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An 8-year-old boy presented with an asymptomatic extratesticular, scrotal epidermoid cyst with extension across the urogenital diaphragm into the pelvis. While routine contrast studies and ultrasound were performed, magnetic resonance imaging was most useful in depicting the anatomical boundaries of the lesion, including the intrapelvic extension. Complete excision was performed transcrotally. The lesion is histologically indistinguishable from epidermoid cysts found elsewhere in the external genitalia, that is the penis, scrotum or testis. The etiology is unknown but it may represent a monolayer teratoma of germ cell origin or abnormal embryological closure of the median raphe.

Abnormal development of the lung in congenital diaphragmatic hernia (CDH) results in a dysfunctional surfactant system. In premature newborns at risk for respiratory distress syndrome, amniotic fluid lecithin/sphingomyelin (L/S) ratios and phosphatidylglycerol (PG) status have been successfully used to predict the surfactant status in the fetus. The objective of this study was to assess the accuracy of L/S ratios and PG in predicting the surfactant status in CDH. The surgically created lamb CDH model was used. Animals were delivered at 140 days' gestation (term 145) and immediately killed. Before delivery amniotic fluid was collected and L/S ratios and PG status were measured. Bronchoalveolar lavage (BAL) was performed and analyzed for total phospholipid and percent phosphatidylcholine (PC). Analysis of the BAL showed that the CDH lungs had both significantly less total phospholipid (CDH 0.10 +/- 0.03 mg/g versus control (CON) 0.76 +/- 0.28 mg/g) and PC (CDH 38 +/- 7.3% versus CON 70 +/- 3.4%) when compared with controls. In contrast the L/S ratios (CDH 2.44 +/- 0.26 versus CON 2.01 +/- 0.32) and PG status (CDH 8.75 +/- 1.01 nmol versus CON 10.2 +/- 0.9) were the same in CDH and control animals. The BAL from the CDH lamb model has a significant surfactant deficiency. Amniotic fluid L/S ratios and PG status were, however, not different between the control and CDH lambs. These results indicate that amniotic fluid L/S ratios and PG do not accurately predict the surfactant status of a fetus with CDH.

The pathophysiology of the lamb model of congenital diaphragmatic hernia (CDH) involves pulmonary hypoplasia, pulmonary hypertension, and surfactant deficiency. Inhaled nitric oxide (NO) is a highly selective pulmonary vasodilator. The aim of this study was to determine the effects of inhaled NO on pulmonary gas exchange, acid-base balance, and pulmonary pressures in a lamb model of CDH with or without exogenous surfactant therapy. At the gestational age of 78 days (full term, 145 days) 11 lamb fetuses had a diaphragmatic hernia created via a left thoracotomy and then were allowed to continue development in utero. After cesarean section, performed at term, six lambs received exogenous surfactant therapy (50 mg/kg, Infasurf) and five served as controls. All animals were pressure-ventilated for 30 minutes and then received 80 ppm of inhaled NO at an F1O2 of .9 for a 10-minute interval. Compared with the control lambs, the lambs with exogenous surfactant therapy had higher pH (7.17 +/- .06 v 6.96 +/- .07; P < .05), lower PCO2 (73 +/- 8 v 122 +/- 20, p < .05), and higher PO2 (153 +/- 38 v 50 +/- 23; P < .05). In control CDH lambs (without surfactant), inhaled NO did not improve pH, PCO2, or PO2, or decrease pulmonary artery pressure. In CDH lambs given exogenous surfactant, NO decreased pulmonary artery pressures (42 +/- 4 v 53 +/- 5; P < .005) and further improved PCO2 and PO2. NO also made the difference between pulmonary and systemic artery pressures more negative in the surfactant-treated lambs (-15 +/- 4 v -2.3 +/- 2.4; P < .005).(ABSTRACT TRUNCATED AT 250 WORDS)

The pathophysiology of congenital diaphragmatic hernia (CDH) results from a combination of pulmonary hypoplasia, pulmonary hypertension, and surfactant deficiency. Previously we demonstrated that inhaled nitric oxide (NO), a known vasodilator, only improves oxygenation and decreases pulmonary artery pressures when the lamb model of CDH is pretreated with exogenous surfactant. Nitric oxide synthase (NOS) in endothelial cells is responsible for the production of NO, a mediator of smooth muscle cell relaxation. Pulmonary hypertension in CDH may result from a defect in the endogenous production of NO. Our aim was to determine whether the main pulmonary artery trunks in CDH lambs have NOS immunoreactivity. Cryostat sections of paraformaldehyde-fixed specimens of pulmonary artery and aortic rings from 10 CDH lambs and five control lambs were processed for NADPH-diaphorase activity. Immunolocalization of NOS was studied in paraformaldehyde-fixed sections and compared with serially cut specimens from identical rings that were tested for NADPH-diaphorase activity. Intense NADPH-diaphorase staining was present in the intimal layer (endothelial lining) of the pulmonary artery and aortic rings of both the CDH and control lambs. This activity colocalized with NOS immunoreactivity in all specimens. Both NOS immunoreactivity and NADPH-diaphorase staining were lacking in cartilage, which were used as negative controls. NOS is present in the main pulmonary artery trunks of CDH lambs. To our knowledge, this is the first report of NOS immunoreactivity in CDH. We can only speculate whether this activity is preserved in other areas of the vascular tree in CDH, ie, pulmonary capillaries and veins. Perhaps the pulmonary hypertension in CDH is not caused by an NOS deficiency.

Two girls accidentally swallowed their toothbrush while inducing emesis; both had bulimia. Early removal of the brush is advised to prevent complications. Endoscopic removal is the preferred method, but because of the toothbrush's geometric qualities, surgical retrieval is often required.

Objective: We determined whether inhaled nitric oxide (NO) could improve systemic oxygenation in human neonates with hypoplastic lungs., Methods: A multicenter nonrandomized investigation was performed to study the efficacy of short-term NO inhalation. Inhaled NO was administered at 80 ppm to nine neonates without evidence of structural cardiac disease by echocardiography. Lung hypoplasia was due to congenital diaphragmatic hernia (CDH) in eight patients and to oligohydramnios in one patient. A total of 15 trials of NO inhalation were performed in these nine patients. Eight trials in seven patients were performed before extracorporeal membrane oxygenation ((ECMO); one patient had two trials) and seven trials were performed in five patients after decannulation from ECMO (two patients had two trials each)., Results: NO inhalation before ECMO did not change postductal PaO2 (42 +/- 3 mmHg vs 42 +/- 4 mmHg), oxygen saturation (SpO2; 89% vs 88%) or oxygenation index (31 +/- 4 cm H2O/torr vs 31 +/- 4 cm H2O/torr) for the group. All patients required ECMO support, which lasted from 5 to 17 days (mean 9). After decannulation from ECMO, NO inhalation increased postductal PaO2 from a median of 56 mm Hg (range 41 to 94) to a median of 113 mm Hg (range 77 to 326), P < .05. It decreased the oxygenation index from a median of 23 cm H2O/torr (range 11 to 7) to a median of 11 cm H2O/torr (range 4 to 21), P < .05. It increased SpO2 from 91% to 96% (P < .05) and pH from 7.48 +/- .03 to 7.50 +/- .03., Conclusion: In our patients with hypoplastic lungs, inhaled NO was effective only after ECMO. This could be due to maturational changes such as activating the endogenous surfactant system. Inhaled NO may be effective in neonates with hypoplastic lungs who have recurrent episodes of pulmonary hypertension after ECMO, even if they were previously unresponsive.

In a previous survey of successful candidates (SC) and unsuccessful candidates (USC) to the pediatric surgery matching program (PSMP), significant discriminating factors associated with SC included previous participation in basic science research, number of publications, number of national presentations, and contact with and letters of recommendation from well-known pediatric surgeons. The purpose of this study was to determine the selection criteria used by directors of the pediatric surgery training programs (PSTP) in choosing residents for the PSMP. A two-part survey of the PSMP was completed by directors of the PSTP (n = 24) to identify the factors (n = 31) that most influence their selection process when choosing a resident, and to help clarify questions (n = 11) posed by the candidates during the first survey. In part I, each factor was scored on a modified Likert Scale, from most (1) to least (5) important in evaluating candidates for training positions, and were ranked according to the mean score. Based on our previous survey, but blinded to the PSTP director respondents, these same factors were divided into groups that, in our first survey, did and did not discriminate between SC and USC for the PSMP, and will be referred to as "discriminating factors" (DF, n = 7) and "non-discriminating factors" (NDF, n = 8), respectively. Also, a series of questions dealt with subjective factors of the candidates, not previously analyzed in our original survey, and will be referred to as "program directors' factors" (PDF, n = 16). The three groups (DF, NDF, PDF) were analyzed by the Kruskal-Wallis test and analysis of variance (ANOVA).(ABSTRACT TRUNCATED AT 250 WORDS)

In addition to pulmonary hypoplasia and an altered pulmonary vascular bed, the abnormal lung development caused by congenital diaphragmatic hernia (CDH) is associated with an impairment in the pulmonary surfactant system. The aim of this study was to correlate fetal serum cortisol and triiodothyronine (T3) levels, known indicators of surfactant development, with maturation of the surfactant system in the lamb CDH model. Analysis of the products of bronchoalveolar lavage in CDH showed decreased phospholipid (P < .01) and increased protein concentration (P < .02) per gram of lung tissue when compared with controls, but cortisol and T3 concentrations did not differ significantly between the two groups. These results suggest that the surfactant deficiency in CDH is not related to an alteration in cortisol or T3 levels but may be directly related to the mechanical effects of the intrathoracic bowel.

Unlabelled: With the need for physicians to help control the rising cost of health care, consideration should be made, when appropriate, to shift more expensive inpatient care (IPC) to the less costly home care (HC) setting. The management of ruptured appendicitis (RA) includes appendectomy and intravenous (IV) antibiotics. Although patients' gastrointestinal function often returns by postoperative day 3 to 5, 10 to 12 days of IPC may be required for completion of the IV antibiotics. A new method of IV access, appropriate for pediatric patients and HC, is the PIC line--a peripherally inserted, centrally placed, intermediate-term silastic catheter. The purpose of this study is to compare the use of short plastic cannulas (SPC) and IPC tl PIC lines and HC in the management of patients with RA with respect to cost, length of hospitalization, morbidity, and patient/family acceptance., Methods: All children under the care of a single surgeon for RA underwent appendectomy and received IV antibiotics. The antibiotics were continued for a minimum of 10 days postoperatively, or until the patient was afebrile and the white blood cell count was less than 10,000/mm3. Group I (n = 8) had IV access via SPC and IPC care. Group II (n = 8) had a PIC line placed and was discharged to HC when intestinal function returned. The two groups were compared for: number of inpatient days (IPD), home care days (HCD), total care days (TCD), inpatient costs (IP$), home care cost (HC$), total care costs (TC$), number of IVs needed (#IV), catheter related complications, patient/family acceptance of the PIC line/HC concept, and overall morbidity., Results: The children with PIC lines (group II) were all discharged to HC by the seventh postoperative day and received an additional 5 days of antibiotics. The children on conventional inpatient therapy (group I) stayed in the hospital an additional 6 days (P = .002). Both groups had equivalent TCD. The children with PIC lines had lower IP$ (P = .002) and significantly lower (40%) TC$ than did IPC patients (P = .004). The children in group I required placement of more than five IV catheters during their hospitalization. In contrast, the PIC lines were successfully placed in group II patients, and no further IV access was necessary (P = .001). There were no complications from the PIC lines. Neither group experienced recurrent infections or required readmission. The patients' and families' acceptance of the PIC line/HC concept was unanimously favorable., Conclusion: When compared with SPC/IPC, PIC line/HC is a safe, efficacious, and significantly cost-effective method for the care of the patient with RA. As experience with PIC line/HC grows, it may become the preferred method of treatment for a variety of illnesses that currently require intermediate-length courses of IV therapy and IPC.

The aim of this study was to assess the impact of surfactant deficiency on the pathophysiology of congenital diaphragmatic hernia (CDH). Pregnant ewes were operated on at 80 days of gestation for creation of a diaphragmatic hernia in the lambs. Twenty-one lambs survived to be delivered by cesarean section and were studied. Compliance was improved when surface tension effects were removed by saline solution in lungs of both control animals and lambs with CDH; however, the lungs of the lambs with CDH still had significantly impaired compliance. In a second series of experiments, two groups were studied: a surfactant-treated and a control, nontreated group. Surfactant was given prophylactically into the liquid-filled lungs before the first breath. All lambs were paralyzed and sedated and their lungs mechanically ventilated with 100% oxygen for 30 minutes; gas exchange was then assessed, pressure-volume data were obtained, and compliance was calculated. Surfactant significantly improved gas exchange; arterial oxygen pressure increased from 39 +/- 11.4 to 316 +/- 53.6 mm Hg, arterial carbon dioxide pressure decreased from 148 to 63 mm Hg, and pH increased from 6.87 to 7.16 (p < 0.001). Lung volume at 25 cm H2O, functional residual capacity, and compliance were all increased (p < 0.02). Thus, in the CDH lamb model, pulmonary mechanics are impaired by both parenchymal and surfactant abnormalities. Both lung mechanics and gas exchange are markedly improved by exogenous surfactant therapy.

Improvements in screening and diagnostic techniques now mean that hundreds of congenital anomalies can be antenatally diagnosed. It is, however, impossible and inappropriate to submit all pregnant women to a barrage of investigations. Screening is necessary before specific invasive investigations are initiated. These include history, physical examination, MS-AFP screening, estriol and hCG screening, and a Level II ultrasonography scan. Once at-risk pregnancies have been identified, a multidisciplinary team approach is commenced and further studies including Level II ultrasonography, amniocentesis, chorionic villus sampling, or cordocentesis can be performed so that an accurate diagnosis is available. Counseling of the parents throughout is essential so that appropriate decisions regarding this and further pregnancies can be made.

Cystic fibrosis is most often the underlying cause of meconium ileus. We describe the diagnosis and treatment of a patient with chronic intestinal pseudo-obstruction, and not with cystic fibrosis, whose initial manifestation was meconium ileus.

The hypothesis of this article is that growth of the fetal lung is stimulated by a pulmonary growth factor (PGF) produced by the kidneys, which is modulated by a feedback signal from the lungs, a pulmonary-derived renotropin (PDR). In the fetus with pulmonary hypoplasia (PH), the lungs may maximally stimulate this feedback loop to release more PDR, resulting in continual stimulation of the kidneys and renal enlargement. If such a schema plays a role in the pathophysiology of PH, newborn infants with congenital diaphragmatic hernia (CDH) or chronic amniotic fluid leak (CAFL) should have enlarged kidneys. To investigate this hypothesis, we created models of CDH in fetal lambs and CAFL in fetal rabbits, and then analyzed lung (Lu) and kidney (K) growth. When compared to controls, newborn CDH lambs had significantly smaller lungs and larger kidneys. The lungs were hypoplastic as defined by either decreased lung weight/body weight (LuW/BW), lung DNA/body weight (Lu DNA/BW), or lung total protein/body weight (LuTP/BW) (P < .01). Renal hyperplasia was confirmed by KW/BW, K DNA/BW (P < .01), and KTP/BW (P < .05). An inverse relationship between lung size and kidney size could be described by the equation KW/BW = 1.04 - 0.12 LuW/BW (r = -.75). The CAFL model in newborn rabbits produced severe oligohydramnios when compared with controls (P < .01). This resulted in fetuses with smaller lungs and larger kidneys as compared with those of controls. The lungs were significantly smaller and more hypoplastic than controls when compared by LuW (P < .01), LuW/BW (P < .01), Lu DNA/BW (P < .05), and Lu TP/BW (P < .01).(ABSTRACT TRUNCATED AT 250 WORDS)

A 19-day-old male infant was seen with full-thickness gangrene of the transverse colon. Hyperosmolar formula feedings caused by inadequate "directions for use" was the probable cause. Improved formula labeling might prevent further cases.

Exogenous surfactant therapy (EST) in surfactant-deficient premature infants has been shown to improve lung compliance, decrease morbidity, and improve survival. Reports have demonstrated that newborns with congenital diaphragmatic hernia (CDH) have lung compliance, pressure-volume curves, and hyaline membrane formation resembling those changes seen in surfactant deficient premature newborns. We hypothesize that EST may also benefit infants with CDH. All high risk cases of prenatally diagnosed CDH at Children's Hospital of Buffalo from November 1988 to February 1991 were prospectively evaluated for EST. In those families who chose to participate, the surfactant preparation, Infasurf (100 mg/kg), was instilled into the newborn's lungs prior to the first breath. The remainder of the perinatal, neonatal, and surgical care was performed in a routine manner. Three high-risk prenatally diagnosed newborns with left CDH were treated with EST. All showed signs of decreased pulmonary compliance, but could still be adequately oxygenated and ventilated. Surgical correction was performed after stabilization and all required patch closures. Two of the three infants suffered no life-threatening episodes of pulmonary hypertension and all survived. These infants had many known indicators for poor outcome in CDH with an expected survival of less than 20%. We believe that EST in these neonates with CDH contributed to their survival with minimum morbidity. These results suggest that surfactant replacement for the high-risk neonate with CDH warrants further consideration and a randomized clinical trial is being planned.

The high mortality for congenital diaphragmatic hernia (CDH) has been attributed to a combination of pulmonary hypoplasia and pulmonary hypertension. We hypothesize that a surfactant deficiency may in part be contributing to the pathophysiology of CDH. This study documents the functional, quantitative, and qualitative aspects of the surfactant status of the alveolar air-liquid interface and the type II pneumocyte in the fetal lamb CDH model. Ten lamb fetuses (gestational age, 80 days) had a CDH created via a left thoracotomy and then were allowed to continue in utero development until term. Three litter mates and three nonoperated time-dated fetuses served as controls. At term, pressure-volume curves were performed to measure pulmonary compliance and total lung capacity. Alveolar lavage was then performed to measure the quantitative and the qualitative aspects of pulmonary surfactant. Finally, isolation of type II pneumocytes allowed quantification of phospholipid synthesis. When compared with controls (N = 6), the CDH lambs (N = 5) had significantly smaller lungs (P = .009), decreased total lung capacity (P less than .001) and compliance (P less than .001), reduced total lavaged phospholipids (P = .006), and decreased percent phosphatidylcholine (P = .02). CDH lambs also had increased total lavaged proteins (P = .05) and higher minimum dynamic surface tension (P less than .001). A surfactant deficiency may be contributing to the pathophysiology of CDH. Surfactant replacement therapy in premature infants has been shown to improve lung compliance, decrease morbidity, and improve survival. Exogenous surfactant may also benefit infants with CDH.

We report a patient with congenital neutropenia or Kostmann's Syndrome who suffered many complications after presenting with Clostridium septicum enterocolitis, including absence of wound healing. Because of several reports of the use of granulocyte colony-stimulating factor (G-CSF) in patients with various complications of neutropenia, we treated this patient with recombinant human (rh) G-CSF. We found that once rhG-CSF restored neutrophil counts to normal, progressive wound healing followed. Thus, rhG-CSF therapy may be useful in treating neutropenic patients with wound complications.

Only one third of the applicants for training in pediatric surgery obtain a position through the pediatric surgery matching program. In order to identify factors that contribute to a successful outcome, we conducted a retrospective survey of all participants in the matching process for positions during the years 1983 to 1991. This survey was designed to identify characteristics associated with success in the match through comparison of successful and unsuccessful applicants. Significant factors associated with a successful match included: a greater incidence of residency-supported research (P = .012) with a greater number of publications (P = .003) and national presentations (P = .014), specifically at the annual meetings of the American Pediatric Surgical Association (P = .05) and the American Academy of Pediatrics (P = .05). In addition, successful candidates had more contact with (P = .004) and letters of recommendation from (P = .015) well-known pediatric surgeons involved in the general surgical residency program. This information should be invaluable to those counseling medical students and residents interested in a career in pediatric surgery.

Multifactorial sex-modified inheritance has been proposed as the model of transmission in familial Hirschsprung's disease (HD). A review of two separate kindreds suggests that aganglionosis may be inherited as an X-linked recessive or an autosomal dominant trait. Chromosomal anomalies and other syndromes, including G6PD deficiency, may occur with familial HD. Recurrence risk counseling for family members depends on accurate pedigree analysis and a comprehensive understanding of the genetic factors involved.

Swallowed toothbrushes were noted in the esophagus of one teenager and the stomach of two others with bulimia. The presence of a toothbrush in the lumen of the gastrointestinal tract should make the radiologist suspicious of bulimia/anorexia nervosa. A toothbrush shows a characteristic radiographic image with parallel rows of short metallic radiodensities due to the metallic plates that hold the bristles in place.

Mediastinitis is a devastating complication following median sternotomy. Continuous povidone-iodine (PVP-I) irrigation has been advocated as therapy because of its broad antimicrobial spectrum and its apparent safety. However, several recent clinical reports have warned of suspected local and systemic iodine toxicity. The purpose of this study is to determine if significant amounts of iodine can be absorbed systemically via the mediastinum, and if so, what toxicity (local and/or systemic) may result. PVP-I (0.5%) was continuously irrigated into the pericardial sacs of three dogs via catheters for 48 hr. Serial serum and urine iodine levels were determined. The serum steady-state concentration (Css), the rate elimination constant (k), the urinary clearance (Cl), and the serum half-life (t 1/2) for iodine were assessed. Serum electrolytes, Bun, Cr, and arterial pH were measured to assess systemic iodine toxicity. Tissue samples of the heart, pericardium, liver, and kidney were examined histologically for evidence of local or end-organ iodine toxicity. This study demonstrated that the absorption of iodine during continuous mediastinal irrigation with PVP-I follows zero-order pharmacokinetics, just as if it were being given by continuous intravenous infusion. The baseline serum iodine concentration was 145.9 +/- 64.3 micrograms/dl, Css was 29,290 +/- 101.4 micrograms/dl, k was 0.0996 +/- 0.009/hr, Cl was 872.4 +/- 119.3 ml/hr, and t1/2 was 6.22 hr. Urinary excretion of iodine increased in proportion to the serum iodine. Measured serum chloride increased in a linear manner (r = 0.949), while serum Na, K, Bun, Cr, and pH were unchanged.(ABSTRACT TRUNCATED AT 250 WORDS)

Anomalies of the urinary tract occur in some 13-27% of infants with congenital posterolateral diaphragmatic defect (CDD) and are often severe (renal agenesis, dysplasia, hypoplasia, or hydronephrosis). To test the hypothesis that urinary and diaphragmatic anomalies share elements of pathogenesis, we reviewed 60 autopsy cases of CDD studied at our institution. Sixteen patients (27%) manifested anomalies of the urinary tract: 12 had markedly altered kidneys, 8 of which were unilateral and ipsilateral to the diaphragmatic defect. Among 27 patients free of gross urinary tract anomalies, kidney weights formed a skewed distribution, with most values above published norms for body weight; by analysis of covariance, kidney weight (as a function of body weight) was significantly greater in CDD than in a control population of infants free of chronic illnesses and congenital anomalies who died suddenly and unexpectedly. Differences in glomerular number and diameter could not be identified between the latter groups. In 71% of patients with isolated left CDD, the left kidney was heavier than the right, a reversal of the usual condition in infancy. These findings demonstrate that both marked and subtle changes of the urinary tract in CDD are generally ipsilateral to the diaphragmatic defect and suggest that the pathogenetic mechanisms responsible for urinary and diaphragmatic anomalies may overlap topographically. Aberrant morphogenesis within a developmental field or fields is one explanation for this.

Survival of newborns with congenital diaphragmatic hernia (CDH) is largely dependent on the severity of pulmonary hypoplasia (PH) present at birth. Intrathoracic compression by the herniated abdominal viscera is thought to be the primary factor involved in the pathogenesis of the PH in CDH. Humoral and/or amniotic pulmonary growth factors (PGF) have been hypothesized to play a role in normal fetal pulmonary development and may be involved in the pathogenesis of CDH as well. The hypothesis of this paper is that growth of the fetal lung is stimulated by a PGF produced by the kidneys, which is modulated by a feedback signal from the lungs, a pulmonary derived renotropin (PDR). In the fetus with CDH, the lungs may be unable to respond to PGF due to compression by the herniated abdominal viscera. Theoretically, PH associated with CDH would maximally stimulate this feedback loop to release more PDR, resulting in continual stimulation of the kidneys and renal enlargement. If such a scheme plays a role in the in utero pathophysiology of CDH, then newborns with CDH should have enlarged kidneys. To investigate this hypothesis, we reviewed 30 autopsy cases of newborns with CDH and analyzed their kidney weights versus body weights, using historical data as control. Kidney weights in CDH cases were greater than the control population in 77% of the cases; 57% of kidney weights were more than one standard deviation above control values. Adjusted group mean kidney weights were 29.8 g (+/- 1.0 SE) in CDH cases and 25.9 g (+/- 1.5 SE) in the control population (P less than .04). These data support our hypothesis and demonstrate that in newborns with CDH and morphologically normal kidneys, there is significant renal enlargement associated with CDH. The presumed mechanism of this renal enlargement, as well as its relationship to normal and aberrant pulmonary growth and regulation are discussed. If such a selective PGF exists, its therapeutic implications for fetuses and newborns with PH are considerable.

Is gastroschisis embryologically distinct from omphalocele or simply a ruptured small omphalocele (hernia of the umbilical cord)? Serial sonographic imaging of a fetus with a small omphalocele at 27 weeks gestation has now provided the "missing link" in the pathogenesis of gastroschisis by documenting in utero rupture resulting in a gastroschisis.

Inhibition of fetal breathing movements or increased loss of fetal lung fluid into the amniotic space have been suggested as two possible mechanisms causing pulmonary hypoplasia in the setting of oligohydramnios (OH). We examined the effect of OH produced by amniotic fluid shunting (AS) into the maternal abdominal cavity, ablation of fetal breathing by high cervical cord transection (CT), and CT and AS combined on fetal rabbit lungs at 24 days gestation. Lung growth at term (31 days) was measured by lung DNA content and wet lung weight. Compared to unoperated controls, newborns undergoing either AS alone or CT alone had much smaller lungs. When compared to CT alone, CT with AS resulted in a further significant decrease in lung growth. Thus, even when fetal breathing was eliminated by CT, AS caused further hypoplasia. If pulmonary hypoplasia in OH is related to increased loss of lung fluid, then tracheal ligation (TL) should prevent this process. TL combined with AS produced lungs with the same DNA content as controls, and thus the hypoplastic effects of OH were reversed by TL. Although fetal breathing is clearly important for lung growth, it appears that inhibition of fetal breathing is not the predominant etiology of oligohydramnios-related pulmonary hypoplasia. Fetal intrapulmonary fluid is formed by active transport across pulmonary epithelium, and may serve to distend potential airways and stimulate growth. These experiments suggest that lung hypoplasia associated with OH is related to loss of this internal stenting force.

Electron irradiation of organic specimens adversely affects the validity and reliability of quantitative microanalytical results through the loss of light element constituents of the specimen. Characteristic and continuum x-ray count rates will vary for the duration of the irradiation effects, which require electron doses of 10(-11) to 10(-8) C/micrometer 2. The magnitude of these variations is dependent upon the light element content, thickness and temperature of the specimen and upon the operating conditions used during analysis. The initial count rate at the onset of the electron beam is the most valid count rate to be used in the quantitation of organic specimens, but it can only be accurately determined when the analyzed element exists in high concentration within the specimen or when the beam is grossly defocused. Low temperature analysis will prolong the duration of the mass loss and decrease its magnitude, but erratic count rates, specimen charging due to condensation and inconsistent mass losses have been observed for different specimens. The physics and chemistry of the electron beam-specimen interactions impose limitations upon the potential accuracy in biological microanalysis whic, if ignored, can result in large errors in quantitative projections.

We compared simultaneous healing processes in fetal, newborn, and maternal rabbits using a miniaturized wound cylinder of expanded Gore-Tex tubing. The tubing was placed subcutaneously in fetal and maternal rabbits on day 23 of pregnancy (term = 31 to 32 days), and in 7-day-old newborn rabbits. At specific intervals, the tubing was removed and analyzed for hydroxyproline accumulation, histology, and cellular enzyme-histochemistry. Granulation tissue ingrowth and accumulation of hydroxyproline were each inversely related to age (fetus greater than newborn greater than maternal). The fetus showed an impressive infiltration of macrophages by day 4, fibroblasts by day 7, and a conspicuous lack of neutrophils in all specimens. Newborns and mothers had few cells until day 7, when a mixture of macrophages, neutrophils, and some fibroblasts appeared. We conclude that the fetus heals wounds rapidly by both mesenchymal cell proliferation and collagen deposition, and that these processes are more rapid in fetuses than in newborn or adult animals despite relative fetal hypoxemia.

Six cases of sonographically diagnosed fetal sacrococcygeal teratoma (SCT) are presented and illustrate the variable features of fetal SCT. The sonographic findings assisted the parents and perinatal team in making decisions, and in two of the cases the children survived after elective Cesarean section and prompt neonatal resection of the tumors. None of the patients showed signs of malignant degeneration of the teratoma or metastases. Fetal SCT no longer should be considered a uniformly fatal condition. The literature on sacrococcygeal teratoma detected after birth indicates that the mortality rate is correlated with the degree of extension of the tumor. Therefore, the classification of sonographically diagnosed fetal SCT according to its size and position is important for decisions regarding pregnancy management.

Continuous povidone-iodine irrigation is frequently used to treat mediastinitis after median sternotomy and has been considered safe and effective. We describe a 34-month-old patient with mediastinitis after median sternotomy who was treated with continuous povidone-iodine irrigation and who absorbed toxic quantities of iodine (total serum iodine, 9,375 micrograms/dl; normal range, 4.5 to 9.0 micrograms/dl). An unexplained metabolic acidosis developed, along with changes in mental status, and the patient died. This experience and a thorough review of the literature lead us to believe that continuous povidone-iodine irrigation of the mediastinum is contraindicated.

A model of congenital hydrocephalus in utero in fetal lambs and rhesus monkeys has been produced by the intracisternal injection of kaolin. Initial attempts to produce hydrocephalus using silicone oil were unrewarding. Hydrocephalus had developed by 2 weeks post-injection and could be followed by ultrasonography. The pathological findings were similar to those reported using kaolin in other species. Ventriculoamniotic shunting, when successful, was capable of partially reversing the deleterious effects of hydrocephalus. The major drawback of the present model is that hydrocephalus is produced during the second rather than the first trimester of pregnancy. However, kaolin produces mainly an obstructive hydrocephalus without other associated brain or systemic anomalies.

Acute normovolemic hemodilution is a safe technique for minimizing operative blood loss during major tumor resection in children. Based on our experience using hemodilution anesthesia in 14 successful extensive tumor resections, we conclude the following: (1) this is an effective means of reducing use of bank blood and thus avoiding the risks of multiple transfusions; (2) it facilitates surgical dissection due to increased visibility with dilute blood, and decreased bleeding due to controlled hypotension; (3) this technique is acceptable for Jehovah's Witnesses; (4) hetastarch is an effective, inexpensive colloid hemodiluent which minimized perioperative edema compared to crystalloid hemodilution.

Six infants with short-gut syndrome refractory to medical management underwent isoperistaltic colon interposition (length 11.7 +/- 3.1 cm.). The abdominal catastrophes that required extensive intestinal resection were: volvulus (3), necrotizing enterocolitis (2), and gastroschisis with intestinal atresia (1). No infant had another major congenital anomaly. The average trial of attempted medical therapy prior to colon interposition was 5.5 +/- 3.6 months. There was no perioperative mortality or morbidity associated with the colon interposition. Following the colon interposition, three infants were weaned from total parenteral nutrition (TPN) in 3 +/- 1 months and all survived. In contrast, three infants could not be withdrawn from TPN and they died secondary to complications of TPN (2 from sepsis and 1 from hepatic failure). Long-term survival was associated with a greater length of small bowel remaining after the initial resection (51 +/- 12 cm v 35 +/- 24 cm), colon interposition at a younger age (3 +/- 1 months v 8 +/- 3.5 months), and a shorter duration of medical management prior to colon interposition (2.8 +/- 0.8 months v 6.7 +/- 5.0 months). All survivors are now tolerating a regular diet and having one to four formed stools per day. Normal somatic growth and developmental milestones are being achieved. The follow-up period is from 24 to 84 months. Our experience with the colon interposition in the patient with short gut syndrome has led us to conclude that when a reasonable trial of medical management has failed, a colon interposition is a safe and effective adjuvant to treatment.

Is the renal dysplasia (RD) commonly seen in babies with urinary tract obstruction a developmental consequence of the obstruction or is it an associated embryologic malformation? We produced complete unilateral ureteral obstruction in six 58- to 66-day old lamb fetuses by clipping a silastic ring on the ureter. Three fetuses survived to term. All obstructed kidneys were not only grossly hydronephrotic but dysplastic by histologic criteria; ie, parenchymal disorganization, primitive epithelial structures, and marked fibrosis. The contralateral unobstructed control kidneys were normal. The changes were similar to those seen in the human neonate with obstruction and RD, suggesting that in this model RD was caused by obstruction to the flow of urine early in fetal development.

To study the effect of in utero ventricular decompression of hydrocephalus on brain development and prognosis, and to evaluate the function and possible complications of different shunt designs, we created fetal hydrocephalus in 28 fetal lambs and 17 fetal monkeys by injecting kaolin into the cisterna magna during the third trimester. One fetal lamb had indwelling intracranial subdural and amniotic cavity pressure catheters placed to study serial changes in the relationship of intracranial pressure (ICP) and amniotic fluid pressure (AFP) from the time of injection until term. Twenty hydrocephalic fetal lambs underwent ventricular decompression--ventriculoamniotic (V-A), N = 10; ventriculo-right atrial (V-RA), N = 9; and ventriculo-pleural (V-PL), N = 1--21 to 25 days after the kaolin was injected; seven hydrocephalic fetuses were left unshunted as controls (CON). Eight fetal monkeys underwent V-A decompression 14 to 21 days after kaolin injection; nine were left unshunted as controls. All animals were delivered by cesarean section near term, assessed for viability and their brains examined grossly and microscopically. After kaolin injection in the fetal lamb, ICP (r = 0.94) and ICP minus AFP (r = 0.93) rose in a linear fashion, while AFP showed no trend (r = 0.22). All unshunted newborn lambs and monkeys had split sutures, dilated ventricles, and thinned cortical mantle. Most shunted lambs showed anatomic improvement with decreased head circumference, overriding sutures, normal-sized ventricles, and improved survival. However, histopathology revealed marked white matter destruction. In contrast, most shunted monkeys showed little anatomic improvement. Both shunted and unshunted monkey brains showed a severe inflammatory ventriculitis. In both lambs and monkeys, shunting was associated with a variety of complications including subdural hematoma, subdural hygroma, shunt infection, shunt occlusion, and improper shunt tip placement. In this experimental model, in utero decompression of obstructive hydrocephalus improves overall survival, improves gross ventriculomegaly, does not improve histopathologic brain damage, and is associated with significant complications. The choice of the optimal shunt design and the effect on postnatal neurologic function requires further study. This work emphasizes the need for continuing research in an animal model prior to human application.

Renal dysplasia (RD) is commonly seen in babies with urinary tract obstruction (UTO). Recent experimental evidence suggests that early fetal UTO leads to the development of RD. The RD seen in children with congenital UTO is usually not reversible, even when the obstruction is relieved soon after birth. Is the RD associated with congenital UTO preventable or reversible by decompression of the urinary tract early in gestation? If so, at what stage of development must this decompression be performed? We produced complete unilateral ureteral obstruction in 25 early second trimester (62 to 65 days) lamb fetuses, a procedure that results in ipsilateral RD at term (140 days). At a second operation, 20, 40, or 60 days after the initial procedure, we decompressed the obstructed kidney by a cutaneous end-ureterostomy. The contralateral unobstructed kidneys served as controls. Renal function (urine output and iothalamate clearance) and histopathology were evaluated after delivery at term. Recovery of renal function was directly proportional to the duration of in utero decompression and inversely proportional to duration of obstruction. In addition, in utero decompression prevented or greatly ameliorated the development of RD. However, some postobstructive changes persisted; these were proportional to the length of in utero obstruction. These results substantiate the clinical impression that some human fetuses with congenital UTO may benefit from early in utero decompression.

The accuracy of absolute quantitation within thick, mineralized tissue specimens is influenced by count rate variations of characteristic X-rays during electron microprobe analysis. The variations occur for electron doses approximately greater than 10(-10) C/micrometer2 and are primarily dependent upon the light element fraction within the irradiated volume. Specimen preparation procedures affect both count rate dynamics and interpretatin of microanalytical results. X-ray intensity data acquird at initial electron exposure and utilized in standard matrix correction schemes will project valid elemental concentrations for known calcium compounds over wide ranges of specimen density. Measurement error could approach +/- 2-3% for the major elemental constituents in mineralized tissues, but only with appropriate control or interpretation of electron irradiation phenomena.

Maternal serum alpha-fetoprotein (MS-AFP) screening has become part of routine obstetric care. Although elevated MS-AFP was originally associated with neural tube defects (NTD), it is also able to detect several fetal anomalies of interest to a pediatric surgeon, ie, ventral abdominal wall defects, intestinal atresias, and sacrococcygeal teratomas. Previously, decreased MS-AFP had only been associated with fetal trisomies, but not surgically correctable lesions. In the present study, we review our recent experience with both elevated and decreased MS-AFP as a marker to detect fetal anomalies of concern to the pediatric surgeon. Forty-one fetal anomalies were associated with 333 pregnancies referred for follow-up after abnormal MS-AFP screening results from November 1985 through November 1986. One hundred ninety-six were elevated and 139 were decreased. In most cases, evaluation included counseling, repeat MS-AFP, level II ultrasound, and amniocentesis. This revealed elevated MS-AFP to be associated with 32 (16.3%) anomalies (2 NTD, 5 anencephalics, 5 ventral abdominal wall defects, 1 stage IV-S neuroblastoma, 1 renal anomaly, 1 ventriculomegaly, 15 fetal demises, and 2 fetal-maternal bleeds). Decrease in MS-AFP was associated with nine (6.4%) anomalies (2 congenital diaphragmatic hernias, 3 Down's syndrome, 1 Turner's syndrome, 2 duodenal atresias, and 1 choroid plexus cyst). In this study, MS-AFP detected several fetal anomalies known to be associated with abnormal MS-AFP and three anomalies not previously described (congenital diaphragmatic hernia, neuroblastoma, and choroid plexus cyst). Elevated as well as decreased MS-AFP are significant and should be pursued by a full prenatal evaluation.(ABSTRACT TRUNCATED AT 250 WORDS)