Primary pulmonary lymphangiectasia in infancy and childhood.

Primary pulmonary lymphangiectasia (PPL) is a rare disorder of unknown aetiology characterised by dilatation of the pulmonary lymphatics. PPL is widely reported to have a poor prognosis in the neonatal period and little is known about the clinical features of patients who survive the newborn period. The current authors report the outcome in nine patients diagnosed in infancy with PPL over a 15-yr period at a single university-based hospital clinic and followed for a median of 6 yrs. Although all of the patients initially experienced respiratory distress, respiratory symptoms improved in most patients after infancy and were notably better by the age of 6 yrs. Many patients had poor weight gain in the first years of life, which eventually improved. Radiological scans showed progressive resolution of neonatal infiltrates, but were characterised by hyperinflation and increased interstitial markings in older children. Most patients had evidence of bronchitis and grew pathogenic organisms from quantitative bronchoalveolar lavage culture. Pulmonary function tests showed predominantly obstructive disease that did not deteriorate over time. In conclusion, these results suggest that primary pulmonary lymphangiectasia does not have as dismal a prognosis as previously described and symptoms and clinical findings improve after the first year of life.

Airway surface liquid recovered by lavage with perfluorocarbon liquid in cats.

BACKGROUND: Airway surface liquid (ASL) is difficult to sample. Lavage with an immiscible perfluorocarbon (PFC) liquid to recover ASL was evaluated in cats. MATERIALS AND METHODS: Six wild-type cats underwent bronchoscopic lavage with a PFC (perfluorohexane), with the bronchoscope wedged in the feline equivalent of the right lower lobe. Two cats (control animals) were lavaged with a saline vehicle only. Four procedures were performed on each animal at 2-3-week intervals. Ionic composition of ASL was determined by flame photometry. RESULTS: Cats lavaged with PFC showed significantly more acute respiratory distress than those lavaged with saline (respiratory rate following procedure: PFC, 47 +/- 5 min-1 vs. saline, 27 +/- 2 min-1, P < 0.05; O2 saturation: PFC 80 +/- 1% vs. saline, 91 +/- 1%, P < 0.01). The PFC group also had clinical evidence of chronic respiratory compromise (mean respiratory rate before next anaesthetic; PFC, 37 +/- 2 min-1 vs. saline, 20 +/- 3 min-1, P < 0.01). The PFC-lavaged lungs demonstrated persistent radiographic changes and histological evidence of small airways obstruction with distal alveolar damage. Six PFC lavages yielded ASL samples (> 100 microL) which were sufficient for analysis. Mean (+/- SEM) ionic concentrations in these samples were Na+ 157.4 +/- 14.5 mmol L-1, Cl- 150.5 +/- 16.8 mmol L-1 and K+ 10.1 +/- 1.7 mmol L-1. CONCLUSIONS: Perfluorocarbon lavage can be used to collect unmodified ASL from the distal lung. However, repeated lavage with perfluorohexane was associated with significant pathological changes in this study.

Canines as sentinel species for assessing chronic exposures to air pollutants: part 1. Respiratory pathology.

A complex mixture of air pollutants is present in the ambient air in urban areas. People, animals, and vegetation are chronically and sequentially exposed to outdoor pollutants. The objective of this first of 2 studies is to evaluate by light and electron microscopy the lungs of Mexico City dogs and compare the results to those of 3 less polluted cities in MEXICO: One hundred fifty-two clinically healthy stray mongrel dogs (91 males/61 females), including 43 dogs from 3 less polluted cities, and 109 from southwest and northeast metropolitian Mexico City (SWMMC, NEMMC) were studied. Lungs of dogs living in Mexico City and Cuernavaca exhibited patchy chronic mononuclear cell infiltrates along with macrophages loaded with particulate matter (PM) surrounding the bronchiolar walls and extending into adjacent vascular structures; bronchiolar epithelial and smooth muscle hyperplasia, peribronchiolar fibrosis, microthrombi, and capillary and venule polymorphonuclear leukocytes (PMN) margination. Ultrafine PM was seen in alveolar type I and II cells, endothelial cells, interstitial macrophages (Mtheta), and intravascular Mtheta-like cells. Bronchoalveolar lavage showed significant numbers of alveolar macrophages undergoing proliferation. Exposure to complex mixtures of pollutants-predominantly particulate matter and ozone-is causing lung structural changes induced by the sustained inflammatory process and resulting in airway and vascular remodeling and altered repair. Cytokines released from both, circulating inflammatory and resident lung cells in response to endothelial and epithelial injury may be playing a role in the pathology described here. Deep concern exists for the potential of an increasing rise in lung diseases in child populations exposed to Mexico City's environment.

Exposure to air pollution is associated with lung hyperinflation in healthy children and adolescents in Southwest Mexico City: a pilot study.

Air pollution produces adverse health effects. The consequences of lifelong daily exposures to atmospheric pollutants upon the respiratory apparatus of healthy children are of considerable clinical importance. We investigated the association between exposure to a highly polluted urban environment with a complex mixture of air pollutants-ozone and particulate matter the predominant ones-and chest x-ray abnormalities in 59 healthy Mexican children who are lifelong residents of Southwest Metropolitan Mexico City (SWMMC), with a negative history of tobacco exposure and respiratory illnesses. Their clinical results and x-ray findings were compared to those of 19 Mexican control children, residents of a low-pollution area, with a similar negative history of tobacco exposure and respiratory illnesses. Ozone concentrations in SWMMC exceeded the U.S. Environmental Protection Agency (U.S. EPA) National Ambient Air Quality Standard (NAAQS) for O(3): 0.08 ppm as 1-h maximal concentration, not to be exceeded more than 4 times a year, on 71% of days in 1986 and 95% in 1997, with values as high as 0.48 ppm. Ozone maximal peaks are usually recorded between 2 and 5 pm coinciding with children's outdoor physical activities. Children in the control group reported no upper or lower respiratory symptomatology. Every SWMMC child complained of upper and/or lower respiratory symptoms, including epistaxis, nasal dryness and crusting, cough, shortness of breath, and chest discomfort. Children aged 7-13 yr had the most symptomatology, while 5- to 6-year olds and adolescents with the lowest number of statistically significant outdoor exposure hours had less respiratory symptoms. Bilateral symmetric mild lung hyperinflation was significantly associated with exposure to the SWMMC atmosphere (p = .0004). Chronic and sustained inhalation of a complex mixture of air pollutants, including ozone and particulate matter (PM), is associated with lung hyperinflation, suggestive of small airway disease, in a population of clinically healthy children and adolescents. Small airways are a target of air pollutants in SWMMC children, with ozone and PM being most likely responsible, based on experimental animal, controlled-chamber, and epidemiological data available. Our main concern is the potential likelihood for the development of chronic lung disease in this highly exposed population.

Imaging of congenital vascular and lymphatic anomalies of the head and neck.

Congenital lymphatic and vascular malformations and infantile hemangiomas can be combined under the heading of endothelial malformations. Based on their biologic behavior, endothelial malformations can be divided into two groups: infantile hemangiomas and vascular malformations. Vascular malformations can be subdivided into lymphatic, capillary, venous, and arteriovenous malformations. Often frightening for the patient and the patient's family, some endothelial malformations, however, will resolve; yet others can lead to long-term disfigurement and even can be fatal-due to airway obstruction, secondary infection, or exsanguination. When recognized early, however, the appropriate therapy, or watchful waiting, can be initiated, and the long-term physical and psychological consequences of these malformations can be minimized.

The pediatric airway: a review of differential diagnosis by anatomy and pathology.

In children, lesions involving and affecting the airway are numerous and vary in origin. Categorizing these lesions by origin (traumatic, inflammatory, congenital or developmental, neoplastic, and vascular) or by anatomy (nasal airway and nasopharynx, oral airway and oropharynx, larynx, trachea, and extrinsic lesions) will aid in narrowing the differential diagnosis and may guide imaging.

Children with congenital pulmonary lymphangiectasia: after infancy.

OBJECTIVE: The objective of this original report is to describe the characteristic chest imaging findings in children with primary congenital pulmonary lymphangiectasia who survive infancy. CONCLUSION: In children with primary congenital pulmonary lymphangiectasia, increased interstitial markings decrease over time and increased hyperinflation is associated with persistent patchy areas of ground-glass opacity.

Cervical neurofibromas in children with NF-1.

BACKGROUND: Children with neurofibromatosis type 1 (NF1) are at increased risk of developing plexiform neurofibroma throughout the body, including the cervical soft tissues. However, the incidence of cervical soft tissue tumors and the value of screening MR for children with NF1 are not known. PURPOSE: The purposes of this study were to determine the incidence and clinical significance of cervical tumors seen on MR imaging in children with NF1. MATERIALS AND METHODS: A retrospective review of the brain and orbit MR with cervical images obtained on 95 children who meet the NIH consensus criteria for NF1 and who are followed at our neurofibromatosis clinic was carried out. RESULTS: Cervical tumors were found on MR imaging in 21 of 95 (22%) children. Of 21 children with cervical tumors, 14 children were determined to be surgical candidates. In nine children, MR imaging altered the clinical management by demonstrating tumors for which surgery was indicated, but the tumors were not suspected prior to MR imaging. CONCLUSION: Cervical tumors are commonly seen in children with NF1. MR imaging may demonstrate a significant number of tumors that require surgery, but were not suspected prior to MR imaging.

Magnetic resonance imaging of neuroblastoma using current techniques.

We evaluated the ability of current magnetic resonance (MR) scanning techniques to detect and stage neuroblastoma in children, using surgical and histopathologic correlation. We prospectively and retrospectively reviewed 16 MR examinations from 14 patients with neuroblastoma (13 patients) or ganglioneuroblastoma (1 patient) and compared these to computed tomography (CT) (5 patients) and pathology (all patients). Sequences included: precontrast T1-weighted and T2-weighted images, and gadolinium-enhanced T1-weighted images. The study time for each MR exam was also calculated. Five primary tumors were intrathoracic paraspinous masses, eight were adrenal, and 1 was presacral. Neural foraminal invasion was demonstrated on MR in four of 14 patients. Three of the four patients had undergone CT and neural foraminal invasion was shown in one. Vascular encasement was demonstrated in five of 14 patients on MR images. Three of the five patients had undergone CT and vascular involvement was shown in two. All cases of neural foramina invasion and vascular encasement were proven at surgery. There were no false positive or false negative MR studies of neural foraminal invasion or vascular encasement. Bone marrow invasion was shown in two of 14 patients on MR images which were confirmed by bone marrow aspirate. No false negative cases of bone marrow invasion was shown. In one patient, CT considered one neuroblastoma to be adrenal in location which was correctly shown to be intrathoracic on MR. The mean study time for MR imaging was 49 min. Current MR techniques are accurate at detecting and staging neuroblastoma, and coverage of chest, abdomen, and pelvis can be performed in less than one hour.

Ehrlichiosis: findings on chest radiographs in three pediatric patients.

OBJECTIVES: Ehrlichiosis is a newly recognized tick-borne infection affecting both children and adults. The disease is usually mild, with flulike symptoms, but can be fatal. We present the chest radiographs and, in one case, the chest CT scan of all recognized pediatric cases of ehrlichiosis to date from our institution. CONCLUSION: Ehrlichiosis should be included in the differential diagnosis of acutely ill pediatric patients with a history of possible tick exposure and radiographic patterns of interstitial prominence, alveolar opacities, or consolidation that may be associated with pleural effusion.

Malignant intraperitoneal neoplasms of childhood.

OBJECTIVE: The purpose of this paper is to review and categorize the CT appearances of primary and secondary intraperitoneal neoplasms in children. MATERIALS AND METHODS: We retrospectively reviewed the CT images of 14 cases of intraperitoneal neoplasms. They were reviewed for bowel wall thickening, mesenteric nodules, ascites, calcification, peritoneal nodules, omental caking, and contrast enhancement. RESULTS: Computed tomography images of 14 cases of intraperitoneal neoplasms were evaluated: rhabdomyosarcoma (5), lymphoma (3), neuroblastoma (2), germ cell tumor (1), mesothelioma (1), Wilms tumor (1), and hepatocellular carcinoma (1). Nodular enhancement was seen in all cases, ascites in most, significant bowel wall thickening only with lymphoma, and calcification only with germ cell tumors. Omental caking was present both with rhabdomyosarcoma and lymphoma. CONCLUSIONS: While rare in pediatrics, intraperitoneal neoplasms occur in children, and CT is useful in identifying these peritoneal neoplasms.

Mediastinal lymphadenopathy caused by Mycobacterium avium-intracellulare complex in a child with normal immunity: successful treatment with anti-mycobacterial drugs and laser bronchoscopy.

We report on the case of a 9-month-old Caucasian girl referred to our institution with a history of fever of unknown origin and wheezing, unresponsive to bronchodilator and anti-inflammatory therapy. Subsequent investigation led to a diagnosis of mediastinal lymphadenopathy caused by Mycobacterium avium-intracellulare (MAI). The infected lymph tissue infiltrated and obstructed the right bronchus and significantly compressed the left bronchus to the point of near closure. Given the high degree of morbidity and potential mortality from thoracic surgery in this patient, we treated her with a combination of anti-mycobacterial drugs (rifabutin, clarithromycin, ciprofloxacin, clofazimine, amikacin, ethambutol) and glucocorticoids to relieve airway compression. The endobronchial granulation tissue was resected by laser bronchoscopy. This combined approach led to eventual normalization of radiologic and endoscopic findings, and the anti-mycobacterial chemotherapy was discontinued 12 months after the first bronchoalveolar lavage culture was negative for MAI. The patient remains asymptomatic 1 year after completion of this course of therapy. We suggest that mediastinal lymphadenopathy with bronchial infiltration and extrinsic airway compression caused by MAI in otherwise healthy children can be successfully treated with aggressive chemotherapy, glucocorticoids, and laser bronchoscopy.

Efficacy and feasibility of breast shielding during abdominal fluoroscopic examinations.

RATIONALE AND OBJECTIVES: The purpose was to measure radiation exposure to the breasts during abdominal fluoroscopic examinations and evaluate the efficacy of breast shielding with a leaded vest. MATERIALS AND METHODS: Sixty-six women underwent routine abdominal fluoroscopic examinations. During the examinations one breast was covered with a leaded shield. Radiation doses to both breasts were measured with a thermoluminescent dosimeter. The amount of radiation at the skin of the shielded breast was then compared with that at the skin of the nonshielded breast. RESULTS: Radiation exposure to the breasts varied substantially with the type of examination being performed and with the individual patient. The average radiation level at the skin of the unshielded breast was 119 mR (range, 0-6,320 mR), compared with 59.6 mR (range, 0-1,640 mR) at the shielded breast. The average reduction in radiation exposure was 50% with shielding. CONCLUSION: Although the average level of radiation exposure to the breast during abdominal fluoroscopic examinations is generally low, use of a leaded vest can further reduce radiation to the breast for different types of examinations.

Coexisting hyperplastic antral polyp and hypertrophic pyloric stenosis.

This report describes a child with hypertrophic pyloric stenosis with persistent vomiting after pyloromyotomy due to a coexistent hypertrophic antral polyp.

MR of neurologically symptomatic newborns after vacuum extraction delivery.

We present the MR findings in three neurologically symptomatic newborns after vacuum extraction delivery. The lesions included subdural hematomas, one tentorial hematoma, and one intracerebellar hemorrhage. One patient had hydrocephalus that required shunting. We propose that the visualized abnormalities are probably the result of vertical stress leading to laceration of bridging veins, venous sinuses, and/or venous hemorrhagic infarctions.