Tratamiento quirúrgico de la estenosis traqueal adquirida en pediatría / Surgical treatment of acquired tracheal stenosis in pediatrics

La estenosis traqueal adquirida es muy poco frecuente en la edad pediátrica y puede responder a múltiples causas; la estenosis posintubación y la secundaria a traqueotomía son las más comunes.El manejo de la estenosis traqueal adquirida sigue siendo controvertido. El tratamiento endoscópico proporciona el alivio inmediato de los síntomas, pero se asocia a una alta tasa de recurrencia, y el uso de prótesis endoluminales puede incrementar la longitud de la estenosis y complicar una cirugía futura. Por el contrario, se han documentado buenos resultados a largo plazo con la resección traqueal con anastomosis primaria.Se describe nuestra experiencia con la resección traqueal con anastomosis término-terminal para el tratamiento de la estenosis traqueal posintubación en 8 pacientes.

Acquired tracheal stenosis is a very infrequent lesion in the pediatric age group and may be due to diverse causes, being post-intubation stenosis and stenosis secondary to tracheostomy the most common ones.The management of acquired tracheal stenosis remains controversial. Endoscopic treatment provides immediate relief of symptoms but it is associated with a high recurrence rate, and the use of endoluminal prostheses can increase the length of the stricture complicating future surgery. Conversely, good long-term results have been documented with tracheal resection and primary anastomosis.We describe our experience with tracheal resection followed by end-to-end anastomosis for the treatment of post-intubation tracheal stenosis in 8 patients.

[Surgical treatment of acquired tracheal stenosis in pediatrics]. / Tratamiento quirúrgico de la estenosis traqueal adquirida en pediatría.

Acquired tracheal stenosis is a very infrequent lesion in the pediatric age group and may be due to diverse causes, being post-intubation stenosis and stenosis secondary to tracheostomy the most common ones. The management of acquired tracheal stenosis remains controversial. Endoscopic treatment provides immediate relief of symptoms but it is associated with a high recurrence rate, and the use of endoluminal prostheses can increase the length of the stricture complicating future surgery. Conversely, good long-term results have been documented with tracheal resection and primary anastomosis. We describe our experience with tracheal resection followed by end-to-end anastomosis for the treatment of post-intubation tracheal stenosis in 8 patients.

La estenosis traqueal adquirida es muy poco frecuente en la edad pediátrica y puede responder a múltiples causas; la estenosis posintubación y la secundaria a traqueotomía son las más comunes. El manejo de la estenosis traqueal adquirida sigue siendo controvertido. El tratamiento endoscópico proporciona el alivio inmediato de los síntomas, pero se asocia a una alta tasa de recurrencia, y el uso de prótesis endoluminales puede incrementar la longitud de la estenosis y complicar una cirugía futura. Por el contrario, se han documentado buenos resultados a largo plazo con la resección traqueal con anastomosis primaria. Se describe nuestra experiencia con la resección traqueal con anastomosis término-terminal para el tratamiento de la estenosis traqueal posintubación en 8 pacientes.

Poroqueratosis de Mibelli en la edad pediátrica / Porokeratosis of Mibelli in pediatric age

Las poroqueratosis son un grupo heterogéneo de trastornos de la queratinización epidérmica, de presentación infrecuente. Se caracterizan clínicamente por pápulas hiperqueratósicas que confluyen y forman placas anulares con un centro atrófico y bordes sobreelevados. Hay seis variantes clínicas. El examen histopatológico evidencia la característica laminilla cornoide. Se presenta el caso de una niña de 2 años con diagnóstico de poroqueratosis de Mibelli y respuesta parcial al tratamiento tópico con tretinoína al 0,025%, que actualmente continúa en seguimiento clínico (AU)

Porokeratosis is a heterogeneous group of disorders in epidermal keratinization. It is an infrequent entity characterized clinically by hyperkeratotic papules that converge forming annular plaques with an atrophic center and raised borders. There are six clinical variants. The histopathological examination evidences the typical cornoid lamella. We present a 2-year-old girl diagnosed with porokeratosis of Mibelli and partial response to topical treatment of tretinoin 0,025%, still under clinical control (AU)

Dermatofibrosarcoma protuberans: lo diagnosticamos en la edad pediátrica / Dermatofibrosarcoma protuberans: we diagnose it in the pediatric age

El dermatofibrosarcoma protuberans (DFSP) es un tumor cutáneo, de baja frecuencia, fibrohistiocítico, infiltrante, de lento crecimiento, de agresividad local, de malignidad intermedia; con escasas probabilidades de metástasis pero con alto índice de recurrencia local. El diagnóstico debe sospecharse y confirmarse con histología e inmunohistoquímica. El tratamiento de elección es con cirugía convencional y/o cirugía micrográfica de Mohs, con márgenes de 2-4 cm. Se considera que la prevalencia del DFSP en la edad pediátrica es baja, debido al escaso índice de sospecha. En el presente trabajo compartimos cinco casos de DFSP en la edad pediátrica, estudiados en el Hospital General de Niños Pedro de Elizalde. (AU)

Dermatofibrosarcoma protuberans (DFSP) is a cutaneous, low frequency, fibrohistiocytic, infiltrating, slow growing, local aggressiveness, intermediate malignancy tumor; with little chance of metastasis but with a high rate of local recurrence. The diagnosis should be suspected and confirmed with histology and immunohistochemistry. The treatment of choice is with conventional surgery and / or Mohs micrographic surgery, with margins of 2-4 cm. The prevalence of DFSP in pediatric age is considered to be low, due to the low index of suspicion. In this paper we share five cases of DFSP in the pediatric age, studied at the Pedro de Elizalde Children's General Hospital. (AU)

Pilomatrixomas en niños: a propósito de 149 casos. Estudio retrospectivo en dos centros pediátricos / Pilomatrixomas in children: Report of 149 cases. A retrospective study at two children's hospitals

El pilomatrixoma es un tumor benigno cutáneo, de frecuente aparición en los niños, que se origina en las células de la matriz del folículo piloso. Se presenta como un nódulo o tumor, aproximadamente, de 0,5 a 3 cm de tamaño, de consistencia cálcica, de bordes facetados, con piel suprayacente eritematoazulada. En ocasiones, la piel puede atrofiarse, dar la apariencia de una ampolla e, incluso, extruir material cálcico. El diagnóstico se sospecha por la presentación clínica característica y los estudios de imágenes complementarios. El diagnóstico definitivo es por los hallazgos histológicos. La extirpación quirúrgica es el tratamiento de elección. Se presentan los datos epidemiológicos, clínicos, estudios complementarios y técnicas quirúrgicas empleadas de 149 pilomatrixomas resecados en 137 pacientes tratados en los Servicios de Dermatología de los hospitales de pediatría Dr. Pedro de Elizalde y Prof. Dr. Juan P. Garrahan.

A pilomatrixoma is a benign skin tumor common in children, which develops from the matrix cells of hair follicles. It presents as a nodule or tumor of approximately 0.5-3 cm in size, with calcium-like consistency, faceted edges, and blue erythematous overlying skin. Sometimes, the skin may atrophy and look like a blister or even extrude calcium. Diagnosis is suspected based on the typical clinical presentation and supplementary imaging tests. The definite diagnosis is made according to histological findings. Surgical removal is the treatment of choice. Here we describe the epidemiological and clinical data, supplementary tests, and surgical techniques in relation to 149 pilomatrixomas resected in 137 patients treated at the Departments of Dermatology of two children's hospitals: Dr. Pedro de Elizalde and Prof. Dr. Juan P. Garrahan.

Pilomatrixomas in children: Report of 149 cases. A retrospective study at two children's hospitals. / Pilomatrixomas en niños: a propósito de 149 casos. Estudio retrospectivo en dos centros pediátricos.

A pilomatrixoma is a benign skin tumor common in children, which develops from the matrix cells of hair follicles. It presents as a nodule or tumor of approximately 0.5-3 cm in size, with calcium-like consistency, faceted edges, and blue erythematous overlying skin. Sometimes, the skin may atrophy and look like a blister or even extrude calcium. Diagnosis is suspected based on the typical clinical presentation and supplementary imaging tests. The definite diagnosis is made according to histological findings. Surgical removal is the treatment of choice. Here we describe the epidemiological and clinical data, supplementary tests, and surgical techniques in relation to 149 pilomatrixomas resected in 137 patients treated at the Departments of Dermatology of two children's hospitals: Dr. Pedro de Elizalde and Prof. Dr. Juan P. Garrahan.

El pilomatrixoma es un tumor benigno cutáneo, de frecuente aparición en los niños, que se origina en las células de la matriz del folículo piloso. Se presenta como un nódulo o tumor, aproximadamente, de 0,5 a 3 cm de tamaño, de consistencia cálcica, de bordes facetados, con piel suprayacente eritematoazulada. En ocasiones, la piel puede atrofiarse, dar la apariencia de una ampolla e, incluso, extruir material cálcico. El diagnóstico se sospecha por la presentación clínica característica y los estudios de imágenes complementarios. El diagnóstico definitivo es por los hallazgos histológicos. La extirpación quirúrgica es el tratamiento de elección. Se presentan los datos epidemiológicos, clínicos, estudios complementarios y técnicas quirúrgicas empleadas de 149 pilomatrixomas resecados en 137 pacientes tratados en los Servicios de Dermatología de los hospitales de pediatría Dr. Pedro de Elizalde y Prof. Dr. Juan P. Garrahan.

Pancreatoblastoma in a paediatric patient: anatomo-pathological aspects of a case with multiple hepatic metastases.

Pancreatoblastoma is a rare paediatric malignant neoplasm. The treatment of choice is complete surgical resection. However, it is often unresectable due to its large size, local infiltration or distant metastasis. Since the condition is rare, there is currently no standard treatment regimen. We outline the case of a 4-year-old child who presented with abdominal pain and distention, together with an enlarged liver and elevated serum α-fetoprotein levels. Imaging studies showed the presence of an abnormal pancreatic tumour and multiple nodular lesions in the liver, the biopsies from which led to a diagnosis of pancreatoblastoma. In this case, the patient received cycles of neoadjuvant chemotherapy, combining cisplatin and doxorubicin. The patient subsequently underwent scheduled surgery in which the primary pancreatic lesion was resected, obtaining a circumscribed and nodular specimen measuring 7 × 6 cm and weighing 150 g. Given the extent of the metastasis, the child is currently awaiting a liver transplant.

Lipoblastoma retroperitoneal gigante: Caso dínico / Giant retroperitoneal lipoblastoma: Case report

El lipoblastoma forma parte de los tumores derivados del tejido adiposo. Es una neoplasia benigna que se puede presentar de forma localizada o difusa, que afecta a niños en la primera infancia y que, generalmente, se localiza en el tronco y las extremidades. Estos tumores son poco frecuentes en la edad pediátrica, pero cuando uno de ellos se presenta, constituye un desafío que se debe resolver rápidamente por el riesgo de malignidad que implican otras lesiones neoplásicas, de similares características. El diagnóstico preoperatorio es dificultoso, ya que los métodos por imágenes no aportan información específica que permita diferenciarlos de otros tumores, como los liposarcomas. El diagnóstico definitivo es anatomopalógico y, en casos dudosos, es necesaria la confirmación citogenética. Se presenta el caso de un niño de 14 meses de edad con diagnóstico de lipoblastoma de localización retroperitoneal de gran tamaño.

Lipoblastoma is part of tumors derived from adipose tissue. It is a benign neoplasm that can be localized or diffuse, affecting children in early childhood and usually located in the trunk and extremities. These tumors are uncommon in the pediatric age, but when one of them is present, it is a challenge that we must resolve quickly due to the risk of malignancy that involves other similar neoplastic lesions. The preoperative diagnosis is difficult, because the imaging methods do not provide specific information that allows us to differentiate them from other tumors, such as liposarcomas. The definitive diagnosis is anatomopathological and, in doubtful cases, cytogenetic confirmation is necessary. We present the case of a 14-month-old boy with diagnosis of large sized lipoblastoma of retroperitoneal localization.

[Giant retroperitoneal lipoblastoma. Case report]. / Lipoblastoma retroperitoneal gigante. Caso dínico.

Lipoblastoma is part of tumors derived from adipose tissue. It is a benign neoplasm that can be localized or diffuse, affecting children in early childhood and usually located in the trunk and extremities. These tumors are uncommon in the pediatric age, but when one of them is present, it is a challenge that we must resolve quickly due to the risk of malignancy that involves other similar neoplastic lesions. The preoperative diagnosis is difficult, because the imaging methods do not provide specific information that allows us to differentiate them from other tumors, such as liposarcomas. The definitive diagnosis is anatomopathological and, in doubtful cases, cytogenetic confirmation is necessary. We present the case of a 14-month-old boy with diagnosis of large sized lipoblastoma of retroperitoneal localization.

El lipoblastoma forma parte de los tumores derivados del tejido adiposo. Es una neoplasia benigna que se puede presentar de forma localizada o difusa, que afecta a niños en la primera infancia y que, generalmente, se localiza en el tronco y las extremidades. Estos tumores son poco frecuentes en la edad pediátrica, pero cuando uno de ellos se presenta, constituye un desafío que se debe resolver rápidamente por el riesgo de malignidad que implican otras lesiones neoplásicas, de similares características. El diagnóstico preoperatorio es dificultoso, ya que los métodos por imágenes no aportan información específica que permita diferenciarlos de otros tumores, como los liposarcomas. El diagnóstico definitivo es anatomopalógico y, en casos dudosos, es necesaria la confirmación citogenética. Se presenta el caso de un niño de 14 meses de edad con diagnóstico de lipoblastoma de localización retroperitoneal de gran tamaño.

Malignant extra-adrenal pelvic paraganglioma in a paediatric patient.

The extra-adrenal paraganglioma is a neoplasm originating in regional structures, uncommon in paediatrics. We report on a case of a 13-year-old patient who began with severe arterial hypertension, tachycardia, dilated cardiomyopathy and elevated levels of catecholamines in the blood and urine. The presence of a retrovesical pelvic mass in contact with the right vaginal dome was determined by imaging studies. A diagnosis of malignant extra-adrenal pelvic paraganglioma with lymph node metastases was reached through biopsy and the surgical resection of subsequent local recurrences. Paragangliomas are usually located in the paravertebral zones from the base of the skull to the retroperitoneum and are benign in 90% of cases. This kind of neoplasia is uncommon in paediatrics, especially those located in the pelvis. In cases of masses of a gynaecological origin, a differential diagnosis should be considered, and a histological and immunohistochemical study is essential in certifying the diagnosis.

Dr. Julio V. Uriburu. Su destacada participación en la micología nacional / Dr. Julio V. Uriburu. His outstanding participation in the national mycology

Destacado dermatólogo, el doctor Julio V. Uriburu propició el estudio de las afecciones micóticas en nuestro medio. Realizamos una breve actualización del diagnóstico y tratamiento de tiñas de cuero cabelludo.

Dr. Julio V. Uriburu was a very important specialist in skin disorders. He worked in the investigation of micotics infections of hair and scalp disorders. We did a review of diagnostic and treatment of tinea capitis.

[Childhood pemphigus vulgaris, a case report]. / Pénfigo vulgar de la infancia, a propósito de un caso.

Pemphigus vulgaris is a chronic disease that is characterized by flaccid blisters and erosions, caused by immunoglobulin G autoantibodies against epidermal components. It is highly unusual in the pediatric population; it represents the 1.4 - 2.9% of the total cases. We present a case of pemphigus vulgaris in a 12 year old girl, which has implied a challenge both in its diagnosis and in its treatment.

El pénfigo vulgar es una enfermedad crónica que se caracteriza por ampollas fláccidas y erosiones, causadas por un fenómeno autoinmune con autoanticuerpos inmunoglobulina G circulantes contra componentes de la epidermis. Su presentación en la infancia es altamente infrecuente; representa el 1,4%-2,9% de todos los casos de pénfigo vulgar. Presentamos un caso de pénfigo vulgar en una niña de 12 años, que implicó un desafío en su diagnóstico y terapéutica.

[Protein-losing enteropathy due to intestinal lymphangiectasis: a rare disease. Report of two cases]. / Enteropatía perdedora de proteínas por linfangiectasia intestinal: una enfermedad rara. Presentación de 2 casos.

Congenital intestinal lymphangiectasis (LIP) is a protein-losing enteropathy that appears sporadically in children. It begins with edema due to hypoproteinemia and hypoalbuminemia, and in some cases with ascites, immunodeficience and hypocalcemic tetania. The purpose of this report is to present two patients with LIP which appeared during the first year of life. The diagnosis was certificated by upper gastrointestinal videoendoscopy and histological findings. Both patients were treated with a new formula containing mean chain triglycerides with an adequate response, not obtained before with a common semielemental formula.

[Congenital esophageal stenosis: a case report]. / Estenosis esofágica congénita: A propósito de un caso.

Congenital esophageal stenosis, a rare disease of unknown cause which reports have increased in the last few years, requires a high index of suspicion for its diagnosis and treatment. It can be classified in three types based on the etiology of the stenosis: tracheobronchial rest, fibromuscular hypertrophy and membranous diaphragm. Symptoms may vary depending on location and severity of the stenosis. Treatment options are based on clinical suspicion of the histologic type and they can be balloon dilation or surgical resection of the stenotic segment. The definitive diagnosis is the histological study.

Pie de madura: primer caso en nuestro país y breve actualización (1904-2013) / Foot of madura: first case in our country and brief update (1904-2013)

Presentamos el primer caso de pie de madura publicado en Argentina y realizamos una breve actualización de su diagnóstico y terapéutica.

We presented the first case of feet of Madura published in Argentine. We did a review of diagnostic and treatment.

Estenosis esofágica congénita: A propósito de un caso. / [Congenital esophageal stenosis: a case report].

Congenital esophageal stenosis, a rare disease of unknown cause which reports have increased in the last few years, requires a high index of suspicion for its diagnosis and treatment. It can be classified in three types based on the etiology of the stenosis: tracheobronchial rest, fibromuscular hypertrophy and membranous diaphragm. Symptoms may vary depending on location and severity of the stenosis. Treatment options are based on clinical suspicion of the histologic type and they can be balloon dilation or surgical resection of the stenotic segment. The definitive diagnosis is the histological study.

Estenosis esofágica congénita: A propósito de un caso / [Congenital esophageal stenosis: a case report].

Congenital esophageal stenosis, a rare disease of unknown cause which reports have increased in the last few years, requires a high index of suspicion for its diagnosis and treatment. It can be classified in three types based on the etiology of the stenosis: tracheobronchial rest, fibromuscular hypertrophy and membranous diaphragm. Symptoms may vary depending on location and severity of the stenosis. Treatment options are based on clinical suspicion of the histologic type and they can be balloon dilation or surgical resection of the stenotic segment. The definitive diagnosis is the histological study.

[Cat scratch disease as fever of unknown origin]. / Enfermedad por arañazo de gato como causa de fiebre de origen desconocido.

The cat scratch disease is a relatively rare disease caused by Bartonella henselae. Only a small proportion of the cases that described extranodal involvement occurs. We report a 11 year old male patient with persistent fever, liver and osteoarticular involvement with a predominance of dorsolumbar spine. Evolution was satisfactory with complete clinical and imaging evidenced recovery in several months of monitoring. Case was analyzed considering EAG published literature, emphasizing the need to include this condition in the differential diagnoses in patients with bone involvement suggestive of systemic disease.

Síndrome de Wells por picadura de insecto en lactante / Wells Syndrome by insect bite in infant

El síndrome de Wells es una dermatosis de etiología desconocida. Clásicamente se presenta con pápulas o placas eritematoedematosas recidivantes, aunque existen otras variantes clínicas. Cada brote evoluciona en 2 a 4 semanas y suele dejar hiperpigmentación residual. Puede ser desencadenado por factores ambientales, entre los que se menciona la picadura de insecto. Se acompaña de eosinofilia en forma variable. El tratamiento es sintomático, con corticoides tópicos o sistémicos y antihistamínicos, pero los casos refractarios requieren una terapéutica más agresiva y la respuesta es diversa. Presentamos un caso de síndrome de Wells causado por la picadura de un insecto...

Wells syndrome is a dermatosis of unknown etiology. It classically presents with recurrent erythematous papules or plaques, but other clinical variants exist. Each bud develops in 2-4 weeks and often leaves residual hyperpigmentation. They can be triggered by various environmental factors, among which is mentioned insect bite. It is variably accompanied by eosinophilia. Treatment is mainly symptomatic, with topical or systemic corticosteroids and antihistamines, although refractory cases require a more aggressive therapeutic and their response is variable. We present a case of Wells syndrome triggered by an insect bite...

Enfermedad por arañazo de gato como causa de fiebre de origen desconocido / Cat scratch disease as fever of unknown origin

The cat scratch disease is a relatively rare disease caused by Bartonella henselae. Only a small proportion of the cases that described extranodal involvement occurs. We report a 11 year old male patient with persistent fever, liver and osteoarticular involvement with a predominance of dorsolumbar spine. Evolution was satisfactory with complete clinical and imaging evidenced recovery in several months of monitoring. Case was analyzed considering EAG published literature, emphasizing the need to include this condition in the differential diagnoses in patients with bone involvement suggestive of systemic disease.

La enfermedad por arañazo de gato (EAG) es una enfermedad infecciosa aguda usualmente benigna, relativamente común, causada por Bartonella henselae. De los casos descritos una pequeña proporción presenta compromiso extranodal. Presentamos el caso clínico de un niño de 11 años con un sindrome febril prolongado, compromiso hepático y óseo múltiple, con predominio en la columna vertebral dorsolumbar. Su evolución fue satisfactoria con resolución clínica y de las imágenes, después de varios meses de seguimiento. Se realiza una revisión de la bibliografía sobre EAG, enfatizando la necesidad de incluir esta enfermedad en el diagnóstico diferencial en pacientes con síndrome febril prolongado con compromiso óseo sugerente de una enfermedad sistémica.