[Preoperatory sonography efficiency in paediatric patients with cholelithiasis undergoing laparoscopic cholecystectomy]. / Eficacia de la ecografía preoperatoria en la colelitiasis en pacientes pediátricos sometidos a colecistectomía laparoscópica.

INTRODUCTION: Biliary lithiasis is not much frequent in paediatric patients. The manegement of cholelithiasis in patients undergoing laparoscopic cholecystectomy is still controversial. We propose the preoperatory echographic study of the biliary tree 24-48 h before surgery, as the first choice, instead of the intraoperatory cholangiography. MATERIAL AND METHODS: We made a retrospective study of 42 patients undergoing laparoscopic cholecystectomy due to symptomatic biliary lithiasis during the last 15 years, with ages between 18 months and 17-years-old (mean age 9,6-years-old) and weight between 11 and 70 kg (mean weight 42 kg) at the moment of surgery. Six of them had haematological illnesses, 17 came to the hospital because of acute abdominal pain, 10 had been studied because of recurrent abdominal pain and 9 had casual diagnoses. Abdominal sonography was performed in all patients 24-48 hours before surgery. RESULTS: Four children were diagnosed of biliary duct lithiasis: two choledocolithiasis and two stones in the cystic duct. One of the cystic stones was extracted in the operating room and the rest resolved spontaneously. One patient presented dilatation of choledocal duct after surgery, without any stones' evidence. Also this patient resolved spontaneously. We had no complications. CONCLUSIONS: Biliary lithiasis is not frequent in children, even if it seems to be increasing. A few of these patients will suffer of choledocolithiasis. The intraoperatory exploration of the biliary tree during laparoscopic surgery is technically difficult due the small size of paediatric patients. Cholangiography is not always successful and can produce some important complications as pancreatitis. Preoperative sonography 24-48 hours before surgery is a safe and efficient method for the diagnosis and follow-up of paediatric patients with biliary lithiasis undergoing laparoscopic cholecystectomy. It is safe enough to be performed without intraoperatory cholangiography.

Eficacia de la ecografía preoperatoria en la colelitiasis en pacientes pediátricos sometidos acolecistectomía laparoscópica / Preoperatory sonography efficiency in paediatric patients with cholelithiasis undergoing laparoscopic cholecystectomy

Introducción. La litiasis biliar es poco frecuente en edad pediátrica. El manejo de la colelitiasis en pacientes sometidos a colecistectomía laparoscópica sigue siendo controvertido. Proponemos el estudio ecográfico preoperatorio de la vía biliar 24-48 h antes de la intervención, como alternativa a la colangiografía intraoperatoria. Material y métodos. Realizamos un estudio retrospectivo de 42pacientes sometidos a colecistectomía laparoscópica por litiasis biliarsintomática en los últimos 15 años, con edades comprendidas entre 18meses y 17 años (edad media 9,6 años) y peso entre 11 y 70 kg (peso medio 42 kg) en el momento de la intervención. De ellos, 6 pacientes tenían enfermedades hematológicas, 17 acudieron a urgencias por dolor abdominal agudo, 10 eran estudiados por dolor abdominal recurrente y9 fueron hallazgos casuales. Todos los niños se sometieron a ecografía abdominal en las 24-48 horas previas a la intervención. Resultados. En cuatro casos se diagnosticaron cálculos de la víabiliar: dos coledocolitiasis y dos cálculos en cístico; en un paciente se extrajo el cálculo del cístico durante la intervención y los demás se resolvieron espontáneamente. Uno de los pacientes presentó dilatación del colédoco en el postoperatorio, sin evidenciar ningún cálculo, y también (..) (AU)

Introduction. Biliary lithiasis is not much frequent in paediatricpatients. The manegement of cholelithiasis in patients undergoing laparoscopic cholecystectomy is still controversial. We propose the preoperatory echographic study of the biliary tree 24-48h before surgery, as thefirst choice, instead of the intraoperatory cholangiography. Material and methods. We made a retrospective study of 42patients undergoing laparoscopic cholecystectomy due to symptomaticbiliary lithiasis during the last 15 years, with ages between 18 months and 17 years-old (mean age 9,6 years-old) and weight between 11 and70 kg (mean weight 42 kg) at the moment of surgery. Six of them had haematological illnesses, 17 came to the hospital because of acute abdominal pain, 10 had been studied because of recurrent abdominal pain and9 had casual diagnoses. Abdominal sonography was performed in all patients 24-48 hours before surgery. Results. Four children were diagnosed of biliary duct lithiasis: two (..) (AU)

[Mammary reconstruction in Poland's syndrome]. / Reconstrucción mamaria en el síndrome de Poland.

Poland's syndrome is considered to be a polimalformative entity with different degrees of severity. We retrospectively reviewed our patients with Poland's syndrome diagnosis, treated between 2000 and 2006. The aim of our study is to assess the different choices of surgical treatment for mammary reconstruction according to the importance of malformation and taking into account our own experience and literature references. A total o f 13 patients (5 males and 8 females) were studied and only 6 of them (1 male and 5 females) underwent surgery. Surgical indication was based on Foucras's classification, considering 3 grades of severity. We propose a surgical approach adapted to each stage, as well as surgical refinements to improve the aesthetic aspect. Most of our patients were very satisfied with final results even though they underwent complex procedures with a considerable rate of complications.

Reconstrucción mamaria en el síndrome de Poland / Mammary reconstruction in Poland´s syndrome

El síndrome de Poland actualmente se considera como un síndrome polimalformativo que se expresa con diferentes grados de severidad. Hemos realizado un estudio clínico retrospectivo de los pacientes diagnosticados en nuestro hospital entre el año 2000 y el 2006 con el objetivo de revisar las opciones de tratamiento quirúrgico para la reconstrucción mamaria según el grado de la malformación, basados en nuestra propia experiencia y en la revisión de la literatura. Recogimos un total de 13pacientes (5 hombres y 8 mujeres) de los cuales fueron operados 6 (1hombre y 5 mujeres). La indicación quirúrgica se basó en la clasificación propuesta por Foucras que divide la deformidad en 3 estadios de severidad. Proponemos una estrategia terapéutica adecuada para cada estadio así como técnicas de refinamiento para mejorar el resultado estético. La mayoría de los pacientes manifestaron un alto grado de satisfacción con el resultado a pesar de tratarse de intervenciones complejas con una tasa de complicaciones no desdeñable (AU)

Poland’s syndrome is considered to be a polimalformativeentity with different degrees of severity. We retrospectively reviewed our patients with Poland’s syndrome diagnosis, treated between 2000and 2006. The aim of our study is to assess the different choices of surgical treatment for mammary reconstruction according to the importance of malformation and taking into account our own experience and literature references. A total of 13 patients (5 males and 8 females) were studied and only 6 of them (1 male and 5 females) underwent surgery. Surgical indication was based on Foucras’s classification, considering3 grades of severity. We propose a surgical approach adapted to each stage, as well as surgical refinements to improve the aesthetic aspect. Most of our patients were very satisfied with final results even though they underwent complex procedures with a considerable rate of complications (AU)

Intraabdominal desmoplastic small round cell tumour.

Desmoplastic small round cell tumour (DSRCT) is an extremely rare neoplasm. Adolescent males and young adults are most frequently affected. It is highly malignant, with only 29% of patients surviving up to 3 years. This paper documents two cases, one of which, at 4 years old, is the second youngest case documented. Case 1, a 10-year old boy, presented with a 20-day history of choluria, acholia, asthenia, anorexia, and right abdominal pain. Laboratory values were altered, and imaging showed multiples masses in the liver and retroperitoneum. A minilaparotomy was carried out, and a biopsy showed a stage III DSRCT. He was treated with chemotherapy but died of hepatic failure. Case 2, a 4-year-old boy, presented with a 2-month history of abdominal distension. Several hard masses were palpated in the abdomen, and a right inguinal mass that compressed the right testis was observed. Biopsy of the inguinal tumour showed a DSRCT. After treatment with chemotherapy, two operations were carried out to resect different intraabdominal masses. The patient died with peritoneal carcinomatosis 2 months after the last operation. The first patient died due to the advanced stage of the disease, and the second died after chemotherapy, peripheral blood stem transplantation, and multiple operations. The occurrence of this type of tumour in the paediatric age group as well as its high malignancy is noteworthy. Until more effective forms of treatment are found, we recommend treatment with chemotherapy, surgery, and radiotherapy, with close monitoring of the patient.

The role of surgery in stage IV neuroblastoma.

BACKGROUND/PURPOSE: The importance of primary tumor resection in stage IV neuroblastoma is controversial. The authors analyzed prospectively the role of surgery in a multicentric series of stage IV neuroblastoma patients. METHODS: Patients were studied according to the International Neuroblastoma Staging System (INSS) recommendations. Age, sex, location of the tumor, type of metastases, time of resection (initial or delayed), extension of resection, surgical complications, pathology, N-myc and Shimada classification results, relapses, and outcome were studied. After diagnosis, children received induction chemotherapy followed by delayed surgery and autologous stem cell transplantation or maintenance chemotherapy. Resection was classified as complete (C), greater than 90% (P1), greater than 50% (P2), less than 50% (P3), and biopsy (B). RESULTS: Ninety-eight stage IV children were admitted in the study from June 1992 to July 1999. Seventy-six were older than one year, and in 78 the primary tumor was abdominal. Bone was the most common metastatic site followed by bone marrow. Initial biopsy was performed in 74 patients, and resection in 6, with one complication in each group. N-myc was amplified in 20 of 80 tumors, and Shimada was unfavorable in 45 of 67. Delayed surgery was performed in 70 cases, achieving gross total resection in 55 (79%); there were minor complications in 10%. Mean survival rate time was 50 months. Event-free survival rate (EFS) at 5 years for the entire series is 0.32, but 0.0 for children having biopsy only, 0.25 for less than 50% resection, 0.31 for 50% to 90% resection, 0.44 for greater than 90% resection, and 0.33 for complete resection. Differences were statistically significant only when compared with the biopsied group. EFS rate for infants was 0.56, but, again, there was no difference in relation to the type of resection. There were 46 relapses, 12 of them local, 7 of 20 N-myc-amplified tumors, and 4 of 60 not amplified (P <.005). CONCLUSIONS: Biopsies of stage IV neuroblastoma allow safe assessment of N-myc and other biological factors on tumor tissue. Delayed surgery after chemotherapy is performed with a low rate of complications, achieving a good local control of disease. N-myc-amplified tumors have higher local relapse rates than nonamplified and therefore would need more intensive local treatment. The final outcome in these patients is determined more by metastatic relapses than by the degree of resection.

Atresia intestinal y atresia de vías biliares en un recién nacido / Small bowel atresia and bikiary atresia in a newborn

Se presenta un caso clínico reciente de atresia de intestino delgado asociado a una atresia de vías biliares. Se realiza una revisión de la literatura encontrando sólo 13 casos, lo que confirma su extraordinaria rareza (AU)

Carcinoma de tiroides con metástasis pulmonares / Thyroid carcinoma with pulmonary metastases

El carcinoma de tiroides es una entidad rara en la infancia. Mucho más rara es su presentación con metástasis pulmonares, de aquí que presentemos un caso clínico reciente en una paciente de 12 años. Hacemos una revisión del tratamiento recomendado y del pronóstico en la literatura (AU)

Malformación adenomatoidea quística pulmonar con vascularización anómala de origen sistémico / Congenital cystic adenomatoid malformation of the lung with abnormal systemic blood supply

Se presenta un caso clínico reciente en que con el diagnóstico de malformación adenomatoidea quística del pulmón, un paciente es intervenido encontrándonos una vascularización sistémica anómala asociada. Se hace una diferenciación entre esta malformación y lo que otros autores reconocen como pseudosecuestro pulmonar o MAQ (AU)

[Intestinal atresia and bile duct atresia in a new born infant]. / Atresia intestinal y atresia de vías biliares en un recién nacido.

We report a recent case of small bowel atresia and biliary atresia in a newborn. We describe the management of the patient and we make a revision of the literature. Only 13 cases have been found, that confirms that this association is extraordinary rare.

[Thyroid carcinoma with lung metastasis]. / Carcinoma de tiroides con metástasis pulmonares.

Thyroid carcinoma is a rare disease in children. Much rare its presentation with pulmonary metastases is. We here report a recent case in 12 years-old girl. We make a review of recommended treatment and the prognosis.

[Pulmonary cystic adenomatoid malformation with anomalous vascularization with systemic origin]. / Malformación adenomatoidea quística pulmonar con vascularización anómala de origen sistémico.

We report an infant with the diagnosis of Congenital Cystic Adenomatoid Malformation. He was operated and we found an abnormal systemic blood supply associated in that lobe. We make a review of the literature, and we make a distinction between this rare combination and others like pseudo pulmonary sequestration, or Congenital Cystic Adenomatoid Malformation associated to pulmonary sequestration.

Barrett's esophagus and chemotherapy, a case report.

There have been few reports of Barrett's esophagus associated with chemotherapy in children. We report the case of a 3-year-old patient diagnosed with acute lymphoblastic leukemia who developed Barrett's esophagus after BMF-90 chemotherapeutic regimen. A stricture appeared as a complication of Barrett's metaplasia and Nissen fundoplication was performed. Symptoms improved shortly after surgery and regression of Barrett's esophagus was observed 2 years later. Children treated with antileukemic chemotherapy may develop Barrett's esophagus without previous clinical apparent gastroesophageal reflux. Endoscopic surveillance has been advised in these patients. Barrett's esophagus may regress after antireflux surgery.

Femoral hernia in childhood: review of 38 cases.

Femoral hernias rarely occur at any age, but are extremely rare in infancy. We present 38 cases of femoral hernia treated in our pediatric surgery department during the last 15 years. The number of patients who underwent surgery was high compared with other series reviewed. This might be due to the fact that these hernias were considered in the differential diagnosis of inguinal pathology in childhood. The preoperative diagnosis was correct in 22 cases (57. 8%). After surgical treatment no recurrence was reported.

Rhabdomyosarcoma of the biliary tree

Rhabdomyosarcoma (RMS) of the biliary tree is a rare tumor in children that has a very poor prognosis. Preoperatively, it is often mistaken for a choledochal cyst. We report a case of RMS of the biliary tree in a 4-year-old girl who presented with abdominal pain and obstructive jaundice. The RMS was diagnosed at laparotomy; excision was not possible due to its size and localization. Chemotherapy achieved complete regression of the tumor observed at second-look surgery. Preoperative chemotherapy can now avoid mutilating surgical procedures and improve survival.

Rhabdomyosarcoma of the biliary tree.

Rhabdomyosarcoma (RMS) of the biliary tree is a rare tumor in children that has a very poor prognosis. Preoperatively, it is often mistaken for a choledochal cyst. We report a case of RMS of the biliary tree in a 4-year-old girl who presented with abdominal pain and obstructive jaundice. The RMS was diagnosed at laparotomy; excision was not possible due to its size and localization. Chemotherapy achieved complete regression of the tumor observed at second-look surgery. Preoperative chemotherapy can now avoid mutilating surgical procedures and improve survival.

Gallbladder malformation with gastric wall-like architecture.

A 3-year-old girl was found to have a distended gallbladder, which pathologically consisted almost entirely of a gastric-type wall, featuring muscularis mucosae and a well-developed bilayered muscularis propria. This appears to be a unique, not previously recognized, malformation of the gallbladder.

[The colonic atresia: an uncommon cause of neonatal intestinal obstruction]. / La atresia de colon: causa poco frecuente de obstrucción intestinal neonatal.

The intestinal atresia is a common cause of neonatal bowel obstruction, but the colonic atresia is an uncommon cause of neonatal intestinal obstruction. We present a newborn with congenital colon atresia who underwent laparotomy, revealing a colonic atresia type III. We recommend resection of the dilated proximal colon with primary anastomosis end to end if the patient is not perforated or in bad general conditions.

[Intussusception: barium vs. pneumatic reduction]. / Invaginación intestinal: Neumoenema versus enema de bario.

At the Hospital Niño Jesús air pressure enema has recently replaced the use of barium as treatment of choice in the reduction of intussusception. The purpose of this study was to review the air enema reductions done from June 1992 to February 1994 in 137 patients (group II) and to compare them with a previous series of 153 patients that underwent barium reduction treatment (group I). The groups were similar with regard to sex, age and presentins symptoms as well as clinical signs. Successful reduction was achieved in 131 patients (85.6%) out of 146 attempts treated with barium enema and in 124 patients (90.5%) out of 134 attempts treated with air enema. Among the unsuccessful reduction cases, operation was performed in 22 cases in group I and in 13 cases of group II. In the group I there were two perforations during the reduction attempt, while in group II we did not have any with the air enema. There were two recurrent intussusceptions following barium enema reduction and nine following air enema reduction in the first 48 hours. Patients treated with air enema stayed less days at hospital (1,6 days) than those treated with barium (3 days). We believe that the air enema treatment is easy to perform, is lower in cost and presents less morbility, and that it should be considered as the treatment of choice in the initial management of intussusception.

[Intestinal pseudo-obstruction secondary to systemic neuropathies and myopathies]. / Pseudoobstrucción intestinal secundaria a neuropatías y miopatías sistémicas.

The term intestinal pseudo-obstruction, is applied to a group of clinical disorders in which there are obstructive symptoms, in the absence of a mechanical agent, secondary to a disorder of intestinal motility. In this report, we show manometric studies done in four patients, with acute intestinal pseudo-obstructions, secondary to von Recklinghausen's neurofibromatosis (VRNF), in two patients, and in one patient with Duchenne's muscular dystrophy (DMD) and in another with Werdnig-Hoffmann's disease (WHD). We studied 24 hours antroduodenal motility, specially during phase 3 of the migratory motor complex (CMM), which showed a neuropathic disorder in one case of von Recklinghausen's disease and a myopathic disorder in patients with Duchenne's muscular dystrophy and Werdnig-Hoffmann's disease. We analysed results and conclusions and specially the kinetic action of erythromycin.