Adult respiratory distress syndrome in full-term newborns.

Since 1984, 11 newborns with severe respiratory distress have been treated whose clinical characteristics appear distinctive. Characteristics of these neonates were as follows: (1) they were full term by obstetric and neonatal criteria, (2) they had diffuse bilateral alveolar opacification on chest radiographs during the acute illness, (3) each had an acute perinatal triggering insult, (4) the neonates required continuous positive pressure ventilation for at least 48 hours with FiO2 greater than 0.50 for at least 12 hours, (5) they needed positive end-expiratory pressure of 6 cm of H2O or greater within three days of the triggering event, (6) there were no other known causes of these clinical conditions. Ten (91%) neonates had evidence of other organ dysfunction in addition to the lungs. Trials of hyperventilation in nine and tolazoline in five failed to improve oxygenation. Ten infants who underwent trials of increased positive end-expiratory pressure greater than or equal to 6 cm of H2O without other concurrent changes in ventilator settings responded with prompt increases in PaO2 (median increase 84 mm Hg, range 22 to 196 mm Hg). All 11 babies survived but required prolonged mechanical ventilation and supplemental oxygen. We suggest that adult respiratory distress syndrome can and does occur in newborns. A trial of positive end-expiratory pressure greater than or equal to 6 cm of H2O should be considered in full-term infants with severe respiratory distress in whom other causes can be excluded.

Apert syndrome with frontonasal encephalocele.

We describe a female infant with Apert syndrome (acrocephalosyndactyly, type I) and a frontonasal encephalocele with unremarkable family history.

Ureaplasma urealyticum and chronic lung disease in very low birth weight infants during the exogenous surfactant era.

BACKGROUND: An association between recovery of Ureaplasma urealyticum from the respiratory tract of very low birth weight (VLBW) infants (< or =1500 g) and later chronic lung disease (CLD) was reported by several authors before the routine use of exogenous surfactant (SURF). We sought to assess whether this relation persists in the era of routine SURF. METHODS: We prospectively studied a cohort of 105 VLBW infants who required mechanical ventilation at < 12 h of age. Tracheal aspirates for U. urealyticum culture were obtained before administration of SURF or antibiotics. Clinicians were unaware of U. urealyticum status. Chest radiographs at 28 days were reviewed by a single pediatric radiologist, blinded to U. urealyticum status. Sample size was predetermined to detect a 30% increase in CLD among those with U. urealyticum recovery from tracheal culture (U. urealyticum-positive) with alpha <0.05 and beta <0.20. RESULTS: Of the study infants 22 were U. urealyticum-positive and 83 were U. urealyticum-negative. No differences were found between the groups for birth weight, gestational age, gender, inborn, antenatal or postnatal steroid use, SURF therapy, non-U. urealyticum infection, necrotizing enterocolitis, patent ductus arteriosus, intraventricular hemorrhage or cystic periventricular leukomalacia. At 28 days U. urealyticum-positive patients were significantly more likely to have CLD than U. urealyticum-negative [15 of 22 (68%) vs. 30 of 83 (36%); P < 0.02]. The U. urealyticum-positive patients also required significantly longer courses of supplemental oxygen and mechanical ventilation. No significant differences were found for CLD at 36 weeks postconception or duration of hospitalization, although type II error could not be excluded for these secondary endpoints. CONCLUSIONS: Respiratory U. urealyticum at or shortly after birth remains associated with CLD at 28 days despite routine use of SURF. Controlled trials of anti-Ureaplasma therapy in U. urealyticum-positive VLBWs as soon after birth as possible may determine whether CLD, duration of respiratory support and attendant costs can be decreased.

Erroneous sonographic identification of fetal lateral ventricles: relationship to the echogenic periventricular "blush".

We show, through correlation of fetal, neonatal, and necropsy brain sonography, that the three-parallel-echogenic-line configuration classically thought to represent fetal lateral ventricular margins on high axial images are actually extraventricular, lying within cerebral white matter. Accurate sonographic identification of fetal hydrocephalus is essential for appropriate patient management. Both qualitative and quantitative methods of determining ventriculomegaly have been described. The latter require identification of lateral ventricular walls for subsequent ventricular mensuration and comparison with hemispheric width. The parallel-line configuration, thought to represent ventricular margins, has been used for such measurements. We have--through careful fetal, neonatal, and necropsy specimen scanning in several planes--determined that the presumed ventricular wall echoes are actually extraventricular, arising from fibers within the cerebral white matter aligned perpendicular to the sonographic beam. This is identical to the etiology of the normal echogenic periventricular "blush" described in the neonate. Since these parallel echogenic lines do not represent actual ventricular margins, they should not be relied upon for the diagnosis of ventriculomegaly.

Periventricular cavitations in the first week of life.

Eleven infants were encountered (nine premature, two term) in whom well-defined small periventricular cavitations were found by sonography in the first week of life. The sonographic findings bore remarkable similarity to subependymal pseudocysts in neonates previously described in autopsy specimens. The cavitations, which were identified predominantly along the superolateral aspects of the lateral ventricles, did not evolve in the manner of postnatally acquired periventricular leukomalacia. The location of the cavitations differed from the site of previously reported lesions of posthemorrhagic and postinfectious germinolysis along the medial aspect of the caudothalamic groove. Neurosonologists and neonatologists should be alerted to this finding and encouraged to follow these infants as a separate group to learn whether neurodevelopmental sequelae occur in these children.

Spinal ultrasonography. Intraoperative and pediatric applications.

Ultrasonography has had limited usefulness in the diagnosis of diseases of the spine and spinal cord in older children and adults. However, its use intraoperatively in patients of all ages, and nonoperatively in infants and young children, is finding increased application. The authors, who perform and rely on spinal sonography in both clinical situations, present an overview of technique, normal anatomy, and selected pathologic entities based on their experience and the literature.

Tracheal schwannoma presenting as status asthmaticus in a sixteen-year-old boy: airway considerations and removal with the CO2 laser.

A 16-year-old with clinical features of atypical asthma is presented, with a description of the workup leading to the diagnosis of an intratracheal mass. The mass was visualized with a flexible fiberoptic bronchoscope, then surgically removed through a rigid bronchoscope using a CO2 laser. We believe this is the first report of resection using this technique. A discussion of tracheal neurilemmomas (schwannoma) is included. This case reinforces the age-old adage that "not all that wheezes is asthma."

An unusual complication of tracheostomy.

Synthetic pediatric tracheostomy tubes have been a major advance for both short- and long-term airway support. We present a late complication from the use of a Shiley silicone pediatric tracheostomy tube (Silastic), and its radiologic manifestations. To our knowledge, this complication has not been previously reported.

Cranial sonography and neurological examination at term and motor performance through 19 months of age.

This study examined the role of cranial sonography and neurological examination in the prediction of developmental progress during the first 19 months of life in extremely preterm infants. Forty-seven infants with mean birth weight 993 +/- 197 g and gestational age 27.9 +/- 1.9 weeks were studied. Each infant was classified as normal, suspect, or abnormal using cranial sonography and a structured neurological examination. Developmental outcome was assessed using the Infant Neurological International Battery (INFANIB) at 4, 8, and 14 months and the Rockford Infant Developmental Evaluation Skills (RIDES) at 19 months. Sonographic classification was the best predictor of outcome through 14 months, F(2.72) = 12.4, p < .001. The neurological examination predicted performance only at 4 months. No infant classified as normal on both examinations was abnormal on follow-up. Infants with suspect or abnormal neurological examinations had normal outcomes if their sonographic findings were normal.

Utility of sonography in the diagnosis of bronchopulmonary sequestration.

Sonography in six patients with pulmonary sequestration demonstrated findings associated with and indicative of that diagnosis. The most useful feature, which was seen in three cases and is diagnostic of sequestration, is the identification of an anomalous systemic artery arising from the aorta.

Diagnosis and management of congenital cystic disease of the lung in children.

Pulmonary sequestration, congenital adenomatoid malformation (CAM), congenital lobar overinflation (CLO), and bronchogenic cysts are four congenital lesions that may present as abnormal cystic areas within the pleural cavity in early life. They share similar clinical and embryologic characteristics, are frequently difficult to diagnose, and all require surgical treatment. From December 1974 to January 1985, 22 patients were operated upon on the Pediatric Surgical Service for congenital cystic disease of the lung. There were eight females and 14 males, ranging in age from 1 day to 18 years; 12 were under 6 months of age, and 14 were under one year. There were 7 CAMs, 5 extralobar pulmonary sequestrations, 5 intralobar pulmonary sequestrations, 3 bronchogenic cysts, and 3 cystic lobes caused by CLO. One patient had bilateral pulmonary sequestrations, intralobar on one side and extralobar on the other, with both sharing a common systemic artery arising from the infradiaphragmatic aorta. All except two asymptomatic patients presented with either progressive respiratory distress or recurrent pulmonary infections, and had cystic changes noted on chest roentgenograms. The differentiation of these congenital lesions from resolvable inflammatory cysts is important. Diagnosis was aided by selective utilization of barium contrast studies, ultrasonography, computed tomography, and arteriography. Operation consisted of resection alone of the extralobar pulmonary sequestrations and bronchogenic cysts, and total lobectomy in CAM, intralobar sequestration, and CLO. One infant with CAM died 1 day postoperatively from bilateral hypoplastic lungs. The other 21 patients are alive and well with follow-up ranging from 1 month to 9 years, with a mean of 3.5 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Protocol for the use of videofluoroscopy in pediatric swallowing dysfunction.

A clinical and research protocol was developed to compare the sensitivity and clinical value of videofluoroscopy with the traditional bedside clinical evaluation in the evaluation of children's swallowing dysfunction. The bedside and videofluoroscopic evaluations of 33 children were reviewed retrospectively. Our findings indicated that recommendations for feeding changed for 14 of the children as a result of the information gained from videofluoroscopic evaluation. The results suggest that videofluoroscopy provides therapists with more objective evidence than a bedside evaluation for determining the etiology of swallowing dysfunction and directing the management or treatment of this dysfunction.

The conus medullaris: normal US findings throughout childhood.

A prospective ultrasound (US) study of children without neurologic or spinal problems or midline cutaneous abnormalities on the back was performed to determine the level of the conus medullaris in healthy children. The tip of the conus was identified in sagittal and transverse planes, and a radiopaque bead was placed on the skin to mark the location of the tip. Findings at subsequent radiography confirmed the vertebral level. A total of 161 children, aged 4 days to 13 years 5 months (mean, 3 years 7 months; median, 3 years 1 month), composed the study population. Conus levels in these healthy children varied from the level of T10-11 interspace to the superior aspect of the L-3 vertebra. However, in only two children were the conus levels below the middle portion of L-2. Estimations of vertebral levels of the conus based on the US scan alone were within one vertebral level of the level determined at radiography in 145 of 161 (90%) cases. Palpable landmarks, the tip of the lowest rib and the iliac crest, corresponded to the level of the L-2 and L-5 vertebrae, respectively. The conus medullaris can be located at US in most children, even beyond infancy. The normal levels are constant throughout childhood.

Superior mesenteric vascular anatomy at US in patients with surgically proved malrotation of the midgut.

The purpose of this study was to determine the frequency with which inversion of the superior mesenteric artery (SMA) and superior mesenteric vein (SMV) is seen at ultrasound (US) in children with malrotation. Nine patients with malrotation at upper gastrointestinal (UGI) radiographic examination (surgically confirmed) were studied prospectively. Anatomic relationships between the SMA and SMV were evaluated with real-time US in an axial projection, with the transducer placed at the midline of the anterior abdomen. Use of duplex imaging confirmed which vessel was the artery and which was the vein. Vessels were considered to be inverted if the SMV was left of the left lateral margin of the SMA. The SMA and SMV were inverted in six patients (67%) and were normal in three (33%). One patient with normal mesenteric vessels had midgut volvulus; duodenojejunal resection was required. Inversion of the SMA and SMV at US is not sufficiently sensitive to screen patients for malrotation. UGI examination remains the standard modality to diagnose this disorder.

Mesenteric vascular anatomy at CT: normal and abnormal appearances.

The appearance of the mesenteric vessels on computed tomographic scans was evaluated in 187 patients to determine the variations in the anatomic relationships between the mesenteric vessels in normal patients, in patients with malrotation, and in patients with abdominal masses. In 166 patients (88.8%), a portion of the cranial segment of the superior mesenteric vein (SMV) was anterior and to the right of the superior mesenteric artery (SMA), although other normal configurations also occurred. A portion of the cranial segment of the SMV was directly anterior to the SMA in 15 patients (8.0%) and directly to the right of the SMA in 16 patients (8.6%). Of eight patients with complete inversion of the SMA and SMV, one had normal midgut rotation, two had malrotation, and six had neoplasms that displaced the vessels (one of these six also had malrotation). Of eight patients with inversion of only a part of the mesenteric vessels, none had malrotation. Although mesenteric inversion should stimulate a careful evaluation for malrotation, it is not pathognomonic of this condition.

Imaging of idiopathic biliary duct dilatation.

Gray-scale ultrasonography and 99mTc-HIDA radionuclide scanning are complementary studies which may be helpful in the diagnosis of idiopathic dilatation of the biliary ducts. We present an overview of this entity and representative cases of a Type I choledochal cyst and Caroli's disease.

Symptomatic Chiari I malformation in childhood: a report of 7 cases.

The Chiari I malformation, once thought to be a disorder of clinical importance only in the teen years and beyond, is now recognized as being significant in the pediatric population as well. We have reviewed 7 cases of Chiari I malformation in children less than 12 years of age. Hydrosyringomyelia was often, but not invariably, an accompanying feature, and this in turn was associated with scoliosis and motor weakness. All patients had posterior fossa and cervical decompression with dural grafting procedures, as well as various types of syringosubarachnoid shunts placed. Intraoperative ultrasound was found to be a valuable surgical adjunct. Symptoms and signs were improved or stabilized in all patients. We feel that this disorder is now more commonly reported in young children because of the increased availability of noninvasive neuroimaging techniques, especially magnetic resonance imaging. Questions are raised as to the actual incidence of undiscovered cases of this disorder in children with commonly seen conditions such as scoliosis.