Rare urologic pathology presenting as abdominal masses in children

The key to correct diagnosis of abdominal masses in children is awareness of all the possibilities. Three children with abdominal masses of uncommon urologic origin are described. The lesions were adrenal cortical carcinoma, a pancake pelvic kidney and a non-functioning hydronephrotic segment of a horseshoe kidney. Management of these patients is discussed and the literature is reviewed.     

Intestinal atresia and stenosis: a 25-year experience with 277 cases

OBJECTIVE: To evaluate the causes, clinical presentation, diagnosis, operative management, postoperative care, and outcome in infants with intestinal atresia. DESIGN: Retrospective case series. SETTING: Pediatric tertiary care teaching hospital. PATIENTS: A population-based sample of 277 neonates with intestinal atresia and stenosis treated from July 1, 1972, through April 30, 1997. The level of obstruction was duodenal in 138 infants, jejunoileal in 128, and colonic in 21. Of the 277 neonates, 10 had obstruction in more than 1 site. Duodenal atresia was associated with prematurity (46%), maternal polyhydramnios (33%), Down syndrome (24%), annular pancreas (33%), and malrotation (28%). Jejunoileal atresia was associated with intrauterine volvulus, (27%), gastroschisis (16%), and meconium ileus (11.7%). INTERVENTIONS: Patients with duodenal obstruction were treated by duodenoduodenostomy in 119 (86%), of 138 patients duodenotomy with web excision in 9 (7%), and duodenojejunostomy in 7 (5%) A duodenostomy tube was placed in 3 critically ill neonates. Patients with jejunoileal atresia were treated with resection in 97 (76%) of 128 patients (anastomosis, 45 [46%]; tapering enteroplasty, 23 [24%]; or temporary ostomy, 29 [30%]), ostomy alone in 25 (20%), web excision in 5 (4%), and the Bianchi procedure in 1 (0.8%). Patients with colon atresia were managed with initial ostomy and delayed anastomosis in 18 (86%) of 21 patients and resection with primary anastomosis in 3 (14%). Short-bowel syndrome was noted in 32 neonates. MAIN OUTCOME MEASURES: Morbidity and early and late mortality. RESULTS: Operative mortality for neonates with duodenal atresia was 4%, with jejunoileal atresia, 0.8%, and with colonic atresia, 0%. The long-term survival rate for children with duodenal atresia was 86%; with jejunoileal atresia, 84%; and with colon atresia, 100%. The Bianchi procedure (1 patient, 0.8%) and growth hormone, glutamine, and modified diet (4 patients, 1%) reduced total parenteral nutrition dependence. CONCLUSIONS: Cardiac anomalies (with duodenal atresia) and ultrashort-bowel syndrome (<40 cm) requiring long-term total parenteral nutrition, which can be complicated by liver disease (with jejunoileal atresia), are the major causes of morbidity and mortality in these patients. Use of growth factors to enhance adaptation and advances in small bowel transplantation may improve long-term outcomes.     

Sarcoidosis presenting as an intra- or extra-axial cranial mass: report of two cases

Sarcoidosis may also present as an extra- or intra-axial mass involving the central nervous system. These lesions are sometimes operated upon, because a neoplasm is suspected. We report two cases of unusual tumour-like extra- and intra-axial sarcoidosis. The extra-axial mass was just medial to the jugular foramen. Its morphology and signal characteristics differed from the more common lesions in this area. The intra-axial mass was in the temporal lobe, with only minor leptomeningeal involvement. Extra-axial sarcoidosis can be confused with a meningioma because these lesions can give relatively low signal on T2-weighted images. Intra-axial masses are presumed to represent a propagation and fusion of multiple leptomeningeal granulomas through the Virchow-Robin spaces in the brain; this pattern can be sought on contrast-enhanced T1-weighted images.     

Tracheomalacia with esophageal atresia and tracheoesophageal fistula in fetal rats

BACKGROUND: Many patients who have esophageal atresia and tracheoesophageal fistula (EA-TEF) have associated tracheomalacia, which is thought to be one of the reasons for respiratory complications after surgical correction of the abnormality. METHODS: In this study, tracheas from Adriamycin-induced EA-TEF fetal rats were examined histologically and relevant cross-sectional parameters of the tracheas were measured. RESULTS: The tracheal lumen in tracheomalacia was small and irregular, losing its normal "D" shape. In most rats, the cartilaginous ring was broken into two to four segments, making the trachea lose its rigid support. The submucosa was thickened with prominent bulging of its membranous part into the tracheal lumen. The ratio of the inner luminal cross-sectional area to the outer tracheal cross-sectional area in EA-TEF rats was 15.7%, compared with a control ratio of 47.2%. In EA-TEF rats, the length of the cartilaginous ring was significantly shortened (P < .001), but not the length of membranous trachea, thus resulting in a cartilaginous/membranous (C/M) ratio of 1.55:1, markedly lower than that of normal rats (4.34:1, P < .001). The reduction of anterior-posterior diameter of the tracheal lumen was more marked than that of the transverse diameter. CONCLUSIONS: These observations suggest that the trachea in EA-TEF rats has a smaller lumen and is more flaccid than normal, making it prone to airway obstruction. The fact that tracheomalacia developed only in fetuses who had EA-TEF indicates that the factors that result in EA-TEF also cause tracheomalacia.     

Does duodenal atresia and stenosis prevent midgut volvulus in malrotation?

The risk-benefit balance when aspirin is compared with aspirin combined with ticlopidine is being investigated in several multicentre trials (MUSIC and WEST II versus TASTE, MUST, and STARS respectively). Cardiologists follow one of two strategies. Some prefer a more aggressive antiplatelet regimen, disregarding the risk of neutropenia (0.7%) because they want to avoid lessening the therapeutic effect of vessel patency obtained with stent implantation. Others give only aspirin (a money saving approach) confident that IVUS inspection (an expensive approach) will allow an adequate evaluation of full stent expansion and lesion coverage, despite a more pronounced activation of the coagulation cascade. Our impression so far is that the combination of ticlopidine and aspirin has a more favourable risk-effect balance.     

Anatomic variations in underdeveloped right ventricle related to tricuspid atresia and stenosis

In the anatomy of 416 hearts, the seat of tricuspid stenosis or atresia is examined, with special reference to Fontan-like surgical procedures. A classification is offered which includes cases with and without regular or inverted transposition, and with decreased or increased pulmonary flow. The size and thickness of the right atrium, the size and architecture of the right ventricle, the size of the pulmonary tree, the types of atrial and ventricular septal defects, the condition of the mitral valve, and the size and thickness of the left atrium and left ventricle are analyzed. In addition the various intracardiac and extracardiac abnormalities are enumerated. Reference is also made to the tendency of the aorta and pulmonary trunk to override the septum, in some cases producing double-outlet left ventricle. It is considered that many cases of tricuspid valve atresia and stenosis with or without transposition may be amenable to Fontan-like procedures in the proper age group. All the above anatomic considerations have a bearing on the suitability and type of operative tricuspid bypass procedures, and they may influence the prognosis of surgical therapy.     

Determinants of successful balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum

OBJECTIVES: This study reviewed our experience with percutaneous balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum and defined the anatomic and hemodynamic characteristics of infants in whom this procedure is successful and provides definitive therapy. BACKGROUND: Unlike children with valvular pulmonary stenosis, the follow-up of infants with critical pulmonary stenosis undergoing percutaneous balloon valvotomy is limited. METHODS: Between December 1987 and August 1992, percutaneous balloon valvotomy was attempted in 12 infants with critical pulmonary stenosis (n = 10) or pulmonary atresia with intact ventricular septum (n = 2). Two outcome groups were identified: Group A patients are acyanotic, have mild residual pulmonary stenosis and have not required operation; Group B patients have required operation. RESULTS: Of the 12 infants, 11 had a successful balloon valvotomy procedure. Group A patients (n = 7) have a residual gradient of 22 +/- 18.7 mm Hg (mean +/- SD) at follow-up of 3.2 years (range 1.2 to 5.0). In Group B (n = 5), operation was required for inability to cross the pulmonary valve (n = 1) or persistent severe hypoxemia for > or = 2 weeks after valvotomy (n = 4). Significant differences (p < or = 0.01) between the two groups (Group A vs. Group B) were identified in pulmonary valve annulus (Z value) 8.1 mm (-1.1) versus 5.5 mm (-3.4); tricuspid valve annulus (Z value) 14.0 mm (0.8) versus 8.8 mm (-1.8); right ventricular volume 65 versus 29 ml/m2; and Lewis index 10.9 versus 8.9. CONCLUSIONS: Percutaneous balloon valvotomy is effective and likely to provide definitive therapy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum who have a tricuspid valve annulus > 11 mm, pulmonary valve annulus > or = 7 mm and right ventricular volume > 30 ml/m2.     

Effectiveness of an umbilical artery "snare assisted" approach for critical pulmonary valve stenosis or atresia in the neonate

Thirteen neonates with critical pulmonary valve stenosis/atresia underwent successful transcatheter balloon valvuloplasty using an umbilical artery "snare assisted" approach. This technique simplifies the procedure and avoids femoral artery injury by using the umbilical artery, reduces fluoroscopy exposure, and eliminates the need for a gradational approach which reduces costs.     

Non-neoplastic stenosis of the aqueduct presenting in adolescence and adult life

Twenty-six cases of non-neoplastic stenosis of the aqueduct are reviewed. This condition may take a very chronic course and may not be revealed until decompensation of hydrocephalus takes place. Poor athletic record in the school or intellectual impairment in early life, commonly overlooked, can presage the more obvious symptoms. A prolonged follow-up of these cases is essential to exclude a slowly growing neoplasm near the aqueduct. Our management and the outcome of these cases is discussed.     

Learning and understanding the Kruskal-Wallis one-way analysis-of-variance-by-ranks test for differences among three or more independent groups

When several treatment methods are available for the same problem, many clinicians are faced with the task of deciding which treatment to use. Many clinicians may have conducted informal "mini-experiments" on their own to determine which treatment is best suited for the problem. These results are usually not documented or reported in a formal manner because many clinicians feel that they are "statistically challenged." Another reason may be because clinicians do not feel they have controlled enough test conditions to warrant analysis. In this update, a statistic is described that does not involve complicated statistical assumptions, making it a simple and easy-to-use statistical method. This update examines the use of two statistics and does not deal with other issues that could affect clinical research such as issues affecting credibility. For readers who want a more in-depth examination of this topic, references have been provided. The Kruskal-Wallis one-way analysis-of-variance-by-ranks test (or H test) is used to determine whether three or more independent groups are the same or different on some variable of interest when an ordinal level of data or an interval or ratio level of data is available. A hypothetical example will be presented to explain when and how to use this statistic, how to interpret results using the statistic, the advantages and disadvantages of the statistic, and what to look for in a written report. This hypothetical example will involve the use of ratio data to demonstrate how to choose between using the nonparametric H test and the more powerful parametric F test.     

Metastatic choriocarcinoma presenting as multiple intracerebral haemorrhages: the role of imaging in the elucidation of the pathology

Eighteen-year-old adolescents with alpha1-antitrypsin (alpha1AT) deficiency have mostly normal lung function tests. We hypothesized that compensatory increases in other protease inhibitors and/or a decreased leukocyte activity might favorably affect the protease/protease-inhibitor balance in alpha1AT-deficient adolescents. At the age of 18 y 46 PiZZ (severe deficiency), 22 PiSZ (moderate deficiency), and 41 control subjects were studied. The plasma protease inhibitors alpha2-macroglobulin (alpha2M), alpha1-antichymotrypsin (Achy), and secretory leukocyte protease inhibitor (SLPI) were studied, and the protease elastase complexed with alpha1AT (HEAT) and neutrophil gelatinase-associated lipocalin (NGAL) as indicators of neutrophil leukocyte activity. Significantly higher concentrations of alpha2M were found in PiZ (p < 0.0001) and PiSZ (p < 0.0001) individuals compared with control subjects. The PiZZ and SZ adolescents had low levels of NGAL (p < 0.0001). Low levels of HEAT were found in PiZZ subjects (p < 0.0005). Higher concentrations of Achy were found in PiZZ (p < 0.04) and PiSZ (p < 0.05) individuals. Increased concentrations of alpha2M and Achy combined with decreased levels of HEAT and NGAL, indicating decreased leukocyte activity may, to some extent, compensate for the protease/protease inhibitor imbalance in the alpha1AT-deficiency state.     

Combined esophageal atresia and duodenal stenosis in polyhydramnios. Ante-, peri- and postpartum management]

Three-dimensional sonography of the mamma with a Voluson annular array transducer (10 MHz) (Kretztechnik, Austria) is a new method applicable in differential diagnosis of mamma foci. 50 patients (19 of them with breast cancer) were thus pre-surgically examined. Both sectional and stereoscopic representations were made use of. The suspected diagnoses and their correspondences with post-surgical findings were compared to the correspondences obtained through 2D-sonography. 3D-sonography produced 4 cases of the incorrectly positive diagnosis breast cancer, 2D-sonography 2 cases of incorrectly negative diagnoses. Other advantages of 3D-sonography over 2D-sonography include: better judgement of the conditional of focal environs (infiltration), existence and form of intracystic structures and of multifocal disease, short duration of examination, possible re-diagnosing of stored data.     

Systemic collateral and pulmonary artery stenosis in patients with congenital pulmonary valve atresia and ventricular septal defect

Angiograms of 30 patients with congenital pulmonary valve atresia, ventricular septal defect, and large systemic-pulmonary collateral arteries (SPCAs) were evaluated. All had aortography, 28 had SPCA arteriography, and 26 had right ventriculography. Seventeen (65%) of 26 patients had a right ventricular infundibulum, 23 (77%) had a pulmonary artery confluence, and five of the nine patients without a right ventricular infundibulum had a confluence. Sixty-six SPCAs of aortic origin were seen; 28 (42%) had narrowing and 21 patients (70%) had one or more narrowed SPCAs. Five patients had collaterals from internal mammary, subclavian or innominate arteries. Fourteen (47%) had hilar pulmonary artery stenosis. Of these 14 patients mild peripheral stenosis was demonstrated in five. Right aortic arch was present in 15 patients (50%). Complete angiographic delineation of pulmonary vasculature is an essential procedure for preoperative detection of pulmonary and SPCA stenoses in these patients.     

Vaginal lever pessary in patients with multiple gestation, preterm labor and low fetal station. A report of three cases

BACKGROUND: The vaginal pessary has been utilized previously in patients with cervical incompetence, resulting in improvement in pregnancy outcome. The mechanical advantages generated by the vaginal lever pessary could theoretically be applied to patients in preterm labor with low station of the presenting part to prevent cervical dilatation. CASES: One patient with twins and two with triplet pregnancies presented in preterm labor with advanced cervical dilatation and low fetal station. They were treated with tocolytic drugs, and a vaginal lever pessary was placed. The gestational age at delivery was delayed with the addition of the vaginal pessary. Based on previous experience with these difficult cases, it was judged that the use of the pessary achieved a delay in delivery that would not have occurred without the device. CONCLUSION: Vaginal pessaries can be used as mechanical adjuvants in the treatment of preterm labor. These devices work by altering the pressure dynamics on the cervix and lower uterine segment. They may also help limit cervical change by preventing engagement of the presenting part into the maternal pelvis. In these cases, a vaginal pessary appears to be beneficial in delaying delivery in multiple gestations presenting with advanced cervical dilatation and low station of the presenting part.     

Abnormalities of neuropeptides and neural markers in the esophagus of fetal rats with adriamycin-induced esophageal atresia

BACKGROUND/PURPOSE: To investigate the distribution of neural markers and neuropeptides in esophageal atresia (EA). METHODS: A fetal rat model with Adriamycin-induced EA was used. The animals were divided into four groups: (1) control group, (2) saline-injected group, (3) Adriamycin administered but without the development of EA, and (4) Adriamycin-induced EA group. Specimens of the distal esophagus from each group were immunostained using antibodies to S100, protein gene product 9.5 (PGP), somatostatin, vasoactive intestine peptide (VIP), bombesin, galanin, substance P, neuropeptide Y (NPY), calcitonin gene-related product (CGRP), met-encephalin, nitric oxide synthase, and tyrosine hydroxylase. RESULTS: The total cross-sectional area of the distal atretic esophagus was significantly smaller than controls (P = .01), the submucosa being the component most affected (0.0465 v 0.0234 mm). Immunoreactivity for S100 and galanin were significantly elevated in the atresia group (0.0288 v 0.0079 and .001 v 0.000). In addition, there was also an increase in CGRP and Substance P in the atretic group. CONCLUSION: The elevated levels of S100 and galanin could explain the disordered motility observed in patients who had esophageal atresia.     

Concurrence of supravalvular aortic stenosis and peripheral pulmonary stenosis in three generations of a family: a form of arterial dysplasia

Isolated supravalvular aortic stenosis (SVAS) commonly is an autosomal dominant trait; it may also occur in the Williams syndrome (WS). While peripheral pulmonary stenosis (PPS) can occur in the same individual with familial isolated SVAS, concurrence of these lesions in different relatives of a family is uncommon. We describe five affected individuals in one family; three had isolated SVAS, one had isolated PPS, and one had SVAS and PPS. Based on this family and review of literature, we suggest that SVAS is a form of arterial dysplasia encompassing PPS in its spectrum. It is developmentally distinct from other left heart obstructive lesions that are hypothesized to be related to blood flow abnormalities in the developing embryo. We also conclude that the clinical disorder in this family represents one that is distinct from WS.     

Coexistence of papillary and medullary carcinoma of the thyroid gland-mixed or collision tumour? Clinicopathological analysis of three cases

We present three thyroid carcinomas displaying medullary and papillary components. In two cases the papillary component was characterized by typical papillae with a fibrovascular core; in one a follicular variant of papillary carcinoma was found. The papillary component was dominant in two and the medullary in one case. One tumour showed clear-cut borders between the two components, the others displayed an intermingled pattern. Both tumour components were seen in lymph node metastases with immunostaining with antibodies to calcitonin, chromogranin A, carcinoembryonic antigen, other neuroendocrine markers and thyroglobulin. At least two of our cases are true mixed carcinomas probably arising from a common stem cell.