Concordancia entre el diagnóstico por imagen y la histología en las malformaciones pulmonares congénitas / Diagnostic accuracy of imaging compared to histology in congenital lung malformations

Introducción: El objetivo fue evaluar la concordancia entre las pruebas de imagen, la ecografía prenatal y la TC posnatal empleadas en el diagnóstico de malformaciones pulmonares congénitas (MPC) y el estudio anatomopatológico (AP).Material y métodos: Estudio retrospectivo de pacientes diagnosticados prenatalmente de MPC en los que se realizó seguimiento posnatal incluyendo una TC y un estudio AP de la lesión. Las variables estudiadas incluyeron: datos demográficos, edad gestacional al diagnóstico, hallazgos ecográficos y existencia de gestación múltiple. Utilizamos el coeficiente estadístico Kappa para establecer la concordancia entre la ecografía y las pruebas postnatales (TC y AP). Se analizaron de forma pareada la presencia de lesiones, la localización, el tipo y el tamaño, y la presencia de vascularización sistémica.Resultados: Se incluyeron 56 pacientes con 57 lesiones. La edad gestacional media al diagnóstico fue 22,42±3,94 semanas y el 57% fueron varones. El pulmón izquierdo y los lóbulos inferiores fueron los más afectados. La concordancia entre TC y AP en la detección de lesiones quísticas fue moderada (Kappa=0,55) pero más relevante que la detectada entre ecografía y AP (Kappa=0,10), siendo discreta entre ambas pruebas de imagen. La concordancia TC/AP fue sustancial (Kappa=0,66) en la detección de vascularización sistémica de la lesión y superior a la determinada entre ecografía y AP. Ambas pruebas de imagen demostraron una precisión muy buena en la identificación de la localización de las lesiones.Conclusiones: La TC posnatal ofrece una concordancia sustancial con el estudio histológico, especialmente en la detección de vascularización, y nos aporta datos predecibles sobre la anatomía de la lesión.(AU)

Introduction: The aim of this study was to evaluate the accuracy of imaging tests (prenatal ultrasound [US] and postnatal computed tomography [CT]) in comparison to histology for diagnosis of congenital lung malformations (CLMs).Material and methods: Retrospective study of patients with a prenatal diagnosis of CLM whose postnatal followup included thoracic CT scan and histological examination of the lesion. We collected data on demographic variables, gestational age at diagnosis, US findings and the history of multiple gestation. We used the kappa coefficient to determine the level of agreement between the findings of prenatal US and postnatal tests (CT and histology). We analysed paired data on the size of the lesion, its location and the presence or absence of systemic arterial vascularization.Results: The sample included 56 patients with 57 lesions. The mean gestational age at diagnosis was 22.42 weeks (SD, 3.94) and 57% were male. Malformations most frequently involved the left lung and the lower lobes. The agreement between CT and histology in the detection of cystic lesions was moderate (κ=.55) but stronger compared to the agreement between US and histology (κ=.10). The agreement between CT and histology was substantial (κ=.66) in the detection of systemic vascularization of the lesion and stronger compared to the agreement between US and histology. Both imaging methods were highly accurate in the identification of the location of the pulmonary lesions.Conclusions: Postnatal CT offers a substantial concordance with histological findings, especially in the detection of systemic vascularization, and an accurate prediction of the anatomy of the lesion.(AU)

Diagnostic accuracy of imaging compared to histology in congenital lung malformations.

INTRODUCTION: The aim of this study was to evaluate the accuracy of imaging tests (prenatal ultrasound [US] and postnatal computed tomography [CT]) in comparison to histology for diagnosis of congenital lung malformations (CLMs). MATERIAL AND METHODS: Retrospective study of patients with a prenatal diagnosis of CLM whose postnatal follow-up included thoracic CT scan and histological examination of the lesion. We collected data on demographic variables, gestational age at diagnosis, US findings and the history of multiple gestation. We used the kappa coefficient to determine the level of agreement between the findings of prenatal US and postnatal tests (CT and histology).We analysed paired data on the size of the lesion, its location and the presence or absence of systemic arterial vascularization. RESULTS: The sample included 56 patients with 57 lesions. The mean gestational age at diagnosis was 22.42 weeks (SD, 3.94) and 57% were male. Malformations most frequently involved the left lung and the lower lobes. The agreement between CT and histology in the detection of cystic lesions was moderate (κ = 0.55) but stronger compared to the agreement between US and histology (κ = 0.10). The agreement between CT and histology was substantial (κ = 0.66) in the detection of systemic vascularization of the lesion and stronger compared to the agreement between US and histology. Both imaging methods were highly accurate in the identification of the location of the pulmonary lesions. CONCLUSIONS: postnatal CT offers a substantial concordance with histological findings, especially in the detection of systemic vascularization, and an accurate prediction of the anatomy of the lesion.

Úlcera rectal solitaria secundaria a prolapso rectal recurrente / Solitary rectal ulcer secondary to recurrent rectal prolapse

El síndrome de úlcera rectal solitaria (SURS) es una entidad excepcional en Pediatría, en especial si se acompaña de prolapso rectal recurrente. Es habitual que se confunda con otras entidades, como la enfermedad inflamatoria intestinal (EII), lo que puede conllevar retraso en el diagnóstico. Clínicamente, se manifiesta como tenesmo, urgencia defecatoria, rectorragia leve que puede estar acompañada de moco y sensación de evacuación incompleta. Para el diagnóstico es fundamental la realización de una rectoscopia con toma de biopsias. El tratamiento es controvertido, ya que no existen guías consensuadas para el manejo de esta entidad. La presencia de prolapso recurrente suele requerir cirugía (AU)

Solitary rectal ulcer syndrome (SURS) is a rare disease in paediatrics, especially when it is associated with recurrent rectal prolapse. It can be easily confused with other conditions, such as inflammatory bowel disease (IBD), which can lead to a delayed diagnosis. It manifests in the form of tenesmus, faecal urgency, mild rectal bleeding possibly accompanied by mucus and incomplete evacuation. Performance of a rectoscopy with collection of biopsy samples is essential for diagnosis. Its treatment is still subject to controversy, as there are no consensus guidelines for the management of this disease. If there is recurrent prolapse, surgery is usually required. (AU)

Quiste de colédoco: nunca bajar la guardia / Choledocal cyst: never let your guard down

El quiste del colédoco es una dilatación congénita de la vía biliar. La presentación clínica es inespecífica y a menudo supone un dilema diagnóstico. Se debe tener un elevado índice de sospecha ante casos de ictericia, dolor abdominal y masa abdominal palpable. Para su diagnóstico es fundamental un estudio de imagen, siendo el indicado de forma inicial la ecografía. Pueden presentar múltiples complicaciones, incluyendo colangitis, pancreatitis, colangiocarcinoma, cirrosis biliar y hepática. Para su manejo se recomienda la resección del quiste, para evitar complicaciones y disminuir el riesgo de malignización. Se recomienda un seguimiento a largo plazo, con ecografías y análisis anuales, dado que el riesgo de malignización se mantiene elevado incluso tras la escisión del quiste. El abordaje óptimo de esta patología requiere un enfoque multidisciplinar, desde la sospecha diagnóstica que a menudo procederá de los servicios de Atención Primaria, incluyendo también gastroenterólogos, cirujanos, patólogos y radiólogos (AU)

Bile duct cyst is a congenital dilation of the bile duct. Clinical presentation is non-specific and often poses a diagnostic dilemma. It should be highly suspected in cases of jaundice, abdominal pain and a palpable abdominal mass. An imaging study is essential for diagnosis, an ultrasound being the most suitable initially. It can present multiple complications, including cholangitis, pancreatitis, cholangiocarcinoma, biliary and hepatic cirrhosis. For its management, resection of the cyst is recommended to avoid complications and reduce the risk of malignancy. Long-term follow-up with annual ultrasound scans and tests is recommended, as the risk of malignancy remains high even after excision of the cyst. The optimal approach to this pathology requires a multidisciplinary perspective, starting from the diagnostic suspicion that will often come from Primary Care services, and including also gastroenterologists, surgeons, pathologists and radiologists (AU)

Estenosis esofágica congénita. La sospecha diagnóstica se inicia desde Atención Primaria / Esophageal congenital stenosis. The diagnostic suspect is started from Primary Care

La estenosis esofágica congénita es una malformación rara que ocurre debido a la separación incompleta del esófago y del sistema respiratorio. Generalmente cursa con vómitos persistentes que suelen debutar o exacerbarse con la introducción de la alimentación complementaria y con ello de los alimentos sólidos. Dados los síntomas de presentación, la sospecha diagnóstica se puede realizar desde las consultas de Pediatría de Atención Primaria. El diagnóstico se realiza mediante una endoscopia digestiva alta que mostrará normalidad de la mucosa y un tránsito digestivo. Existe controversia en cuanto al tratamiento de elección, prefiriéndose el tratamiento conservador mediante dilataciones endoscópicas de inicio

Esophageal congenital stenosis is an unusual type of malformation that occurs due to incomplete division of the esophagus and respiratory system. Generally, it shows up with persistent vomiting that tends to debut or exacerbate with the introduction of complementary feeding and solid foods. A high degree of suspicion is necessary for diagnosis from Primary Care pediatricians. A high digestive endoscopy with normal mucosa and a digestive transit confirm the diagnosis. There is controversy regarding the treatment of choice, conservative treatment is preferred by initial endoscopic dilations

Thoracoscopic approach for complications after esophageal atresia repair: initial experience.

INTRODUCTION: Improvements in technology and health care have helped reduce morbidity and mortality in patients with esophageal atresia. However, postoperative complications such as dehiscences, strictures, and recurrent fistulas still occur in a large percentage of patients. Here, we present our initial experience using the thoracoscopic approach for complications after esophageal atresia repair. METHODS: We retrospectively analyzed the medical records of patients who developed complications after esophageal atresia repair and underwent thoracoscopic reoperation at two centers. Anastomotic leakage, anastomotic stricture, and recurrent tracheoesophageal fistula were assessed as complications after esophageal atresia repair. RESULTS: We reoperated on four patients (range of age 3 days - 2 years) by thoracoscopy. Two patients who had undergone thoracoscopic atresia repair presented with anastomotic dehiscence. The anastomoses were thoracoscopically reconstructed without tension. One patient had undergone thoracoscopic atresia repair presented with esophageal stricture refractory to endoscopic dilatations. Thoracoscopic esophagoesophagostomy was performed in this 2-year-old patient. One patient who had undergone esophageal atresia repair and tracheoesophageal fistula closure by thoracotomy presented with a recurrent fistula at 1 year of age. The patient underwent thoracoscopic fistula closure with a 5-mm endostapler. All patients remain asymptomatic after reoperation. CONCLUSIONS: Reoperation after esophageal atresia repair is challenging and carries a relatively high risk of developing complications. Compared to conventional surgery, the thoracoscopic approach in experienced hands offers better visualization and more accurate dissection and drainage of the thoracic cavity. Therefore, we want to encourage the use of thoracoscopy in the treatment of complications after esophageal atresia repair.

Thoracoscopic esophagoesophagostomy for a refractory stricture in a patient with esophageal atresia.

Anastomosis stricture is a well-known complication after esophageal atresia repair. Endoscopic dilatation is the gold standard treatment for esophageal stenosis. However, surgical interventions are indicated for refractory cases. We present a 2-year-old girl with esophageal stricture refractory to regular endoscopic dilatation after esophageal atresia repair that underwent thoracoscopic stricture resection and reanastomosis. Although thoracoscopic approach is widely used for esophageal atresia repair, this approach has not been used before for the treatment of anastomosis stricture.

The Accuracy of Silk Glove Sign Compared with Inguinal Bulge in the Diagnosis of Indirect Inguinal Hernia in Children.

Introduction: The diagnosis of indirect inguinal hernia is usually based on clinical grounds. Physical examination generally showed an inguinal bulge. However, when no bulge is seen, the silk glove sign (SGS) or palpating the processus vaginalis over the pubic tubercle can be useful. The aim of our study is to compare the accuracy of the presence of inguinal bulge with the presence of SGS. Materials and Methods: We conducted a retrospective of all patients undergoing laparoscopic inguinal hernia repair between January 2002 and November 2015. Preoperative diagnosis was obtained by physical examination. The presence of an inguinal bulge or SGS was considered diagnostic of indirect inguinal hernia. Intraoperative diagnosis was made to laparoscopic findings. The sample was divided into two groups: group 1 including the patient with inguinal bulge and group 2 including those with SGS. Results: A total of 1024 inguinal canals were evaluated. Inguinal bulge was observed in 379 inguinal canals (group 1), whereas SGS was detected in 196 (group 2). There were statistically significant differences between both groups regarding gestational age, birth weight, surgical age, and surgical weight. Prematurity and previous episodes of incarceration were statistically more common in group 1 (P < .001). The positive predictive value in group 1 was 98.7%, whereas in group 2 was 86.73% (P < .001). Conclusions: Although we have found that the success rate for accurate diagnosis is higher in patients with inguinal bulge, SGS can be useful in detecting indirect inguinal hernia.

Torsión simultánea de epidídimo y quiste epididimario / Simultaneous torsion of epididymis and an epididymal cyst

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Is the Laparoscopic Approach Safe for Inguinal Hernia Repair in Preterms?

Introduction: Although laparoscopic inguinal hernia repair in children has gained popularity in the last decades, this approach remains uncommon in preterm infants. The aim of this study was to compare the characteristics and the outcomes of indirect inguinal hernias in term and preterm infants. Material and Methods: From January 2002 to November 2015, all charts of the pediatric patients who underwent laparoscopic indirect inguinal hernia repair in one single institution within the first 6 months of life were revised. The data of 156 patients were analyzed retrospectively. Patients were divided in two groups: group I, including the preterm patients, and group II, including the term patients. Results: A total of 90 preterm infants and 66 term infants were included. In the group I, preoperative diagnosis was right-sided inguinal hernia in 20% of patients, left sided in 22.2%, and bilateral in 57.5%; while in the group II, preoperative diagnosis was right-sided inguinal hernia in 42.4% of infants, left sided in 15.2%, and bilateral in 42.4% (P = .01). In group I intraoperative diagnosis was right-sided inguinal hernia in 10% of patients, left sided in 16.7%, and bilateral in 73.3%; while in the group II, intraoperative diagnosis was right-sided inguinal hernia in 25.8% of infants, left sided in 12.1%, bilateral in 60.6%, and there was no hernia in one patient (P = .02). However, there was no statistically significant difference in the correct intraoperative diagnosis between both groups (P = .59). No statistical significance was observed between the two groups regarding postoperative complications. Conclusions: Bilateral inguinal hernia is more frequent in preterm infants compared to term infants, whereas the incidence of right-sided inguinal hernia is higher in term patients. Laparoscopic inguinal hernia repair in preterm infants seems to be safe and effective.

Association between gallbladder agenesis and choledochal cyst: cause or coincidence?

This case report describes an extremely rare association between gallbladder agenesis and choledochal cyst (CC). A 9-year-old girl presented with recurrent abdominal pain in the right upper quadrant. Radiological studies revealed a CC type IVa and an agenesis of gallbladder and cystic duct. Due to the possibility of biliary neoplasm, the patient underwent cyst resection and hepaticoduodenostomy. Histopathological findings showed inflamed fibrous tissue covered by biliary epithelium with no evidence of malignancy.

Thoracoscopic recurrent tracheo-oesophageal fistula repair with mini endostapler: promising solution.

Recurrent tracheo-oesophageal fistula (TOF) is a common complication in children who underwent oesophageal atresia repair. The traditional surgical approach performed either by thoracotomy or cervicotomy is associated with a high rate of morbidity, mortality and new recurrence. In the last decades, endoscopic techniques have emerged as the minimally invasive alternative. However, it seems that the optimal treatment is still unknown. We present a patient with a recurrent TOF who underwent thoracoscopic closure using a 5.8 mm endostapler. The patient was extubated at the end of the procedure, and he started feeding the day after surgery. At 15 months of follow-up, he is asymptomatic. Thoracoscopic closure of TOF using endostaplers seems to be a safe alternative with some possible benefits compared with traditional and endoscopic approach.

Wilkie's syndrome in monozygotic twins treated by 3-D laparoscopic duodenojejunostomy.

Superior mesenteric artery syndrome, also known as Wilkie's syndrome, is a rare cause of proximal duodenum obstruction in children. Here, we describe the first pediatric case of superior mesenteric artery syndrome in monozygotic twin brothers. Both patients underwent 3-D laparoscopic duodenojejunostomy at the same age with an uneventful recovery.

Is the laparoscopic approach appropriate for pediatric subhepatic appendicitis?

INTRODUCTION: Subhepatic appendicitis in children is an unusual condition that can be challenging for the pediatric surgeons to treat. The aim of our study was to compare the outcomes of laparoscopic appendectomies based on the position of the appendix. METHODS: The data of 1736 patients who had undergone laparoscopic appendectomy in our tertiary center were retrospectively reviewed. We compared two groups: subhepatic location (n = 56) and non-subhepatic location (n = 1680). A P-value of less than 0.05 was considered statistically significant. RESULTS: There was no statistically significant difference between the demographic variables of the groups. More than half of the subhepatic appendicitises were gangrenous (44.6%) or perforated (16.1%), whereas most of the non-subhepatic appendicitises were phlegmonous (56.9%). Extracorporeal ligation of the appendix was the preferred technique in both the subhepatic and non-subhepatic groups (69.6% and 89.8%, respectively). The subhepatic group had a statistically significant higher incidence of technical difficulties (1.6%) and abdominal drain (18.6%) than the non-subhepatic group, as well as a longer operative time and hospital stay. However, intraoperative and postoperative complications were similar in both groups. CONCLUSION: Laparoscopic subhepatic appendectomy is safe and does not lead to increased complications. However, the technique is made difficult by the fact that the appendix is an atypical location, and the rate of complicated appendicitis is higher.

Pediatric femoral hernia in the laparoscopic era.

INTRODUCTION: Femoral hernia is a rare and often misdiagnosed condition in childhood. The aim of our study was to demonstrate that the laparoscopic approach improves diagnostic accuracy and offers a safe and effective treatment. METHODS: A retrospective study of 687 pediatric patients who underwent laparoscopic inguinal hernia repair from January 2000 to December 2015 was performed. RESULTS: Femoral hernias were identified in 16 patients (2.3%). The right side was affected in 10 cases (62.5%), the left side in 5 (31.2%), and 1 case was bilateral (6.2%). The mean age of patients was 8.00 ± 3.81 years, and there was a male predominance. Preoperative diagnosis was femoral hernia in eight cases (50%) and indirect inguinal hernia in the remaining eight (50%). Seven children (43.8%) presented with hernia recurrence after having undergone an open ipsilateral indirect hernia repair. A modified laparoscopic McVay technique was performed in 12 cases (70.6%). An epigastric artery injury by trocar occurred in one patient. All operations were completed laparoscopically. The mean surgical time was 45.6 ± 22.9 min for unilateral cases and 110 ± 10.0 min for bilateral cases. No immediate postoperative complications were noted. The mean postoperative hospital stay was 0.6 ± 0.4 days. No recurrence was observed after a median follow-up of 11 years (range, 4-16 years). CONCLUSION: Femoral hernia is a rare pathology in pediatric patients that is often difficult to diagnose. The laparoscopic approach is effective in the diagnosing and treating these hernias, and it allows for the simultaneous repair of multiple groin defects.