Testicular-sparing surgery for benign testicular tumors.

BACKGROUND/PURPOSE: Although there has been a precedent of testicular-sparing surgery in some centers, the authors find it is still not general practice among pediatric surgeons. To address this and emphasize the role of testicular-sparing surgery in children, four patients with testicular masses are presented who underwent this procedure. METHODS: Four patients who underwent testicular-sparing surgery between the years 1993 and 1998 were reviewed. Demographic data, histopathology, and follow-up data were obtained from office charts. The period of follow-up ranged from 1 to 5 years. RESULTS: Four patients whose ages at diagnosis were 1, 2, 4, and 17 years presented with unilateral testicular masses. The alpha-fetoprotein and beta-human chorionic gonadotropin levels were within normal limits. Testicular ultrasonography was carried out on all patients, and groin exploration with spermatic cord isolation was performed in each case. After enucleation, frozen sections to confirm benignity was carried out before repair of the testis. Follow-up of 6 months to 5 years has shown no recurrence, and on examination, testicular volume is normal in all cases. CONCLUSIONS: Testicular-sparing surgery preserves testicular volume, which is important for both cosmetic and functional purposes. It is a viable and useful method in the management of benign testicular tumors in children.

High-dose induction chemoradiotherapy followed by autologous bone marrow transplantation as consolidation therapy in rhabdomyosarcoma, extraosseous Ewing's sarcoma, and undifferentiated sarcoma.

PURPOSE: To improve response and survival rates in patients with high-risk rhabdomyosarcoma (RMS), extraosseous Ewing's sarcoma, and undifferentiated sarcoma, we used a short course of induction with multi-agent chemotherapy, hyperfractionated radiotherapy, and surgery when possible. Consolidation was with intensive chemotherapy and autologous bone marrow transplantation (ABMT). PATIENTS AND METHODS: Twenty-six patients (21 with RMS, three with undifferentiated sarcoma, and two with extraosseous Ewing's sarcoma) were entered onto the protocol between June 1990 and March 1994. Induction consisted of ifosfamide, etoposide, doxorubicin, dactinomycin, cyclophosphomide, and vincristine, and a split course of hyperfractionated radiotherapy. Patients who attained a complete response (CR) or good partial response (GPR) received consolidation with high-dose melphalan and etoposide followed by ABMT. RESULTS: Of 26 previously untreated patients 19 (73%) achieved a CR (n=13) or GPR (n=6) at the completion of induction and underwent ABMT. Two-year overall survival (OS) was 56% (95% confidence interval [CI], 36% to 76%) and progression-free survival (PFS) was 53% for the whole group (95% CI, 33% to 73%). CONCLUSION: Consolidation of response by myeloablative chemotherapy was well tolerated. Split-course hyperfractionated radiotherapy did not increase the rate of local control. The results of this short-course therapy were comparable to previous therapies of 1 to 2 years' duration. Induction and consolidation chemotherapy, as well as radiation dose, could be further intensified, since no death due to toxicity occurred among these patients.

Adrenal neoplasms in children.

Neuroblastoma is the most common extracranial neoplasm of childhood. Although it most commonly occurs in the adrenal gland, it may be found anywhere along the sympathetic chain. The characteristic clinical and imaging features of neuroblastoma are discussed in this article. Less common neoplasms of the adrenal gland, adenoma, adrenocortical carcinoma, and pheochromocytoma are also discussed.

Splenic lymphangiomatosis in children.

PURPOSE: To study the clinical and imaging features of splenic lymphangiomatosis. MATERIALS AND METHODS: The clinical and abdominal imaging data of 10 children with splenic lymphangiomatosis were retrospectively reviewed. The modalities used in the study included computed tomography (CT) (10 patients), sonography (five patients), and magnetic resonance (MR) imaging (two patients). Pathologic confirmation of lymphangiomatosis was obtained in nine patients. RESULTS: Splenic lymphangiomatosis was discovered incidentally in all cases and was a key finding in enabling the correct diagnosis in six children with extrasplenic disease as well. Only two children had clinical splenomegaly. Sonograms and MR images showed multiple, wEll-defined cysts. Multiple, low-attenuation lesions that did not enhance with intravenous administration of contrast material (n = 8) or a mottled spleen (n = 2) were seen at CT. One of the mottled spleens had target lesions on an early (arterial) image obtained after administration of a bolus of contrast material. One child underwent a splenectomy; one child underwent therapeutic embolization. Eight patients remained asymptomatic with respect to the spleen 1-20 years later. CONCLUSION: Splenic lymphangiomatosis is often an incidental imaging finding that frequently has a characteristic imaging appearance. The recognition of this appearance helps in diagnosis of this disease and may prevent the need for further invasive procedures. Splenic changes can be isolated or can coexist with bone or soft-tissue lymphangiomas.

The location and appearance of second malignancies in patients with bilateral retinoblastoma.

Purpose. This paper describes the clinical history and radiographic appearance of second malignancies in patients with bilateral retinoblastoma.Subjects/methods. The imaging studies and clinical data of 14 patients with a history of bilateral retinoblastoma who were treated for second malignancies were reviewed.Results. A total of 17 tumors were identified in 14 patients during the period 1978-1996. The median age of occurrence of the second malignancy was 17 years (range 10-32 years). Fourteen of the 17 malignancies occurred in the facial structures and three developed in the lower extremities. The histologies included osteosarcoma (n = 5), malignant fibrous histiocytoma (n = 3), high-grade spindle cell sarcoma (n = 3), malignant mesenchymoma (n = 1), leiomyosarcoma (n = 4) and angiosarcoma (n = 1). The tumors were locally aggressive and had a similar appearance to those found in nonretinoblastoma patients. Six of the 14 patients are alive and disease free.Discussion. Most of the adolescent and young adult retinoblastoma survivors developed second malignancies in the irradiated facial structures but some occurred in distal sites. Radiologically, these tumors do not differ in appearance from those seen in non-retinoblastoma patients with the exception of their location.

Stage IVN neuroblastoma: MRI diagnosis of left supraclavicular "Virchow's" nodal spread.

Stage IV neuroblastoma is associated with high mortality; an exception are patients whose stage IV status includes distant positive nodes, but no skeletal metastases-stage IVN neuroblastoma. We describe our experience with preoperative MRI in three patients with extensive abdominal neuroblastoma without cortical bony involvement but with unsuspected metastatic involvement to the left supraclavicular (Virchow's) node. We review findings of left supraclavicular nodal spread in five earlier cases of IVN neuroblastoma.

Neuroimaging of brain injury in neonates treated with extracorporeal membrane oxygenation: lessons learned from serial examinations.

The head ultrasound (US) is used extensively at most extracorporeal membrane oxygenation (ECMO) centers to screen for intracranial pathology in the ECMO candidate. Daily head US examinations are obtained in patients on ECMO to detect the emergence of intracranial hemorrhage (ICH). The authors asked whether these serial studies could be correlated with more definitive diagnostic studies, such as computed tomography (CT) and magnetic resonance imaging (MRI) scans, autopsy data, or the long-term neurodevelopmental status, to discern the predictive value of these daily examinations. Seventy-four consecutively treated neonates with reversible respiratory failure refractory to conventional support met institutional criteria for placement on ECMO. In addition to a pre-ECMO US, daily real-time portable head US images were evaluated for changes in echotexture, ventricular configuration, and extraaxial fluid. Follow-up CT and MRI scans were evaluated for the presence of hemorrhagic or ischemic lesions. Autopsy data were obtained from nonsurvivors. Survivors were examined by a neurodevelopmental specialist at regular intervals and classified as normal or delayed for chronological age. In this series of 74 patients, CT/MRI scanning and autopsy data demonstrated structural injury in 19 patients; there were 16 ischemic infarctions and three hemorrhages. The incidence of hemorrhage in this series was considerably lower than that previously reported. Ten of the 19 patients had serial head US findings demonstrating a progression from focal increases in echotexture to diffuse effacement of cerebral architecture. In the remaining nine, serial head US examinations did not show injury. An additional 10 children had a clear delay in neurological development despite no evidence of anatomic injury on serial head US examinations or CT/MRI scanning.(ABSTRACT TRUNCATED AT 250 WORDS)

Pediatric HIV infection in its second decade--the changing pattern of lung involvement. Clinical, plain film, and computed tomographic findings.

The changing pattern of pediatric HIV infection is illustrated in this article with plain films and computed tomographic scans. Today, vertical transmission from infected mothers results in HIV infection of exposed infants in about one third of pregnancies. Although the high mortality with catastrophic illnesses such as Pneumocystis carinii pneumonia seen in early life appears to be decreasing, chronic illness with pulmonary involvement due to diffuse lymphocytic infiltrative syndromes continues. Lymphocytic interstitial pneumonitis (LIP) has evolved in some patients to cystic lung disease and bronchiectasis. There are increasing numbers of patients infected with Mycobacterium. Masses seemingly of smooth muscle origin, thought to be leiomyosarcoma are appearing; they may be pseudotumors related to concomitant Mycobacterium avium intracellulare infection.

ACTH induced adrenal enlargement in infants treated for infantile spasms and acute cerebellar encephalopathy.

ACTH is used in childhood to treat infantile spasms and acute cerebellar encephalopathy (opsoclonus-cerebellar ataxia syndrome). We report two infants with ultrasonographic and CT evidence of massive, reversible adrenal enlargement secondary to exogenous ACTH therapy for these disorders.

Cervical neuroblastoma in eleven infants--a tumor with favorable prognosis. Clinical and radiologic (US, CT, MRI) findings.

Cervical neuroblastoma, a disease primarily of infants, has a favorable prognosis. Eleven patients are reported. Clinical presentations (other than mass) included stridor and swallowing problems. Masses when felt were commonly mistaken for infectious adenitis. Imaging studies (US, CT, MRI) showed solid masses with vascular displacement and narrowing; intraspinal extension was absent though extension into the adjacent sites of mediastinum and skull occurred. Horner syndrome was seen in five patients with accompanying heterochromia iridis in one. Five tumors had calcification. A high index of suspicion will lead to biopsy and less delay in diagnosis once a mass is felt or imaged.

The diagnosis of abdominal neuroblastoma: relative roles of ultrasonography, CT, and MRI.

Abdominal neuroblastoma is reviewed in terms of its diagnosis, including in utero, at birth, and through infancy into childhood. Age at diagnosis remains the best predictor of survival, with infants under 1 year of age having almost 100% cure. Ultrasonography and magnetic resonance imaging (MRI) are the recommended diagnostic modalities to stage the abdominal primary, although computed tomography (CT) (including myelography) is still widely and efficiently used. Examples are given of each stage.

Acquired coarctation of the aorta.

Coarctation of the aorta is usually caused by a congenital narrowing of the aorta. This report describes two children who developed hypertension secondary to an acquired coarctation of the aorta. In one patient the coarctation was temporally related to umbilical artery catheterization and was associated with thrombosis and aneurysmal dilatation of the aorta. In the second patient, the coarctation occurred after surgical aortotomy during the removal of an intrathoracic neuroblastoma. Patients who have interventional damage to the aorta should be periodically examined for the appearance of a coarctation. Although an acquired coarctation of the aorta is an infrequent complication of invasive or surgical procedures, it should be identified since it represents a remediable cause of hypertension in children.

Benign gaseous distension of the bowel in premature infants treated with nasal continuous airway pressure: a study of contributing factors.

Continuous positive airway pressure (CPAP) administered as a mixture of oxygen and compressed air via nasal prongs has dramatically improved survival rates and lessened the frequency of barotrauma and bronchopulmonary dysplasia in the premature infant with respiratory distress syndrome. Associated with the increased use of nasal CPAP has been the development of marked bowel distension (CPAP belly syndrome), which occurs as the infant's respiratory status improves and the baby becomes more vigorous. To identify contributing factors, we prospectively compared 25 premature infants treated with nasal CPAP with 29 premature infants not treated with nasal CPAP. Infants were followed up for development of distension, defined clinically as bulging flanks, increased abdominal girth, and visibly dilated intestinal loops. We evaluated birth weight, weight at time of distension, method of feeding (oral, orogastric tube), and treatment with nasal CPAP and correlated these factors with radiologic findings. Of the infants who received nasal CPAP therapy, gaseous bowel distension developed in 83% (10/12) of infants weighing less than 1000 g, but in only 14% (2/14) of those weighing at least 1000 g. Only 10% (3/29) of infants not treated with nasal CPAP had distension, and all three weighed less than 1000 g. Presence of sepsis and method of feeding did not correlate with occurrence of distension. Neither necrotizing enterocolitis nor bowel obstruction developed in any of the patients with a diagnosis of CPAP belly syndrome. Our study shows that nasal CPAP, aerophagia, and immaturity of bowel motility in very small infants were the major contributors to the development of benign gaseous bowel distension.

Real time ultrasonographic evaluation of the infant hip.

Although the technique for ultrasonographic evaluation of the infant hip is difficult to learn, it is a valuable diagnostic tool. In many institutions, hip ultrasonography expands upon and complements the clinical examination and minimizes radiographs.

MR imaging of diffuse bone marrow replacement in pediatric patients with cancer.

In the magnetic resonance (MR) imaging examinations of three children with tumors (two neuroblastoma, one rhabdomyosarcoma) and three with leukemia, the marrow demonstrated a diffuse, uniform pattern of hypointensity on T1-weighted images and hyperintensity on T2-weighted images. The authors observed that this reversal ("flip-flop") of the usual MR characteristics of fatty marrow was seen in the epiphyses, metaphyses, and diaphyses. The purpose of this study was to establish the radiographic and clinicopathologic correlates of this MR finding on the basis of findings from plain radiographs, bone scans, and bone marrow aspirates. Plain radiographs and bone scans demonstrated either normal findings or changes limited to the metaphyses. In all patients, analysis of bone marrow aspirates demonstrated metastases. The authors concluded that even in the absence of evidence of discrete bone metastases on a plain radiograph or a bone scan, this diffuse and uniform "flip-flop" pattern reflects diffuse marrow replacement by tumor cells.

Colonic diverticulitis in young patients with chronic renal failure and transplantation.

The association of colonic diverticulitis with chronic renal failure is well known. In those patients with "adult" autosomal dominant polycystic kidney disease, colonic diverticulitis is an especially common complication. We present two young patients (one teenager and one mid-twenties) who developed intra-abdominal abscess several years after renal transplantation. Neither patient had autosomal dominant polycystic disease nor a known history of gastrointestinal problems but both proved to have underlying, previously unsuspected colonic diverticular disease with abscess formation.

Salt losing nephropathy simulating congenital adrenal hyperplasia in infants with obstructive uropathy and/or vesicoureteral reflux--value of ultrasonography in diagnosis.

Salt losing nephropathy, occurring predominantly in male infants, has been reported in association with a spectrum of urologic diseases including obstructive uropathy and massive, infected vesicoureteral reflux (VUR). This has been called pseudo-hypoaldosteronism (PHA) or alternatively, pseudo salt-losing congenital adrenal hyperplasia (CAH), and is thought to reflect a tubular unresponsiveness to aldosterone. We report our experience with six cases, discuss one case in detail and review the 39 cases previously reported. A one month old male infant presented with a left upper quadrant mass. Signs and symptoms included vomiting, dehydration, hyponatremia and hyperkalemia. This suggested the diagnosis of CAH for which therapy was instituted. Ultrasonographic examination subsequently revealed the mass to be a urinoma in an infant with posterior urethral valve (PUV) and obstructive hydronephrosis.

Posterior mediastinal cystic teratoma surrounded by fat in a 13 month old boy. Value of magnetic resonance imaging.

A 13 month old boy had a large left sided posterior cystic mass initially thought to be in lung. MRI showed the cystic mass to be surrounded by fat. A benign mediastinal teratoma was removed.

Esophageal dilatation and reflux in neonates supported by ECMO after diaphragmatic hernia repair.

Extracorporeal membrane oxygenation (ECMO) for survivors of surgical repair of congenital diaphragmatic hernia was used in 14 neonates. All showed marked esophageal dilatation on postoperative chest radiographs; the dilated esophagus simulated an air- or fluid-filled mass. All patients were later shown to have marked gastroesophageal reflux as well. The finding suggests a problem in swallowing exists before birth, which may explain the recent correlation between diaphragmatic hernia and hydramnios. The observation of a mediastinal mass in neonates with congenital diaphragmatic hernia may represent esophageal ectasia and may be related to significant gastroesophageal reflux.