Luis Morales

Paraneoplasic precocious puberty. Report of a new case with hepatoblastoma and review of the literature.

A new case of precocious puberty secondary to production of human chorionic gonadotropin (HCG) by an hepatoblastoma was studied in an 8‐month‐old infant. A review was made of the 35 cases of paraneoplasic precocious puberty previously reported in the literature. The most frequent cause is hepatoblastoma, which was responsible for 18 cases. There are nine reports of mediastinal teratoma, six of which were observed in patients with Klinefelter Syndrome. In another six cases, the paraneoplasic syndrome was attributed to intracranial tumoration, and two of these patients showed teratoma with areas of choriocarcinoma, whereas germinoma was suspected for clinical reasons in another three. Lastly, one case of presacral teratoma and one of retroperitoneal carcinoma were reported. The differential characteristics of paraneoplasic precocious puberty are: almost exclusive occurrence in males, because of tumoral production of HCG, moderate or absent increase of testicle size, hyperplasia of Leydigs cells without spermatogenesis, and rapidly progressive signs of puberty. Cancer 56: 1725‐1729, 1985.

Segmental dilatation of the duodenum

In this article two cases of idiopathic duodenal dilation are described. These cases meet the present criteria for segmental dilations of the intestine. The onset of the clinical features was acute in one case and chronic in the other. However, the radiological images were similar in both cases and provided the diagnosis. Surgical treatment consisted of duodenal resection and tapering and was curative in one case. The other patient died shortly after surgery. The duodenal specimens showed normal three-layer duodenal architecture in the pathology study. The intramural nervous plexi were unimpaired. Angiodysplasia was found in one case. Vascular abnormalities were also found in 10 other segmentary intestinal dilatations reported in the literature. The surgical implications of this new localization of segmentary intestinal dilatation are analyzed.

Intestinal damage in gastroschisis is independent of the size of the abdominal defect

Abstract The intestinal damage in gastroschisis (GS) has been attributed to a narrow abdominal wall defect (AWD), among other causes, but this causal effect is difficult to prove in humans. The present experimental study was done to ascertain the damaging effect of clinically extrapolable mild and moderate constriction at the AWD on the intestine of fetuses with GS. AWDs of two different sizes were carried out in the fetal rabbit model: small-ring GS (1.5× bowel diameter, SRG) and large-ring GS (3× bowel diameter, LRG); a group of unoperated littermates served as controls. Fetal body weight, intestinal length and weight, bowel diameter and wall thickness, and histology were checked 7 days later. No statistical difference was found in body weight and bowel diameter among the groups. Intestinal length, weight, and wall thickness were significantly different in the GS groups compared to the controls, but no difference was found between the GS groups. Histology did not show venous stasis, ischemic lesions, or differences in the degree of edema between groups SRG and LRG. Mesothelial hyperplasia was seen in both GS groups. The intestinal changes in length, weight, diameter, wall thickness, and histology in GS should thus not be attributed to the diameter of the AWD.

Morphology and mucosal biochemistry of gastroschisis intestine in urine-free amniotic fluid.

BACKGROUND/PURPOSE The aim of this study was to determine the role of urine exposure in gastroschisis on the pathologic and biochemical aspects. METHODS The intestines of fetal rabbits with gastroschisis (group G), gastroschisis and urethral ligation (group GL), and normal controls (group C) were studied by measuring weigh and length, intestinal diameter and wall thickness, and thickness of each intestinal layer. Number and length of villi and villi edema were evaluated. Total protein and DNA were measured in intestinal homogenate. Lactase activity and alkaline phosphatase activity were analyzed in isolated microvilli membranes. RESULTS Intestinal length, diameter, and wall thickness were significantly different in groups G and GL compared with C but not between groups G and GL. The same was true for the thickness of the internal muscular and serosa, villi length, and villi edema. Serosal reaction was milder in group GL than in group G, and absent in C. Total protein, lactase activity, and alkaline phosphatase activity were also significantly different in G and GL compared with C but not between G and GL. CONCLUSIONS Urine in amniotic fluid causes an increased serosal reaction but does not account for the mucosal enzyme dysfunction.

Effect of the environment on fetal skin wound healing

Skin wound healing has been shown to be a different process in the fetus than in the adult animal. Some of these differences have been attributed to the unique fetal environment (i.e., amniotic fluid). The aim of the present study is to compare fetal skin healing in intraamniotic and extraamniotic wounds. A fetal rabbit model has been used in which three types of skin wounds were induced on 23-day-old fetuses in contact with either amniotic fluid or maternal peritoneal fluid. The wounds consisted of a sutured skin incision, a nonsutured incision, and an electrocautery burn. Seven days later all wounds were examined mechanically (scar resistance), biochemically (collagen and noncollagen protein concentration), and histologically. Biochemical and growth factor studies of both environments, the amniotic and the peritoneal fluids, were also conducted. The results showed excellent healing by first intention and absence of healing by second intention in both environments, greater scar resistance in the intraperitoneally positioned fetal wounds, and a higher concentration of insulin-like growth factor-1 (IGF-1) in the peritoneal fluid than in the amniotic fluid. It can be concluded that fetal skin wounds in contact with peritoneal fluid show the same healing pattern as in the natural fetal environment (amniotic fluid). The higher concentration of IGF-1 in the peritoneal fluid suggests that this growth factor, through its relationship with the growth hormone, plays a role in increasing the scar resistance of fetal skin wounds in contact with maternal peritoneal fluid.

Wound healing in the fetal period: The resistance of the scar to rupture

Wound healing in the fetal period is fundamentally different from that of the adult. In order to better understand this difference, we have studied wound healing in three types of wounds which are the most common in surgical practice, and have paid special attention to tensile strength in the scar. A sutured wound, a nonsutured wound, and an electrocautery burn were performed on a group of 30 rabbit fetuses with a gestational age of 23 days. Seven days later, the resulting scars were examined using histological, mechanical, and biochemical studies. The results were compared with those obtained in a group of 30 newborn rabbits and in a group of 30 adult rabbits who had had the same type of wounds performed on them. The body weight of operated fetuses was smaller than their control siblings. The sutured wound in the fetuses showed a more perfect macroscopic repair than in older subjects. However, the nonsutured wounds and the burns of the fetal group did not close. In the fetuses, the tensile strength of the sutured wound was 20% of the tensile strength of healthy skin. A similar relationship occurred in the group of adult rabbits. In the sutured wound of the fetal group, the synthesis of collagen was significantly less than in the adult group. The ratio between hydroxyproline (HYP) concentration in the sutured wound and HYP concentration in healthy skin was also significantly less in the fetal group than in the adult group.(ABSTRACT TRUNCATED AT 250 WORDS)

Intraspinal migration of a rectal foreign body

This is a description of a rare case of intraspinal foreign body, a clinical thermometer bulb, which after perforating the rectum migrated through the second left sacral foramina and sacral canal up to the epidural space at S1-L5 level. A first attempt to remove it by laparotomy while it was still in the retrorectal space proved unsuccessful. Retrieval was finally achieved by laminectomy.

Adrenocortical tumors in childhood: A report of four cases

In the last 10 years, four children with adrenocortical tumors were treated in our hospital. Three of them had symptoms of adrenogenital virilizing syndrome, and another, nonfunctional, was found during a routine examination. A diagnosis of adrenocortical tumor was established given the symptoms, hormonal tests, and radiologic studies. Two of the tumors were located in the left side, and the histologic diagnosis was pleomorphic cortical adenoma; the ones that affected the right side were adenocarcinomas. All the patients were treated by surgery and none received chemotherapy. At both presurgical and postsurgical stages, the patients were treated with cortisol. All four children have had a favorable course, with normal growth and the disappearance of public hair and hirsutism; however, macrogenitals still persist.

Congenital epigastric evisceration: a case report

A rare case of abdominal wall defect at the epigastric midline is presented. This newborn boy had only the greater omentum eviscerated, and no other abnormalities could be detected. This case does not seem to relate to the abdominal wall defects reported so far in the literature. This singular case contributes to enlarge the spectrum of congenital defects of the abdominal wall.

Solitary Pelvic Kidney and Neuroblastoma in a Child

We describe a case of a solitary pelvic kidney coexisting with adrenal and pelvic neuroblastoma in a child with multiple malformations, including microcephaly, hypertelorism, aortic coarctation and a bifid uterus. The association of a solitary kidney and neuroblastoma has not been reported previously.