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Biomedical subjects

J Estevão-Costa

Publications and source records attributed to J Estevão-Costa.

At least 19 recordsLinked to original sources

Neonatal splenic necrosis not related to wandering spleen.

Splenic necrosis is extremely rare in neonates, the cases recorded so far being secondary to torsion of wandering organs. A newborn with an abdominal mass who underwent exchange transfusions through an umbilical catheter is presented here. Comprehensive investigation led to the suspicion of enteric duplication, but a splenic necrosis with no features of wandering spleen was found at laparotomy. The pathogenesis and preoperative diagnostic work-up of splenic necrosis are emphasised.

Humans↗

Histochemical and immunohistochemical study of the intrinsic innervation in colonic dysganglionosis.

Defective innervation of the neuromuscular junctions (NMJ) was recently described in intestinal neuronal dysplasia type B (IND B). The aim of the present study was to correlate the alterations in NMJs to other classically described parameters in dysganglionoses and to determine the relationship between NMJ abnormalities in IND B and clinical symptoms. The rectal biopsies and full-thickness colonic biopsy specimens of 17 patients were studied applying histochemical (acetylcholinesterase [AChE], lactic dehydrogenase [LDH], and succinic dehydrogenase [SDH] reactions) and immunohistochemical (neuronal-cell adhesion molecule [NCAM] and SY antibodies) methods. Thirteen patients had Hirschsprung's disease (HD). IND B was diagnosed in 11 (associated with HD in 8 cases, isolated in 2, and associated with hypoganglionosis in 1). In the aganglionic segment of HD there was very intense AChE activity; in contrast, NCAM- and SY-immunoreactive nerve fibers were markedly decreased. A spectrum of abnormalities was observed in IND B, usually more severe in the most distal segments: giant and immature ganglia in the submucous plexus were observed in all cases; heterotopic myenteric ganglia were frequent (72.7%); hyperganglionosis was observed in 6 (54.5%) and was not related to the patients' age; thick and tortuous NCAM- and SY-immunoreactive nerve fibers, irregularly distributed in the colonic wall, were observed in 81.8% of the cases. No relationship was observed between abnormalities of NCAM- and SY-immunoreactive nerve fibers and AChE activity, ganglion-cell maturity, heterotopy, or the clinical symptoms presented by the patients with IND B. In hypoganglionism, low AChE activity and a slight decrease in NCAM- and SY-immunoreactive nerves were observed. Thick and tortuous, irregularly-distributed intrinsic NCAM- and SY-immunoreactive nerves were observed in every colon layer in IND B. Our results do not support IND B as a NMJ disorder.

Child↗

Laparoscopic colonic mapping of dysganglionosis.

Retention of a proximal aganglionic segment or the unrecognized coexistence of other dysganglionoses may jeopardize the definitive surgical treatment of Hirschsprung's disease (HD). To assess the extent of the disease and/or the presence of other dysganglionoses without an additional laparotomy, we developed a laparoscopic-assisted technique to perform colonic full-thickness biopsies. After creation of a pneumoperitoneum, a 5-mm laparoscope is inserted in the supraumbilical area and a working 10/12-mm port is placed in the left iliac fossa. The sigmoid/descending colon is grasped and pulled through the abdominal wall and a full-thickness biopsy done. The same procedure is applied to the transverse and ascending colon. Rectal and colonic biopsy specimens were studied using enzyme histochemical methods. Over the last year, five children aged 7 months to 12 years with dysganglionosis underwent laparoscopic-assisted mapping of the colon. Previous rectal suction biopsies were diagnostic of HD in three patients and suspicious of hypoganglionosis in two. Proximal full-thickness biopsies revealed: normal colon in two cases of HD; coexistent type B intestinal neuronal dysplasia up to the descending colon in the other case of HD; and hypoganglionosis up to the ascending colon in the two patients with suspected hypoganglionosis. The procedures were performed easily, the patients being discharged after 36 to 48 h with no complications. Four children have already undergone pull-through procedures with resection of the affected colon in adhesion-free abdominal cavities and did not develop constipation or enterocolitis. Laparoscopic-assisted mapping of the entire colon is a simple, safe, and effective procedure that may contribute to improving the outcome of intestinal dysganglionosis by better characterization of the disease.

Child↗

A new fetal rat model of gastroschisis: development and early characterization.

BACKGROUND/PURPOSE: The perinatal management and pathophysiology of gastroschisis remain controversial. Large animal experimental models of gastroschisis are inherently limited by expense and length of gestation, making multiple studies and statistical analysis difficult. To address these limitations the authors have developed a model of gastroschisis in the fetal rat. METHODS: Twenty-one time-dated pregnant rats underwent laparotomy at 18 (1/2) day's gestational age. The exposed uterus was bathed in ritodrine for tocolysis. The right posterior leg was exteriorized through a hysterotomy, and under a dissecting microscope (16x) the fetal small bowel was exteriorized through a small incision performed on the right lower abdominal quadrant. The amniotic fluid was restored with saline solution and the hysterotomy closed with a purse-string suture. Control fetuses underwent hysterotomy and leg manipulation only. The surgical time was uniformly less than 60 minutes. Fetuses were harvested by cesarean section at 21 (1/2) days' gestational age. Fetal intestine was assessed by microscopic examination, and fetal weight, intestinal length, and intestinal weight per unit length were evaluated. RESULTS: There was a significant surgical and anesthetic learning curve, which is not included in this report. After this, the authors achieved a maternal survival of 100% (n = 21). We created gastroschisis in 64 fetuses (58 survivors, 90.6%), and 33 fetuses were only manipulated (30 survivors, 90.9%). The number of induced gastroschisis per pregnant rat varied between 2 and 5 with median of 3. On gross examination, eviscerated intestine appeared dilated, edematous, and covered by peel when compared with control intestine. Fetuses with gastroschisis had significantly reduced body weight (4.1+/-0.5 v 5.6 g +/- 0.5 g) and intestinal length (102+/-19 v 210+/-17 mm) relative to controls, whereas the intestinal weight per unit length (1.75+/-0.29 v 0.71 +/- 0.1 mg/mm) was markedly increased (P<.001). CONCLUSIONS: The pathophysiology observed in this experimental model appears to resemble human gastroschisis. In comparison with large animal models, the rat model offers the advantages of low expense, short gestation, littermate controls, and high maternal and fetal survival rates. In addition, there are specific probes and reagents available for application of molecular methodology to clarify the mechanisms responsible for the intestinal damage. This model appears appropriate for future experimental studies on gastroschisis.

Animals↗

Neonatal perforation of a Y-shaped sigmoid duplication.

Tubular colonic duplications are exceedingly rare; the "Y"-shaped forms are exceptional. In the absence of associated low vertebral or urogenital malformations (often fistulas), the tubular colonic malformations frequently stay hidden for several years until a complication develops. The authors report a case of a spontaneous perforation of a Y-shaped tubular colonic duplication during the neonatal period.

Anastomosis, Surgical↗

Antireflux role of free muscle transplantation. Experimental study in a reflux esophagitis rat model.

PURPOSE: Experimental work on free muscle transplantation has resulted in some successful clinical applications. After a previous experimental study that established the viability of this procedure on the distal esophagus of the rat, we investigated the antireflux efficacy of free muscle transplantation on the distal esophagus in a reflux esophagitis rat model. METHODS: Two groups of Sprague-Dawley rats were submitted to a reflux-inducing operation. Thirteen animals underwent esophagojejunostomy only (EJ) and fourteen were submitted to esophagojejunostomy plus free muscle transplantation of plantaris on the distal esophagus (ETx). Eight weeks later, the esophagi of survivors were removed after sacrifice (EJ, n = 10; ETx, n = 10) and the macroscopic longitudinal extension (mm) and the histological grade (1-4) of esophagitis evaluated. RESULTS: All esophageal specimens of both groups had esophagitis. However, the extent of esophagitis (median: ETx: 23 vs EJ: 32) and the frequency of high grades of esophagitis (ETx: 30 % vs. EJ: 100 %) were significantly lower in the ETx group (p = 0.005 and p = 0.001, respectively). All except one ETx subject presented with an extent and/or a grade of esophagitis which was less than the EJ subjects' lowest values. CONCLUSIONS: Experimental free muscle transplantation on the distal esophagus seems to have antireflux function, thus its therapeutic implications deserve further investigation. The incomplete efficacy of the procedure may suggest that a putative anatomic sphincter per se is not enough to promote an effective antireflux mechanism.

Animals↗

Delayed gastric emptying and gastroesophageal reflux: a pathophysiologic relationship.

BACKGROUND: Delayed gastric emptying (DGE) is frequent in patients with gastroesophageal reflux (GER), but its pathophysiologic role has not yet been established. To identify a relationship between DGE and GER, we assessed whether DGE increases esophageal acid exposure and the related importance of possible mechanisms. METHODS: Thirty pediatric patients with pathological GER were divided according to gastric emptying scintigraphy into a DGE group (n = 14) and normal-emptying group (n = 16). The esophageal pH-monitoring parameters of the two groups were compared with respect to the individual variation between postprandial and fasting periods. RESULTS: Patients with DGE had less total acid exposure than did those with normal emptying, but patients in both groups had a pathological fraction of time when pH was below 4 in both the postprandial (median: 18 vs. 27.6; P = 0.49) and fasting (8.5 vs. 23.9; P = 0.01) periods. Patients in the normal-emptying group had similar fraction of time when pH was below 4 in the postprandial and fasting periods. However, patients in the group with DGE had a fraction of time when pH was below 4 in the postprandial period that was almost double that presented in fasting period (postprandial to fasting ratio: 2.11:0.90; P = 0.002). The postprandial to fasting ratio for episodes per hour was similar in the two groups (1.81 vs. 1.79; P = 0.62). Patients with DGE had a significantly higher frequency of long episodes in the postprandial period than did those with normal emptying (62.5% vs. 38.2%; P = 0.04). The occurrence of the longest episode in the postprandial period was also significantly higher for patients with DGE (57.1% vs. 6.2%; P = 0.003). CONCLUSIONS: DGE seems to accentuate postprandial reflux by increasing the volume of refluxate per episode of reflux through an underlying incompetent lower esophageal sphincter.

Case-Control Studies↗

Prenatal diagnosis of abdominal enteric duplications.

Alimentary tract duplications are rare congenital malformations with few reports of the antenatal sonographic appearance. Early diagnosis is of paramount importance to prevent complications. We present a case of a cystic gastric duplication diagnosed antenatally at 31 weeks' gestation, which was treated successfully. Simultaneously, we review all published cases of prenatally diagnosed enteric duplications.

Adult↗

Acute gastric volvulus secondary to a Morgagni hernia.

Gastric volvulus (GV) is a rare surgical emergency in infancy and childhood. The first case of a child with an acute GV secondary to a Morgagni hernia (MH) is reported. The authors briefly discuss the etiology, clinical features, and management of acute GV and review the scattered reports of strangulated MHs in children.

Child, Preschool↗

Autotransplantation of the vermiform appendix for ureteral substitution.

Ureteral substitution with vermiform appendix has been reported rarely. Herein is reported a case of a traumatic ureteropelvic disruption with a 7-cm long ureteral defect that was bridged successfully by appendix interposition for the first time in a child. The potential use of vermiform appendix to repair long ureteral defects provides an additional argument against incidental appendectomy.

Anastomosis, Surgical↗