Treatment of balanitis xerotica obliterans in pediatric patients. / Tratamiento de la balanitis xerótica obliterante en pacientes pediátricos.

OBJECTIVES: Balanitis xerotica obliterans (BXO) is a chronic inflammatory disease with a little known incidence in pediatric population. The objective of this work was to describe our experience in the treatment of BXO. MATERIALS AND METHODS: Retrospective study carried out in 419 patients undergoing circumcision surgery between January 2014 and January 2017. Demographic, clinical, therapeutic, and anatomical and pathological variables, as well as complications during follow-up, were analyzed. RESULTS: Of the 419 patients, 41 (9.78%) were diagnosed with BXO. 6 patients were excluded owing to lack of follow-up, so 35 patients were analyzed. Mean age at diagnosis was 8.6 years. Suspicion diagnosis was clinical at physical exploration in 17 patients (48.6%), and at surgery in 18 patients (51.4%). Anatomical and pathological confirmation was performed in a total 35 patients (100%). During follow-up, 6 patients (17.14%) had lesions in the glans, 3 (8.57%) in the urethra, and 9 (25.71%) in both. 6 meatotomies (17.14%) and 5 new circumcisions (14.28%) had to be carried out. Mean recurrence time was 32.43 months. In 19 patients (54.28%), topical corticoids - ointment - were applied, and 1 patient (2.85%) received topical immunosuppressants. CONCLUSIONS: A close follow-up of patients with clinical or anatomical and pathological diagnosis of BXO is required given its high morbidity. The complications described in pediatric population include meatal and urethral stenosis, as well as recurrent phimosis, unless a sufficient amount of foreskin is resected.

OBJETIVOS: La balanitis xerótica obliterante (BXO) es una enfermedad crónica inflamatoria de incidencia poco conocida en la población pediátrica. El objetivo de este trabajo es describir nuestra experiencia en el tratamiento de las BXO. MATERIAL Y METODOS: Estudio retrospectivo de 419 pacientes intervenidos de circuncisión en el periodo comprendido entre enero de 2014 y enero de 2017. Analizamos variables demográficas, clínicas, anatomopatológicas, terapéuticas y complicaciones durante el seguimiento. RESULTADOS: De los 419 pacientes, 41 fueron diagnosticados de BXO (9,78%). Seis pacientes fueron excluidos por falta de seguimiento, por lo que se analizaron 35 pacientes. La media de edad al diagnóstico fue de 8,6 años. El diagnóstico de sospecha fue clínico durante la exploración física en 17 pacientes (48,6%) y durante la intervención en 18 (51,4%), realizando la confirmación anatomopatológica en un total de 35 pacientes (100%). Durante el seguimiento 6 pacientes (17,14%) presentaron lesiones en glande, 3 (8,57%) en uretra y 9 (25,71%) en ambas localizaciones, siendo necesaria la realización de 6 meatotomías (17,14%) y de nueva circuncisión en 5 (14,28%). El tiempo medio de recidiva fue de 32,43 meses. En 19 pacientes (54,28%) se aplicaron corticoides tópicos en pomada y en 1 paciente inmunosupresores tópicos (2,85%). CONCLUSIONES: Es necesario un seguimiento estrecho de los pacientes con diagnóstico clínico o anatomopatológico de BXO dada su elevada morbilidad. Las principales complicaciones descritas en la población pediátrica son la estenosis meatal y uretral, así como la recidiva de la fimosis si no se reseca el prepucio suficientemente.

Tratamiento de la balanitis xerótica obliterante en pacientes pediátricos / Treatment of balanitis xerotica obliterans in pediatric patients

Objetivos: La balanitis xerótica obliterante (BXO) es una enferme-dad crónica inflamatoria de incidencia poco conocida en la población pediátrica. El objetivo de este trabajo es describir nuestra experiencia en el tratamiento de las BXO. Material y métodos: Estudio retrospectivo de 419 pacientes intervenidos de circuncisión en el periodo comprendido entre enero de 2014 y enero de 2017. Analizamos variables demográficas, clínicas, anatomopatológicas, terapéuticas y complicaciones durante el seguimiento. Resultados: De los 419 pacientes, 41 fueron diagnosticados de BXO (9,78%). Seis pacientes fueron excluidos por falta de seguimiento, por lo que se analizaron 35 pacientes. La media de edad al diagnóstico fue de 8,6 años. El diagnóstico de sospecha fue clínico durante la exploración física en 17 pacientes (48,6%) y durante la intervención en 18 (51,4%), realizando la confirmación anatomopatológica en un total de 35 pacientes (100%). Durante el seguimiento 6 pacientes (17,14%) presentaron lesiones en glande, 3 (8,57%) en uretra y 9 (25,71%) en ambas localizaciones, siendo necesaria la realización de 6 meatotomías (17,14%) y de nueva circuncisión en 5 (14,28%). El tiempo medio de recidiva fue de 32,43 meses. En 19 pacientes (54,28%) se aplicaron corticoides tópicos en pomada y en 1 paciente inmunosupresores tópicos (2,85%). Conclusiones: Es necesario un seguimiento estrecho de los pacientes con diagnóstico clínico o anatomopatológico de BXO dada su elevada morbilidad. Las principales complicaciones descritas en la población pediátrica son la estenosis meatal y uretral, así como la recidiva de la fimosis si no se reseca el prepucio suficientemente

Objectives. Balanitis xerotica obliterans (BXO) is a chronic inflam-matory disease with a little known incidence in pediatric population. The objective of this work was to describe our experience in the treatment of BXO. Materials and methods: Retrospective study carried out in 419 patients undergoing circumcision surgery between January 2014 and January 2017. Demographic, clinical, therapeutic, and anatomical and pathological variables, as well as complications during follow-up, were analyzed. Results: Of the 419 patients, 41 (9.78%) were diagnosed with BXO. 6 patients were excluded owing to lack of follow-up, so 35 patients were analyzed. Mean age at diagnosis was 8.6 years. Suspicion diagnosis was clinical at physical exploration in 17 patients (48.6%), and at surgery in 18 patients (51.4%). Anatomical and pathological confirmation was performed in a total 35 patients (100%). During follow-up, 6 patients (17.14%) had lesions in the glans, 3 (8.57%) in the urethra, and 9 (25.71%) in both. 6 meatotomies (17.14%) and 5 new circumcisions (14.28%) had to be carried out. Mean recurrence time was 32.43 months. In 19 patients (54.28%), topical corticoids - ointment - were applied, and 1 patient (2.85%) received topical immunosuppressants. Conclusions: A close follow-up of patients with clinical or anatomical and pathological diagnosis of BXO is required given its high morbidity. The complications described in pediatric population include meatal and urethral stenosis, as well as recurrent phimosis, unless a sufficient amount of foreskin is resected

[Multicystic Kidney: is there any new scientific evidence to recommend nephrectomy?]. / Riñón multiquístico: existe alguna nueva evidencia científica que recomienda la nefrectomía?

With the aim to create a scientific evidence of the convenience or not of removing the Multicystic Kidney (MK), a systematic review has been done over the last 20 years, selecting those articles with determinant criterions. Our experience has been also evaluated. We have obtained an evidence table of 1082 MK, with a follow-up from 2 to 7 years. No case degenerated. The complications were: urinary tract infection (UTI) in 5% and hipertensión (HTA) in 0.7%. In our serie (68 cases): nephrectomy was done in 10 cases. 82% completely involved (66.6% before 5 years and 15.6% from 5 to 15 years of follow-up). 18% involved partially. No case degenerated. The complications were UTI (6 cases) and HTA in one. Periodical ultrasound follow-up is our recommendation for MK due to the results of our serie and from the systematic review of the literature.

Riñón multiquístico: ¿existe alguna nueva evidencia científica que recomienda la nefrectomía? / Multicystic kidney: is there any new scientific evidence to recommend nefrectomy?

Con el fin de tratar de crear evidencia científica sobre la conveniencia o no de la extirpación del riñón multiquístico (RM), se ha realizado una revisión sistemática de la bibliografía de las últimas 2 décadas, seleccionando los estudios con determinados criterios. Asimismo se analiza nuestra casuística en los últimos 20 años. Se ha obtenido una tabla de evidencia con 1.082 casos de RM, con un seguimiento entre 2 y 7 años. Ningún caso malignizó. Las complicaciones han sido infección urinaria (IU) en un 5% e hipertensión arterial (HTA) en un 0,7%. En nuestra serie (68 casos) se realizó nefrectomía en 10 casos. El 82% desaparecieron totalmente (66,6% antes de los 5 años y 15,6% entre los 5 y 15 años de seguimiento). El 18% involucionó parcialmente. No ha aparecido ningún caso de malignización, limitándose las complicaciones a IU (6) e HTA en un caso. El seguimiento ecográfico periódico en el RM continúa siendo la aproximación terapéutica más razonable según los resultados de nuestra serie y de la revisión sistemática de la literatura relacionada (AU)

With the aim to create a scientific evidence of the convenience or not of removing the Multicystic Kidney (MK), a systematic review has been done over the last 20 years, selecting those articles with determinant criterions. Our experience has been also evaluated. We have obtained an evidence table of 1082 MK, with a follow-up from 2 to 7 years. No case degenerated. The complications were: urinary tract infection(UTI) in 5% and hipertensión(HTA) in 0.7%. In our serie (68 cases): nephrectomy was done in 10 cases. 82% completely involved (66.6% before 5 years and 15.6% from 5 to 15 years of follow-up). 18% involved partially. No case degenerated. The complications were UTI (6 cases) and HTA in one. Periodical ultrasound follow-up is our recommendation for MK due to the results of our serie and from the systematic review of the literature (AU)

[Diagnosis and surgical treatment for male pseudohermaphroditism in a multidisciplinary unit of intersexual conditions]. / Diagnóstico y tratamiento quirúrgico del pseudohermafroditismo masculino en una unidad multidisciplinaria de estados intersexuales.

The birth of a child with ambiguous genitalia represents a very stressing situation for the family, and afterwards has great social and psychological repercussion for the patient itself. Until now, the sex assignation is being done according to the phenotype. Now, with the molecular diagnosis of the genes that play a role in the sexual development, the assignation must also take into account the prognosis of response to androgens. The aim of this work is to review the 40 male pseudohermaphroditism cases controlled in our hospital and the genetic molecular diagnosis done in 19 cases, thus obtaining the certainty diagnosis. In 15 patients the mutations were located in the AR gene (androgen receptor). In 2 cases the mutation affected the SRD5A2 gene (deficiency of 5a-reductase) and in the other 2 cases it affected the HSD17BIII gene (deficiency of 17-ketoreductase). If the mutations affect the AR gene they must be assigned to the feminine sex, because of the impossibility of virilisation at puberty (lack of response to androgens). If the mutations are located in the other 2 genes they can be assigned to the masculine sex, since in puberty, they will present good response to androgens and will virilize. The molecular diagnosis offers us also the possibility to establish a prenatal diagnosis and to offer genetic advice.

[Biliary atresia and polysplenia syndrome: outcome and growth post-transplantation]. / Atresia de vías biliares y síndrome de poliesplenia: evolución y crecimiento postrasplante.

Biliary atresia (BA) is the most common indication for liver transplantation in children. Approximately 7-10% of these patients have the associated polysplenia syndrome (PS). The prognosis of patients with BA and PS has been reported to be poorer than that in patients with BA without PS. All patients who underwent liver transplantation for BA and who still attend periodic controls at the outpatient clinic were considered. A retrospective study of outcome and growth in children with BA was made, and compared with a subgroup of patients with BA and PS. There were no significant differences on complications, liver and renal function tests, lipids and growth data. We concluded that BA and PS do not preclude successful liver transplantation.

Hematologic abnormalities in liver-transplanted children during medium- to long-term follow-up.

OBJECTIVE: Hematologic abnormalities as adverse effects related to immunosuppressive drugs in liver-transplanted children are rarely reported. We have observed anemia, neutropenia, and thrombocytopenia in our pediatric liver-transplant population. The aim of this study was to exclude all suspected etiologies to define the association of immunosuppressants with these abnormalities. METHODS: Patients under 18 years old who still attend periodic controls at liver-transplant outpatient clinics were considered. Seventy patients met the inclusion criteria, 36 girls and 34 boys. Mean patient age was 5.6 years (range: 7 months to 17 years) and mean follow-up 6 years (range: 1-10 years). Medical records were reviewed beginning 1 month posttransplant. Treatment exposures, irradiation, blood product administration, and all laboratory studies were reviewed. When a hematologic abnormality was detected, we recorded the management for its resolution, the clinical response to therapy and the length of treatment. RESULTS: Twenty-five of the 70 children suffered 26 abnormal hematologic episodes (anemia 14, neutropenia 2, thrombocytopenia 3, simultaneous anemia and neutropenia 5, and pancytopenia 2). Eleven episodes (42%) had unclear etiologies and the process of elimination suggested an association with the immunosuppressant. Switching immunosuppressant was required in four patients and dose reduction in seven. CONCLUSIONS: Hematologic abnormalities in liver-transplanted children are common. The etiology is readily attributable to several causes. When the immunosuppressant appears to be a possible cause, the first step is dose reduction. If the hematologic abnormality persists despite dose reduction, a trial switch may be required.

[First orthotopic liver transplantation in patient with biliary atresia and situs inversus in spain]. / Primer trasplante hepático en un paciente con atresia de vías biliares y situs inversus en España.

Biliary atresia is the most common indication for liver transplantation in the pediatric age group. The Kasai portoenterostomy has become established as the primary treatment for biliary atresia. If portoenterostomy fails, death before 2 years of age is likely without liver transplantation. The most common multiple malformation syndrome associated with biliary atresia is polysplenia syndrome, which forms a constellation of defects of body symmetry, splenic development and vascular anomalies, including situs inversus, polysplenia and others. The situs inversus was formerly considered an absolute contraindication for liver transplantation. Recently however, several case reports have been published suggesting that neither situs inversus nor this particular subset of vascular abnormalities should be considered contraindications to liver transplantation. We present one case of liver transplantation performed in patient with biliary atresia, situs inversus and polysplenia. This is the first report described in Spain for a liver transplant in a child with biliary atresia plus situs inversus.

Primer trasplante hepático en un paciente con atresia de vías biliares y situs inversus en España / First orthotopic liver transplantation in patient with biliary atresia and situs inversus in Spain

La atresia de vías biliares es la indicación más frecuente para trasplante hepático en la edad pediátrica. La portoenterostomía de Kasai es el tratamiento primario para la atresia de vías biliares. Si la portoenterostomía falla, el fallecimiento suele ocurrir antes de los 2años de edad sin el trasplante hepático. El síndrome de múltiples malformaciones más comunmente asociado con la atresia de vías biliares es el síndrome de poliesplenia, formado por una constelación de defectos en la simetría corporal, desarrollo del bazo y anomalías vasculares, incluyendo situs inversus, poliesplenia y otros. El situs inversus era considerado como una contraindicación absoluta para el trasplante hepático. Sin embargo recientemente han sido publicados varios casos, sugiriendo que ni el situs inversus ni la presencia de dichas alteraciones vasculares deben ser consideradas como contraindicaciones para el trasplante hepático. Presentamos un caso de trasplante hepático realizado a un paciente con atresia de vías biliares, situs inversus y poliesplenia. Es el primer caso descrito en España (AU)

[Esophageal dilatation by Savary-Guillard bougies in children]. / Dilataciones esofágicas con sondas de Savary-Guilliard en la infancia.

Dilatations are considered the election treatment for esophageal stenosis of different etiologies. Different methods of dilatation have been used through the years. Security and effectiveness are the main subjects when we choose a dilatation method. We present the results of the last 3 years that Savary-Guilliard have been used, with a guide wire probe, under endoscopic control. Six patients with postsurgical stenosis and 10 with post lye ingestion stenosis were treated with the above mentioned method. The site of stenosis is localized under flexible endoscopy, and a special guide wire probe is introduced to the stomach. Once the wire is in place, different diameter bougies are introduced until a firm resistance is felt or the desired diameter is reach. In complicated cases the progression of the wire was controlled by X-rays. A total of 208 dilatations have done in 16 patients over the last three years. Six patients with postsurgical stenosis needed from two to six dilatations for their cure. Of the 10 patients who ingest lye, none of them had needed a gastrostomy. Three of them have no dysphagia after 9, 13 and 13 dilatations. The other 7 are under dilatations every 6 weeks in 6 cases and every 4 weeks in one case, been all of them in the second year of treatment. All the dilatations have been performed under general anesthesia, as outpatients. We have not had any complication under this treatment. We have found that the Savary-Guilliard method is adequate for esophageal dilatations in pediatric population. Security and effectiveness are the main points of this procedure, there is no need for a gastrostomy, and the child will have a better quality of life. This procedure is less aggressive, and this will give a shorter postop period, with no complications and the child will have a longer period of normal life between dilatations.

[Piriform sinus fistula, a new disease]. / Fístula del seno piriforme, una nueva patología.

Pyriform sinus fistula has recently been described as a new, rare and little known pathology. The process develops as latero-cervical suppurative adenitis or acute suppurative thyroiditis, usually on the left side. Radical surgery is mandatory to avoid recurrence. This congenital malformation must be present in the differential diagnosis of recurrent infectious cervical processes, especially in acute thyroiditis. The only curative treatment is complete exeresis of the fistula after meticulous dissection of the fistulous tract. We present three cases of this pathology. The diagnostic approach and surgical treatment are discussed. The recent literature on this topic is reviewed.

Pseudosubluxation of C2-C3 in childhood: a frequent clinico-radiological diagnostic error. / Pseudosubluxación C2-C3 en la infancia: un error frecuente de diagnóstico clínico-radiológico.

Although serious cervical injuries in pediatric patients are very infrequent, the may occur occasionally as a result of a strong blow to the head. Clinical records and radiological pictures, and in some cases computer tomography, help to provide the correct diagnosis. During childhood there are several normal radiological variants that may be interpreted as pathological findings, of which pseudosubluxation C2-C3 is the most frequent. We present two such cases and discuss the clinical and radiological criteria for the differential diagnosis between normal variants and injuries to the cervical spine in pediatric patients.