Rings, slings, and other things: vascular compression of the infant trachea updated from the midcentury to the millennium--the legacy of Robert E. Gross, MD, and Edward B. D. Neuhauser, MD.

In the first half of the 20th century, pediatric chest imaging was limited mainly to the performance of conventional radiography, including barium esophagography and occasionally bronchography and angiography. Despite this limited imaging approach, by 1950 the diagnosis and treatment of vascular "rings" compressing infant airways had been accomplished with the pioneering efforts of Robert E. Gross, MD, in the field of surgery, and Edward B. D. Neuhauser, MD, in the field of radiology. The next two decades brought the recognition of pulmonary arterial "sling," or anomalous left pulmonary artery, in diagnosis and treatment. Recognition of still another vascular compressive syndrome in infants was identified as that due to the absence of the pulmonary valve. These "rings, slings, and other things" are now evaluated with magnetic resonance (MR) imaging, including MR angiography, and computed tomography (CT), including CT angiography, with the added use of three-dimensional reconstruction. These are the legacies of Drs Gross and Neuhauser.

Imaging features of Mycobacterium avium-intracellulare complex (MAC) in children with AIDS.

PURPOSE: The purpose of this paper was to review the imaging features of Mycobacterium avium-intracellulare complex (MAC) in 16 pediatric patients with human immunodeficiency virus (HIV). MATERIALS AND METHODS: We reviewed the pertinent clinical records of 16 children diagnosed with MAC between January 1990 and June 1998. These 16 cases were blood- or biopsy-proven to have MAC infection. Their plain films, abdominal, and chest CT scans were then reviewed and the findings were analyzed with reference to the few reported cases of children with MAC. RESULTS: Abdominal findings: all but one had retroperitoneal adenopathy, mesenteric adenopathy or both. Ten patients had hepatomegaly, while nine patients were found to have splenomegaly. Four patients had nonspecific thickened gallbladder wall, while intestinal wall thickening and thickened stomach folds were identified in six of ten patients. Necrotic, fluid-filled nodes were also found. Chest findings included mediastinal adenopathy, cystic/cavitary lesions and bronchiectasis. One patient developed a fistula between the mediastinal lymph nodes, esophagus, and bronchial tree. CONCLUSION: Pediatric patients with HIV who develop MAC infection may present with massive lymph node enlargement. This can occur not only in mesenteric and retroperitoneal nodes but also in hilar and posterior mediastinal nodes as well. As in MTB infection, these nodes can break down with development of fistulous tracts to both esophagus and adjacent lung. The major differential diagnostic consideration besides MTB is lymphoma.

Hepatic pulmonary fusion in neonates.

OBJECTIVE: This report discusses the relationship of supradiaphragmatic hepatic tissue that is fused to the lung (through a diaphragmatic defect) with pulmonary hypoplasia-a new constellation of findings. CONCLUSION: Hepatic pulmonary fusion should be suspected in instances of apparent diaphragmatic hernia characterized by mediastinal shift towards the hypoplastic lung or when the mediastinum does not shift away from the mass.

CT imaging of splenic sequestration in sickle cell disease.

Pooling of blood in the spleen is a frequent occurrence in children with sickle cell diseases, particularly in the first few years of life, resulting in what is termed "splenic sequestration crisis." The spectrum of severity in this syndrome is wide, ranging from mild splenomegaly to massive enlargement, circulatory collapse, and even death. The diagnosis is usually clinical, based on the enlargement of the spleen with a drop in hemoglobin level by > 2 g/dl, and it is rare that imaging studies are ordered. However, in the patient who presents to the emergency department with non-specific findings of an acute abdomen, it is important to recognize the appearance of sequestration on imaging studies. We studied seven patients utilizing contrast-enhanced CT scans and found two distinct patterns--multiple, peripheral, non-enhancing low-density areas or large, diffuse areas of low density in the majority of the splenic tissue. Although radiological imaging is not always necessary to diagnose splenic sequestration, in those situations where this diagnosis is not immediately obvious, it makes an important clarifying contribution.

Punctate thymic calcification in infants with untreated Langerhans' cell histiocytosis: report of four new cases.

Four new cases of punctate thymic calcification in infants with untreated Langerhans' cell histiocytosis (LCH) are added to the four previously reported cases. All cases were shown on CT scans; plain films were rarely diagnostic. Pathologic correlation remains elusive since the usual biopsies of the LCH have been on skin or bone biopsies. A single prior pathologic study of the thymus in untreated LCH showed microscopic calcospherites. The thymic punctate calcific densities in patients with LCH may represent further accretion so that the calcospherites become macroscopic. The finding of such punctate calcific densities in an enlarged thymus of an infant with skin or bone or lung disease is strongly suggestive of LCH.

Neuroblastoma arising from the organ of Zuckerkandl: an unusual site with a favorable biologic outcome.

BACKGROUND: Prognosis in neuroblastoma has been shown to correlate with age and stage at diagnosis and site of origin. Extra-abdominal tumors (chest, neck, pelvis) do better in terms of survival than tumors arising from the upper abdomen. OBJECTIVE: We evaluated a subgroup of abdominal neuroblastomas arising near to the aortic bifurcation (commonly called organ of Zuckerkandl, O. Z.) to assess their biologic outcome and problems in diagnosis and therapy. MATERIALS AND METHODS: Sixteen O. Z. primary tumors were seen at three children's hospitals. Their clinical records and imaging studies were reviewed, including the sonographic, CT, and MRI findings. When available, MYCN amplification was noted (MYCN is the current term previously called N-MYC). RESULTS: Despite more than half of the tumors being very large, survival was the rule, with only one fatality (following multiple local recurrences). Only one patient (who survived) had bone metastases. The larger masses were usually palpated in otherwise well children, while the smaller ones were found in the course of evaluation for unrelated problems such as urinary tract infection. Intraspinal extension was common, though usually asymptomatic. MYCN amplification was absent in the four patients studied. CONCLUSIONS: Lower abdominal (O. Z.) neuroblastomas present technical problems of surgical removal, but form a group with a favorable outcome similar to cervical and thoracic primary sites. MRI was useful in delineating intraspinal extension.

Malignancies in pediatric patients with ataxia telangiectasia.

BACKGROUND: Patients with ataxia telangiectasia (AT), known to have an inherent increased susceptibility to the development of cancer, may present with malignancies that are unusual for the patient's age, are often difficult to diagnose clinically and radiographically and respond poorly to conventional therapy. MATERIALS AND METHODS: We reviewed the clinical presentation and imaging studies of 12 AT patients who developed malignancies. RESULTS: Eight of the twelve patients developed non-Hodgkin's lymphoma (CNS, thorax, bone), two developed Hodgkin's disease, and two were diagnosed with gastrointestinal mucinous adenocarcinoma. CONCLUSION: The lymphomas were commonly extra nodal, and infiltrative rather than mass-like. The recognition of the tumors was often delayed due to confusion with the known infectious complications in AT patients.

Tracheobronchial anomalies in children with congenital cardiac disease.

OBJECTIVE: To perform preoperative airway evaluations, using radiographic analysis, to review the tracheal anatomy in children with congenital cardiac disease. DESIGN: Prospective. SETTING: A university children's hospital. PARTICIPANTS: One hundred patients. MEASUREMENTS AND MAIN RESULTS: One magnified airway film (high kilovoltage filtered) was performed preoperatively on 100 consecutive children presenting for repair of congenital cardiac disease. Events at intubation, with respect to endotracheal tube size (internal diameter in millimeters) and difficulties with placement of the tube, were recorded. Postoperative morbidity, specifically related to underlying airway anomaly, was documented. Eleven children had positive radiographic findings after review of magnified airway films. Six of 11 patients had evidence of tracheobronchial pathology, and five patients had no tracheal pathology. Difficulties with intubation were noted in two children. No perioperative morbidity was noted in any patient. CONCLUSION: The use of preoperative magnified airway films for tracheal evaluations in children with cardiac disease should be considered.

Spontaneous correction of the malpositioned percutaneous central venous line in infants.

Malpositioning of the percutaneously placed central venous line (PCVL) or percutaneously inserted central catheter (PICC) in infants is not a rare occurrence. It has been occasionally observed that these lines spontaneously correct themselves. This prospective study was done to study the incidence of malposition and spontaneous correction. Using a modification of the standard method, 187 catheters were placed with 98.9 % success. Seven of these were initially malpositioned. All seven corrected themselves within a day when left in and used as a peripheral intravenous line. In many centers malpositioned catheters are taken out and replaced, which imposes great stress on the critically ill infant. Our study suggests that to avoid this stress the catheter should be left in place, since spontaneous correction may occur.

Ureteral obstruction secondary to indinavir in the pediatric HIV population.

Indinavir sulfate is a protease inhibitor used in the treatment of the human immunodeficiency virus (HIV). This case report describes the radiographic and urologic manifestations of indinavir urolithiasis in two pediatric patients with acquired immunodeficiency syndrome (AIDS). Management involves aggressive hydration and surgical intervention when indicated.