[Spigelian hernia: two cases associated to cryptorchidism]. / Hernia de Spiegel: a propósito de 2 casos asociados a criptorquidia.

Spigelian hernia (SH) is rare in children, it occurs primarily en adults the mean age of presentacion is 50 years. We present two patients with hernia associated to cryptorchidism one of them bilateral.

Tiroidectomía profiláctica en pacientes pediátricos con riesgo de carcinoma medular tiroideo / Prophylactic thyroidectomy in children at risk for medullary thyroid carcinoma

El carcinoma medular de tiroides (CMT) es una rara neoplasiaen la infancia. En la actualidad el estudio molecular del proto-oncogén RET, relacionado con los síndromes de neoplasia endocrina múltiple (MEN II) y carcinoma medular familiar de tiroides (CMFT)permiten identificar pacientes con riesgo de padecer carcinoma medularde tiroides en edades precoces, antes de que se manifieste la enfermedad clínicamente. Niños con antecedentes familiares de MEN II o CMFT fueron estudiados bioquímica (test de pentagastrina) y genéticamente con el fin dedeterminar el riesgo de padecer CMT y evaluar la posibilidad de realizar tiroidectomía profiláctica (AU)

The medullary thyroid carcinoma (MTC) is a rare neoplasiaoccurring during childhood. At present time the molecular examinationof the proto-oncogen RET, related to syndromes of multipleendocrine neoplasia (MEN II) and familial medullary thyroid carcinoma(FMTC) to allows identify patients with risk of suffering of medullarythyroid carcinoma in early ages, before the disease becomes clinicallypronunced. Children with familial antecedents of MEN II orFMTC were biochemically (pentagastrin-stimulated) and geneticallystudied with the purpose of determining the risk of developing a MTCand in order to assess the possibilities of making a prophylactic thyroidectomyThe medullary thyroid carcinoma (MTC) is a rare neoplasia occurring during childhood. At present time the molecular examinationof the proto-oncogen RET, related to syndromes of multiple endocrine neoplasia (MEN II) and familial medullary thyroid carcinoma(FMTC) to allows identify patients with risk of suffering of medullary thyroid carcinoma in early ages, before the disease becomes clinically pronunced. Children with familial antecedents of MEN II orFMTC were biochemically (pentagastrin-stimulated) and genetically studied with the purpose of determining the risk of developing a MTCand in order to assess the possibilities of making a prophylactic thyroidectomy (AU)

Hernia de Spiegel: A propósito de 2 casos asociados a criptorquidia / Spigelian: two cases associated to cryptorchidism

La hernia de Spiegel es una hernia rara en la población infantil, más común en la población adulta con predominio en la 5ª década de la vida. Presentamos dos pacientes con hernia asociada a criptorquidia, una de ellas bilateral (AU)

Spigelian hernia (SH) is rare in children, it occurs primarily en adults the mean age of presentacion is 50 years. We present two patients with hernia associated to cryptorchidism one of them bilateral (AU)

Efficiency of the anorectal manometry for the diagnosis of Hirschsprung's disease in the newborn period.

We have used a continuous perfusion probe for manometry in newborns. We carried out 95 pressure recordings in 56 patients labeled as delayed passage of meconium longer than 24 h. When the relaxation reflex of the anal canal was not present after rectal stimulation with a distensible balloon, the tests were repeated weekly up to the first month of life. Thereafter, suction biopsies were carried out to confirm the final Hirschsprung's disease (HD) diagnosis. The amplitudes of the fluctuating waves in HD subjects were higher than those in non-HD patients. Although the procedure could provide false positive results, no possibilities exist for false negative results as proved in our study. All the five cases with HD were diagnosed by our manometric procedure as well as by histochemistry. The method is advantageous and profitable for the diagnosis of HD in case of delayed passage of meconium or distal functional obstruction of the anal canal.

[Neuroblastoma IV-S. A multicenter study. Work Group of Pediatric Oncology. Spanish Society of Pediatric Surgery]. / Neuroblastoma IV-S. Estudio multicéntrico." Grupo de Trabajo de Oncología Pediátrica". Sociedad Española de Cirugía Pediátrica.

A retrospective multicentric study of 23 patients with stage IV-S neuroblastoma treated in 10 national Pediatric Surgery Departments between January 1988 and December 1992, is presented. All cases were classified as stage IV-S following staging criteria proposed by Evans et al. The age diagnosis was an average of 3 months. An acute clinical course was seen in 7 patients. Primary sites of the tumor were: adrenal gland in 6 patients (30.5%), abdominal paravertebral in 3 (13%) and unknown in 2 (9%). Distant tumor sites were: liver in 20 patients, bone marrow in 9, and skin in 5. There were not systematic therapeutic approach, in 16 cases (69%) the primary tumor was resected, 18 (78%) received chemotherapy, the liver was irradiated in 3 (13%), and 1 case (4%) received no treatment at all. One children died. The overall disease-free survival is 95.6% at 43 months post-diagnosis. A common treatment protocol is proposed, with systematic determination of N-myc oncogene which will indicate the appropriate therapy.

Preservation of anaplastic features in Wilms' tumors after preoperative chemotherapy.

In 4 of 35 childhood Wilms' tumors on record in the Hospital Infantil Virgen de Rocio de Sevilla, it was possible to identify diffuse anaplasia despite the fact these children had received cytoreductive chemotherapy preoperatively.

[Caustic burns of the esophagus in childhood. Our 14 years' experience]. / Causticación de esófago en la infancia. Nuestra experiencia de catorce años.

A 14-years revision (1972-1986) is made of 3,600 children attended to after ingesting some type of caustic agent. Only 81 were admitted to hospital. We found a predominance in the 1-3 year age group. Bleach was the caustic most frequently present but lye accounted for most major lesions. The more relevant symptoms were oropharyngeal lesions (85%), vomiting (26%) and sialorrhea (20%). Oropharyngeal burns was the sign most often found in esophageal lesions (45%). The ingestion of ordinary household bleach did not result in serious esophageal lesions nor posterior complications, and does not require esophagoscopy if no other symptom except vomiting is present. Esophagoscopy is the ideal means for evaluating esophageal lesions, as are esophageal dilatations with Rehbein dilators for stenosis due to scarring. With the use of corticosteroids in the acute phase, there were 8 cases (22%) of esophageal stenosis out of 36 with lesions.

[Usefulness of echography in the diagnosis of intestinal intussusception]. / Utilidad de la ecografía en el diagnóstico de invaginación intestinal.

The usefulness of echography in the diagnosis and tracing down of an intestinal intussusception is discussed. An abdominal ecograph was done in 29 children with suspected intestinal intussusception. In 31 cases (63%) the diagnosis was confirmed by the typical "target" image. In 17 cases (34%) the picture of intussusception was not detected, all children evolved favourably except for one case which, upon revision of the ecograph, showed the "target" picture not properly interpreted in the first place. We find the abdominal ecograph a useful means for diagnosis of intestinal intussusception. We present the diagnostic-therapeutic routine in our centre in cases of possible intestinal intussusception.

[Ovarian torsion in early childhood]. / Torsión de ovario en la edad infantil.

Three cases of torsion of normal the uterine adnexa in premenarcheal girls are reported. The possible etiopathogenic mechanisms are commented, and the operative management is discussed. The value of ultrasonography in early diagnosis is pointed out.

Maturation of Wilms' tumor pulmonary metastases to benign fibromas after therapy.

A 3-year-old girl with Wilms' tumor developed pulmonary metastases 5 months after diagnosis. All but one of the visible lesions disappeared after thoracic irradiation and multiple-agent chemotherapy. Subsequent thoracotomy revealed 17 small nodules in addition to the visible lesion, and all of them proved to be fibromas. It would appear that the Wilms' tumor metastases to the lung were made to mature to benign neoplastic processes after the treatments given.

[Treatment of rhabdomyosarcoma of the bladder without radical surgery]. / Tratamiento del rabdomiosarcoma de vejiga sin cirugía radical.

Authors present two cases of rhabdomyosarcoma of the bladder treated previously to surgery with chemotherapy (protocol T-2) and radiation therapy. In one of these patients surgery consisted only in a partial cystectomy, and in the other only a few biopsies were taken from the most suspicious zones, and these were all negative. Three years and four months after diagnosis and 15 months after completion of treatment, both children are alive and free of detectable disease.

[Neonatal adrenal hemorrhage. A surgical problem?]. / Hemorragia suprarrenal neonatal. Problema quirúrgico?

Eight cases of neonatal adrenal haemorrhage are reported, four of which were necropsy findings, two were treated conservatively and the remaining two underwent exploratory and evacuation laparotomy. The four latter patients recovered. Diverse clinical pictures which may be present at the onset of adrenal haemorrhage in newborns are reviewed and particularly the indications for surgery. In this report, one laparotomy was done to establish a differential diagnosis of retroperitoneal mass, and the other after onset of intense hemoperitoneum and signs of hypovolemic shock. Different pathogenic hypotheses about this entity are contemplated, particularly traumas and circumstances surrounding birth; one of the reported cases coincided with a known birth-injury. The various diagnostic methods in use today and their degree of usefulness or risk also come into discussion. Special mention is made about calcifications of the adrenal glands in this disease and they are compared to calcifications found in other pediatric problems.

[Pyloric atresia and epidermolysis bullosa. A coincidence? (author's transl)]. / Atresia del píloro y epidermólisis ampollosa ?Una coincidencia?

Three cases of the rare malformation pyloric atresia are presented, two of which are associated with epidermolysis bullosa, a rare malformation by itself. First two cases had a great pneumoperitoneum which suggested perforation of digestive tract. All three cases belonged to type I of pyloric atresia.

[Foreign bodies in digestive tract (author's transl)]. / Cuerpos extranos digestivos

One hundred and seventy three cases of ingestion of foreign bodies by children are reported. Their nature and evolution inside the organism is discussed. Laparotomy was required in only one case.

[Congenital malformations associated with esophageal atresia (author's transl)]. / Malformaciones congénitas asociadas a las atresias esofágicas

In 67 patients with esophageal atresia we have found associated congenital anomalies in over 80% and 10 children could be considered as carriers of Vater association. The various malformations are reported and their etiopathogenic factors considered.