presentación del caso. Se presenta el caso de una paciente de 44 años, con cuadro clínico de dolor abdominal en cuadrante superior derecho, historia clínica de ictericia y acolia, con elevación del perfil hepático (hiperbilirrubinemia directa) y alta probabilidad de coledocolitiasis. En los estudios imagenológicos (Ultrasonografia y Resonancia Nuclear Magnética de Vías biliares), no hubo hallazgo de vesícula biliar. Por ende, se realizó la colangiografía pancreática retrograda endoscópica (CPRE) en la cual no se logró la extracción de cálculos, y requirió uso de Endoprótesis. Finalmente, el tratamiento derivó a exploración de Vías biliares por laparoscopia, en dónde se confirmó el hallazgo de agenesia de vía biliar sospechado por la Resonancia Magnética y ecografías previas, se realizó entonces litotripsia mecánica dirigida con resolución completa del cuadro clínico. Y seguimiento posterior exitoso, con mejoría de la sintomatología inicial de la paciente. Conclusión. La agenesia vesicular una patología extraordinaria que incluso el cirujano general no se pueda encontrar alguna vez en su vida. Sin embargo, este debe estar preparado para todas las malformaciones y variantes anatómicas.

CONGENITAL SACCULAR CYST OF THE LARYNX. CASE REPORT AND LITERATURE REVIEW

ABSTRACT Introduction: Congenital saccular cyst is a rare but benign lesion, caused by a dilated laryngeal sac full of mucus that does not communicate with the laryngeal lumen. Its definitive treatment is surgical according to the literature. Objective: To review the literature and report a case of congenital laryngeal saccular cyst, as well as its treatment by endoscopic approach and radiofrequency, which is most easily found in our country. Materials and methods: Presentation of a case report and literature review in PubMed and Tripdatabase using the described keywords. Results: This is a rare condition with an incidence of 1.82 cases per 100 000 live births. Diagnosis is achieved by laryngeal endoscopy, images or clinical review. The case reported here corresponds to a newborn patient with respiratory distress and stridor, who was diagnosed with laryngeal saccular cyst that was resected surgically by means of endoscopy and radiofrequency, with no subsequent recurrence. Discussion: Understanding this disease is highly important to achieve proper diagnosis and provide treatment using the resources available in our country, such as radiofrequency. Conclusions: Despite the lack of case reports, knowing the characteristics of congenital saccular cyst is necessary to indicate proper treatment based on the available resources. It is possible to perform endoscopic resection of this lesion if it is <3cm by means of radiofrequency, which is a safe and effective method.

RESUMEN Introducción. El quiste sacular congénito es una lesión rara, pero benigna, dada por la dilatación del sáculo laríngeo que se llena de moco y que no se comunica con el lumen laríngeo. Según la literatura, su tratamiento definitivo es quirúrgico. Objetivo. Realizar una revisión de la literatura y el reporte de un caso de quiste sacular laríngeo congénito y su tratamiento mediante abordaje endoscópico y uso de radiofrecuencia. Materiales y métodos. Se realizó la presentación del caso clínico y se llevó a cabo una revisión de la literatura en las bases de datos de Pubmed y Tripdatabase usando palabras claves descritas. Resultados. Esta patología es escasa, con una incidencia de 1.82 casos por 100 000 nacidos vivos. El diagnostico se realiza por medio de endoscopia laríngea, imágenes o revisión de la clínica. El caso expuesto es de una paciente recién nacida que presenta estridor y dificultad respiratoria y es diagnosticada con quiste sacular laríngeo, el cual es resecado de forma quirúrgica por medio de endoscopia con uso de radiofrecuencia. El procedimiento da como resultado la no reaparición del quiste. Conclusiones. Pese a no existir muchos reportes de caso, hay que conocer las características del quiste sacular congénito para poder realizar el tratamiento adecuado con los recursos disponibles. Es posible realizar resección endoscópica de esta lesión si es <3cm por medio de radiofrecuencia, un método seguro y eficaz.

SPONTANEOUS PNEUMOMEDIASTINUM. CASE REPORT

ABSTRACT Introduction: Spontaneous pneumomediastinum (SPM) is defined as the presence of air in the mediastinum. It is a rare entity considered benign and self-limiting, which mostly affects young adults. Its diagnosis is confirmed through clinical and radiological studies. Case description: 21-year-old male patient with cough and greenish expectoration for four days, associated with dyspnea, chest pain, fever and bilateral supraclavicular subcutaneous emphysema. Chest X-ray suggested pneumomediastinum, which was confirmed by tomography. The patient was hospitalized for observation and treatment. After a positive evolution, he was discharged on the sixth day. Discussion: SPM is a differential diagnosis in patients with chest pain and dyspnea. Its prevalence is lower than 0.01% and its mortality rate is low. It should be suspected in patients with chest pain and subcutaneous emphysema on physical examination. Between 70 and 90% of the cases can be identified by chest X-ray, while confirmation can be obtained through chest tomography. In most cases it does not require additional studies. Conclusion: SPM is a little known cause of acute chest pain, and rarely considered as a differential diagnosis; it is self-limited and has a good prognosis.

ASCENDING AORTIC DISEASE IN A PATIENT WITH MARFAN SYNDROME

ABSTRACT Introduction. Acute thoracic aortic dissection is caused by a tear in the intimai lining of the aorta, and is a symptom of acute aortic syndrome. The dissection allows the blood to pass through the rupture and separates the tunica intima from the tunica media or the tunica adventitia, creating a false intravascular light. Early diagnosis directly affects the chances of survival, since it is a medical emergency that can lead to death, even with optimal treatment. Case description. The following report presents the case of a 26-year-old man with a history of Marfan syndrome, retrosternal lancinating pain, nausea, vomiting, and medium effort dyspnea that evolved to orthopnea, perioral cyanosis, murmur of aortic insufficiency and mitral systolic murmur. Complementary studies (chest x-ray, electrocardiogram, angiography, tomography, and echocardiogram) were performed, obtaining a diagnosis of Stanford type A ascending aortic dissection. Surgical treatment was indicated to replace the aortic root using a composite prosthesis and Bentall and De Bono coronary reconstruction. During the procedure, right coronary button destructuration occurred, so it was necessary to perform a venous bypass with a left internal saphenous venous hemoduct. Weaning extracorporeal circulation was achieved and then low expenditure of refractory character (despite vasopressor support at maximum dose), refractory ventricular fibrillation and asystole were observed. The patient did not recover and died as a consequence of acute transoperative myocardial infarction. Conclusion. The treatment for ascending aortic dissection remains a therapeutic challenge. Timely diagnosis is directly related to life expectancy in patients who suffer from this condition, hence the importance of proper diagnosis and management.

RESUMEN Introducción. La incidencia de síndrome de Marfan que ha sido reportada a nivel mundial es de 1 en 5000 casos, de los cuales aproximadamente el 80% o más desarrollan complicaciones cardiovasculares. La disección aórtica torácica aguda requiere una rotura en la íntima de la aorta, que forma parte del síndrome aórtico agudo. En la disección, la sangre pasa a través de la rotura y separa la íntima de la media o la adventicia, lo que crea una falsa luz intravascular. Un diagnóstico temprano incide directamente en las posibilidades de supervivencia, pues se trata de una emergencia médica que puede llevar a la muerte, incluso con un tratamiento óptimo. Descripción del caso. Se presenta el caso de un hombre de 26 años con antecedente de síndrome de Marfan, dolor retro esternal lancinante, náusea, vómito, disnea de medianos esfuerzos que evolucionó a ortopnea, cianosis peribucal, soplo de insuficiencia aórtica y soplo sistólico mitral. Se realizaron estudios complementarios (radiografía de tórax, electrocardiograma, angiografía, tomografía, eco-cardiograma) y se le diagnosticó disección de aorta ascendente tipo A Stanford, por lo que se decidió iniciar tratamiento quirúrgico mediante reemplazo de la raíz aórtica mediante prótesis compuesta y reconstrucción coronaria tipo Bentall y de Bono. En el procedimiento se presentó desestructuración de botón coronario derecho, por lo que fue necesario realizar puente venoso con hemoducto venoso de safena interno izquierdo. Se logró destete de circulación extra corpórea y luego se observó bajo gasto de carácter refractario (pese a soporte vasopresor a dosis máxima), fibrilación ventricular refractaria y asistolia; el paciente no mostró recuperación y falleció como consecuencia de un infarto agudo de miocardio transoperatorio. Conclusión. El tratamiento de la disección aórtica de aorta ascendente sigue siendo un desafío terapéutico. El diagnóstico oportuno tiene relación directa con la esperanza de vida de quienes lo padecen, de ahí su importancia diagnóstica y su manejo.

APENDICULAR CYSTIC DILATATION. CASE REPORT

ABSTRACT Introduction. Mucocele is a dilatation of the vermiform appendix characterized by viscous mucoid material secretion. Its incidence is low -0.2-0.3% to 0.7% of the total of the appendectomies-, therefore, it is considered as a rare entity, which affects mainly women with a ratio of 4:1. Diagnosis is often incidental, and its management is surgical based on histology. Clinical case. 69-year-old male patient who presented with abdominal pain of 5 days of evolution in the right iliac fossa, accompanied by anorexia, nausea and unquantified fever. Physical examination revealed positive Mc Burney's and Blumberg's signs, indicating peritonism. Appendectomy and appendiceal raffia were performed using the Parker-Kerr technique. Intraoperative findings included an appendicular tumor with a thick base and mucoid content. The histo-pathological study showed a cecal appendix structure with coagulative necrosis of the mucosa and the wall, as well as mucosa with focal deposit of mucoid material. The patient was discharged after 8 days without further complications. Conclusion: Studies on appendicular mucocele are scarce, and due to its complications, radiological controls at a shorter time interval, and even early surgical treatment, are necessary to avoid complications such as intestinal obstruction, peritoneal bleeding or pseudomyxoma.

RESUMEN Introducción. El mucocele es una dilatación del apéndice vermiforme que se caracteriza por su contenido mucoide viscoso. La incidencia de esta entidad es baja -entre el 0.2-0.3% y el 0.7% del total de las apendicectomías- por lo que se considera un caso raro, el cual afecta más a mujeres que a hombres, con una relación 4:1. Con frecuencia, el diagnóstico es incidental y su manejo es quirúrgico. Caso clínico. Paciente de 69 años de edad, de sexo masculino, que acude a emergencias debido a un cuadro clínico de dolor abdominal de 5 días de evolución en la fosa iliaca derecha. El dolor se acompaña de anorexia, náuseas y alza térmica no cuantificada. El examen físico revela signos de Mc Burney y Blumberg positivos e irritación peritoneal, por lo que se realiza apendicectomía más rafia de base apendicular. Entre los hallazgos intraoperatorios se observó tumoración apendicular con base ancha y de paredes gruesas, y contenido mucoide. El estudio histopatológico mostró una estructura de apéndice cecal con necrosis coagulativa en mucosa y pared, así como mucosa con depósito focal de material mucoide. El paciente fue dado de alta a los 8 días sin complicaciones. Conclusión: No existen estudios específicos que lleven a predecir el mucocele apendicular, ni estudios que alerten sobre la futura aparición del mismo; no obstante, conocer sus características es útil para sospecharlo en casos con cuadros similares. Debido a sus complicaciones, es necesario considerar controles radiológicos con un menor intervalo de tiempo e, incluso, un tratamiento quirúrgico precoz, con el objetivo de evitar complicaciones tales como obstrucción intestinal, hemorragia o pseudomixoma peritoneal.

DELUSIONAL INFESTATION. EKBOM'S SYNDROME IN A 53-YEAR OLD WOMAN. CASE REPORT

ABSTRACT Introduction: Delusional infestation is a rare psychiatric disorder defined as a condition in which the patient has the unshakable belief and perception of being infested with parasites. Its treatment is difficult, and frequently includes antipsychotic medications (such as olanzapine or aripiprazole). Non-pharmacological treatment, particularly psychotherapy, can be used for less severe cases. Dermatologists and psychiatrists must take a multi-disciplinary approach (preferably in a psycodermatology dedicated clinic) since this type of patients sometimes refuse treatment. Case description: A 53-year-old female businesswoman describes a clinical history of five years of visual hallucinations, depressive symptoms, and generalized pruritus, along with the use of toxic substances to "clean" her skin and cloths. She reports similar symptoms in some relatives but they were not evaluated. Blood tests and analyses of the "specimen" brought by the patient were performed, yielding negative results. The patient had never been assessed by any specialist, and showed disoriented during the consultation. Follow-up was not possible due to the reluctance of the patient to follow the indications and seek psychiatric treatment. Moreover, the patient did not respond to further communication attempts. Discussion: Delusional infestation is an uncommon disease that endangers the patients and the people around them. Its treatment is difficult and long, and not conducting proper follow-up is a great risk. Its prevalence and incidence is variable and generally unknown. It can affect the patient, their next of kin, pets or the environment, and the "pathogen" can be a living organism or an inanimate object. Conclusion: This case is important as it shows the hardships of treatment, adequate follow-up and care, as well as the need to improve how these patients are approached. Additionally, both classical and uncommon signs and symptoms could be observed as the patient stated that her relatives were affected (possible delusional infestation by proxy).