Laparoscopic Management of Intestinal Obstruction due to a Tight Muscular Cuff Following Transanal Endorectal Pull-through for Hirschsprung's Disease.

Complications of Hirschsprung's disease's surgery include the general complications of any abdominal surgery, but one of the specific complications is obstruction, which occurs in 10% of children after surgical correction and can be due to a narrowing muscular cuff or a coloanal anastomotic stenosis. We report a case of a 4-month baby, diagnosed as suffering from Hirschsprung's disease, who developed postoperative constipation after transanal endorectal pull-through due to unusual folding of the muscular cuff, which narrowed the colon. A laparoscopic approach was performed. During the surgery, it was observed that the muscular cuff was rolled down, surrounding the neorectum. The anterior rectal cuff was completely divided from the pulled-through colon. After surgery, no intraluminal stenosis was revealed on digital rectal examination. A long and tight rectal muscular cuff could be the reason for postoperative obstructive side effects. The use of laparoscopy has the advantage of confirming the suspected diagnosis and a clear visualisation of the rectal cuff. Key Words: Hirschsprung disease, Intestinal obstruction, Laparoscopy.

Cleidocranial dysostosis: a case report with clinical illustration.

Cleidocranial Dysostosis or Dysplasia (CCD) is an infrequent clinical condition, with an autosomal dominant hereditary mode of inheritance. Triad lesions: multiple supernumerary teeth, partial or complete absence of the clavicles and open sagittal sutures and fontanelles. Nine-year-old female patient comes to our service for outpatient consultation with the main complaint of upper limbs mobility restriction with shoulders hypermotility. The chest X-ray showed partial absence of the clavicles and a cone-shaped thorax. The diagnosis of CCD was performed. Treatment of these patients requires a multidisciplinary approach which includes orthopaedic and dental corrections. The premature diagnosis allows a proper orientation for the treatment, offering a better life quality for the patient.

Giant umbilical hernia: "Lazy M-Omega" flap, a case report.

Umbilical hernia, one of the most frequent pathologies in pediatric surgical practice, is usually corrected with a relatively simple intervention, except in cases where there is a major defect, also called proboscoid hernia. We present a case report of a 20-month male patient that underwent surgical intervention of a giant umbilical hernia with the "Lazy-M and Omega" flap novel surgical technique. This technique has to be taken into account in surgical planning, since it is simple and easily reproducible.

Una presentación atípica de fístula perianal / An atypical presentation of a peri-anal fistula

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El hemangioma mediastínico y la importancia del GLUT-1 / Mediastinal haemangioma and the role of GLUT1

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Three-Dimensional Printed Model and Virtual Reconstruction: An Extra Tool for Pediatric Solid Tumors Surgery.

Introduction Three-dimensional (3D) technology is increasingly applied for planning challenging surgical interventions. We report our experience using 3D printing and virtual reconstruction for surgical planning of complex tumor resections in children. Methods Data were obtained from preoperative magnetic resonance. imaging analysis and 3D virtual recreations were performed using specialized computer software. 3D real-scale geometry models, including tumor, adjacent organs, and relevant vascularization, were printed in colorimetric scale and different materials for optimal structures discrimination. Results Four complex cases were selected. The first case was a bilateral Wilms tumor. The volumetric reconstruction proved the presence of enough healthy renal tissue, allowing bilateral nephron-sparing surgery. In the second case, reconstruction contributed to the location of pulmonary metastases. The third case was an abdominal neuroblastoma stage L2. The 3D model was of high value for planning and as a reference during the intervention. The last case is a cervico-thoracic neuroblastoma with an anatomopathological diagnosis of ganglioneuroma, located at the cervico-mediastinal juncture, in close relationship with the cervical vessels. Conclusions 3D reconstruction and the full-scale printing models are a useful tool in cases of complex tumor resections as they contribute to a better understanding of the relationships between the tumor and adjacent organs, helping to anticipate certain surgical complications. They also provide additional information to conventional imaging tests, being able to influence therapeutic decisions and facilitate the understanding by the family, improving doctor-patient communication.

Correlación entre el neumoperitoneo y los hallazgos quirúrgicos y morbimortalidad en recién nacidos con enterocolitis necrosante / Correlation between pneumoperitoneum and surgical findings and morbidity and mortality in newborns with necrotising enterocolitis

INTRODUCCIÓN: La intervención quirúrgica en las enterocolitis necrosantes (EN) es precisa cuando existe gangrena intestinal, hecho evidente cuando produce perforación y neumoperitoneo, siendo este la única indicación radiológica aceptada universalmente para la intervención quirúrgica. OBJETIVO: Analizar a los pacientes intervenidos de EN, saber por qué se les intervino, cómo evolucionan y si los pacientes perforados presentan neumoperitoneo. MÉTODO: Estudio retrospectivo de una cohorte de recién nacidos con EN intervenidos durante un periodo de 10 años (2006-2015). Se analizan los hallazgos radiológicos preoperatorios y se correlacionan con los quirúrgicos y con la morbimortalidad, dependiendo de la presencia de neumoperitoneo (N+) o no (N-). Se evaluó la concordancia interobservador con radiólogo pediátrico enmascarado a la clínica mediante el índice de acuerdo kappa. RESULTADOS: Se analizó a 53 pacientes. El 36% se intervino tras la visualización de neumoperitoneo; en el resto, la indicación fue deterioro clínico y metabólico, junto con hallazgos radiológicos asociados. En el 39% del grupo N- se objetivó perforación. No se encontraron diferencias significativas en ambos grupos con respecto a longitud intestinal resecada, días de intubación, día de inicio de nutrición enteral y mortalidad. La comparación entre duración de síntomas y estancia hospitalaria total en ambos grupos (N-/N+) fue significativa (7 vs. 2 días, p = 0,008; 127 vs. 79 días, p = 0,003 respectivamente), siendo más favorable en el grupo N+. Estas diferencias se mantuvieron al ajustar por peso. CONCLUSIONES: La indicación quirúrgica ha de basarse en un conjunto de datos clínicos y radiológicos, ya que el 39% de los pacientes sin neumoperitoneo presentaron perforación. En nuestro estudio la presencia de neumoperitoneo no se correlaciona con peor pronóstico

INTRODUCTION: Surgical intervention in necrotising enterocolitis (NEC) is correct when there is intestinal gangrene. This is evident when gangrene produces perforation and pneumoperitoneum, with this being the only universally accepted radiological indication for the surgical intervention of NEC. OBJECTIVE: To perform an analysis on patients with surgically managed NEC, including determining how the decision to intervene is reached, the outcomes, and if patients with perforation had a pneumoperitoneum. METHODS: Retrospective review of neonates with surgical NEC over a period of 10years (2006-2015). An analysis was made of pre-surgical x-ray findings, which were compared with surgical ones, in addition to the morbidity and mortality, depending on the presence (N+) or absence (N-) of pneumoperitoneum. An evaluation was also made of the interobserver concordance with a paediatric radiologist blinded to the clinical reason using the kappa agreement index. RESULTS: A total of 53 neonates were included in the study. Surgical treatment was indicated after observing pneumoperitoneum in 36%. In the remaining neonates, the surgical decision was made after noting a clinical and metabolic deterioration with classical x-ray findings. Intestinal perforation was observed in 39% of the N- neonates. There were no statistical differences between either group on analysing the excised intestinal length, days of intubation, starting of enteral nutrition, and the mortality rate. Comparisons in terms of duration of symptoms and total hospital stay were statistically significant (7 vs. 2 days, P = .008; 127 vs. 79 days, P = .003, respectively), with both being more favourable in the N+ group. These differences remained when the groups were adjusted by birthweight. CONCLUSIONS: Surgical indication has to be done on an ensemble of clinical and radiological evidence, as 39% of the neonates in the N- groups were perforated. In our study, the presence of a pneumoperitoneum did not correlate with a worse prognosis

[Correlation between pneumoperitoneum and surgical findings and morbidity and mortality in newborns with necrotising enterocolitis]. / Correlación entre el neumoperitoneo y los hallazgos quirúrgicos y morbimortalidad en recién nacidos con enterocolitis necrosante.

INTRODUCTION: Surgical intervention in necrotising enterocolitis (NEC) is correct when there is intestinal gangrene. This is evident when gangrene produces perforation and pneumoperitoneum, with this being the only universally accepted radiological indication for the surgical intervention of NEC. OBJECTIVE: To perform an analysis on patients with surgically managed NEC, including determining how the decision to intervene is reached, the outcomes, and if patients with perforation had a pneumoperitoneum. METHODS: Retrospective review of neonates with surgical NEC over a period of 10years (2006-2015). An analysis was made of pre-surgical x-ray findings, which were compared with surgical ones, in addition to the morbidity and mortality, depending on the presence (N+) or absence (N-) of pneumoperitoneum. An evaluation was also made of the interobserver concordance with a paediatric radiologist blinded to the clinical reason using the kappa agreement index. RESULTS: A total of 53 neonates were included in the study. Surgical treatment was indicated after observing pneumoperitoneum in 36%. In the remaining neonates, the surgical decision was made after noting a clinical and metabolic deterioration with classical x-ray findings. Intestinal perforation was observed in 39% of the N- neonates. There were no statistical differences between either group on analysing the excised intestinal length, days of intubation, starting of enteral nutrition, and the mortality rate. Comparisons in terms of duration of symptoms and total hospital stay were statistically significant (7 vs. 2 days, P=.008; 127 vs. 79 days, P=.003, respectively), with both being more favourable in the N+ group. These differences remained when the groups were adjusted by birthweight. CONCLUSIONS: Surgical indication has to be done on an ensemble of clinical and radiological evidence, as 39% of the neonates in the N- groups were perforated. In our study, the presence of a pneumoperitoneum did not correlate with a worse prognosis.

Evaluación funcional y estética de los teratomas sacrococcígeos. No todo termina con la cirugía / Functional and aesthetic evaluation of sacrococcygeal teratomas. Not everything ends with surgery

Introducción: El teratoma sacrococcígeo es el tumor neonatal sólido más frecuente. El aumento de la supervivencia de estos pacientes ha permitido diagnosticar y tratar secuelas posquirúrgicas. Evaluamos el resultado funcional y estético a largo plazo de los pacientes intervenidos en nuestro centro. Material y método: Estudio retrospectivo de los teratomas sacrococcígeos intervenidos en nuestro hospital desde 1977 hasta 2014. Se recogieron datos personales de los pacientes y se realizó encuesta telefónica sobre hábito intestinal, urinario y autoevaluación estética y social. Resultados: En el período de estudio se intervino a 14 pacientes, 11 mujeres y 3 varones, con una edad mediana en el momento de la encuesta de 17 años (8 meses-37 años). Se encuestó a 8 pacientes (57,1%). La edad mediana de esos 8 pacientes fue de 23 años (4-37 años). El 37,5% se intervino por un teratoma sacrococcígeo tipo I, 25% tipo II, 25% tipo III y 12,5% tipo IV. De los pacientes estudiados, 2 (25%) presentan estreñimiento y uno (12,5%) incontinencia fecal. Dos pacientes (25%) presentan infecciones urinarias recurrentes y 3 (37,5%) incontinencia urinaria. Cinco pacientes (62,5%) presentan alterada su percepción física con limitación en su vida social. Conclusiones: En nuestra serie, se ha encontrado un porcentaje similar a la literatura en la incidencia de estreñimiento. La incontinencia fecal está levemente disminuida respecto a series publicadas. Sin embargo, la frecuencia de infecciones y de incontinencia urinarias es mayor en nuestra serie. Cinco pacientes presentan problemas psicosociales según el cuestionario DAS-59. Los pacientes con teratoma sacrococcígeo requieren asesoramiento urológico, digestivo y psicológico, para poder adquirir un completo desarrollo funcional y emocional (AU)

Introduction: Sacrococcygeal teratoma is the most common solid neonatal tumour. The improvement in survival has meant that postoperative sequelae can be diagnosed and treated. The aim of this article is to evaluate the long-term outcomes of patients treated in our centre. Material and methods: Records of patients treated for a sacrococcygeal teratoma in our hospital from 1977 to 2014 were retrospectively reviewed. Personal data was collected and a telephone questionnaire was used to assess long-term bowel and urinary habits, as well as an aesthetic and functional self-assessment. Results: A total of 14 patients were treated during the study period, of whom 11 were females and 3 males, with a mean age at the time of the survey of 17 years (8 months-37 years). Eight patients completed the questionnaire (57.1%). The mean age of the 8 patients was 23 years (4-37 years), of whom 37.5% were operated on due to a sacrococcygeal teratoma type I, 25% type II, 25% type III, and 12.5% type IV. Two of them (25%) had constipation, and one (12.5%) had faecal incontinence. Two (25%) patients suffered from recurrent urinary tract infections, and 3 (37.5%) patients had urinary incontinence. Five patients (62.5%) had a perception of being physically impaired, with limitation of their social life. Conclusions: The incidence of constipation does not differ from that found in the literature. Faecal incontinence is slightly improved compared to what has been published. However, urinary tract infections and incontinence are more prevalent in our series. Five patients out of the eight that responded suffered from psychosocial problems, according to DAS-59 questionnaire. Patients with SCT require urological, bowel, and psychological counselling, until they have a complete functional and emotional development (AU)

[Functional and aesthetic evaluation of sacrococcygeal teratomas. Not everything ends with surgery]. / Evaluación funcional y estética de los teratomas sacrococcígeos. No todo termina con la cirugía.

INTRODUCTION: Sacrococcygeal teratoma is the most common solid neonatal tumour. The improvement in survival has meant that postoperative sequelae can be diagnosed and treated. The aim of this article is to evaluate the long-term outcomes of patients treated in our centre. MATERIAL AND METHODS: Records of patients treated for a sacrococcygeal teratoma in our hospital from 1977 to 2014 were retrospectively reviewed. Personal data was collected and a telephone questionnaire was used to assess long-term bowel and urinary habits, as well as an aesthetic and functional self-assessment. RESULTS: A total of 14 patients were treated during the study period, of whom 11 were females and 3 males, with a mean age at the time of the survey of 17 years (8 months-37 years). Eight patients completed the questionnaire (57.1%). The mean age of the 8 patients was 23 years (4-37 years), of whom 37.5% were operated on due to a sacrococcygeal teratoma type i, 25% type ii, 25% type iii, and 12.5% type iv. Two of them (25%) had constipation, and one (12.5%) had faecal incontinence. Two (25%) patients suffered from recurrent urinary tract infections, and 3 (37.5%) patients had urinary incontinence. Five patients (62.5%) had a perception of being physically impaired, with limitation of their social life. CONCLUSIONS: The incidence of constipation does not differ from that found in the literature. Faecal incontinence is slightly improved compared to what has been published. However, urinary tract infections and incontinence are more prevalent in our series. Five patients out of the eight that responded suffered from psychosocial problems, according to DAS-59 questionnaire. Patients with SCT require urological, bowel, and psychological counselling, until they have a complete functional and emotional development.

Migration of a ventriculoperitoneal shunt catheter through the processus vaginalis in infants: how serious inguinal hernia and hydrocele might be.

Migration of the catheter tip of a ventriculoperitoneal shunt (VPS) through a patent processus vaginalis (PV) has seldom being reported. In this series, we analyzed all cases of inguinoscrotal migration of an VPS catheter at our center. Between 1975 and 2015, 295 VPS insertions were performed. In seven infants (2.3%), migration of the catheter through a patent PV was demonstrated. Cases with hydroceles had associated VPS malfunction, but cases with hernias were not associated with it. The current recommendation is to delay a PV closure until 2 years of age in cases of infants with hydrocele without further radiological examinations, but this might not be the case in infants with VPS.