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While pectus excavatum is the most common congenital chest wall deformity in children, indications for surgical repair are still debated among experts. Some centers require demonstration of physiologic criteria prior to repair, even in the face of significant anatomic compression. We present a case of a 16-year-old male with severe pectus excavatum who presented with a 3-year history of a chronic barking cough and exercise-induced shortness of breath. Radiographic imaging demonstrated compression of the trachea and bilateral mainstem bronchi secondary to severe pectus excavatum deformity. Despite his severe pectus deformity, preoperative physiologic testing demonstrated normal to near-normal cardiopulmonary function. Minimally invasive repair of the chest wall defect (modified Nuss procedure) provided rapid alleviation of symptoms, and a significant improvement in quality of life.

Chorioamnionitis and elevated cord blood inflammatory cytokine concentrations are associated with detectable disturbances of systemic and cerebral hemodynamics in premature newborns. Fifty-five infants (25–31 wk gestation) were enrolled. Chorioamnionitis was defined by placental histology. IL-6, IL-1β, and tumor necrosis factor-α were quantified by ELISA. Blood pressure, heart rate, cardiac output, stroke volume, fractional shortening, and middle cerebral artery blood flow velocities were measured at 3 ± 1 h after birth. Chorioamnionitis was evident in 22 placentas and was associated with increased IL-6 (p < 0.001), IL-1β (p = 0.035), and heart rate (p = 0.027); and with decreased mean and diastolic blood pressure (p = 0.026 and p = 0.019, respectively). IL-6 concentration correlated inversely with systolic, mean, and diastolic blood pressures. Right ventricular cardiac output was elevated (p = 0.028) in infants with fetal vessel inflammation. Maternal temperature ≥38.0°C and newborn immature-to-total white blood cell ratio ≥0.4 were associated with significant decreases in left ventricular fractional shortening (p = 0.001 and p = 0.005, respectively). Neither chorioamnionitis nor elevated cytokine concentrations were associated with changes in middle cerebral artery Doppler blood flow velocities. Chorioamnionitis and elevated cord blood IL-6 concentrations are associated with decreased blood pressure in premature newborns. Inflammation of the fetal vessels and nonspecific indicators of infection are associated with disturbances in cardiac function. Infants with chorioamnionitis and elevated cytokine concentrations do not manifest changes in cerebral Doppler indices within the first few postnatal hours. We speculate that cytokine-associated systemic hemodynamic disturbances in premature infants born after chorioamnionitis predispose such infants to perinatal brain injury.

Chorioamnionitis and elevated cord blood inflammatory cytokine concentrations are associated with detectable disturbances of systemic and cerebral hemodynamics in premature newborns. Fifty-five infants (25–31 wk gestation) were enrolled. Chorioamnionitis was defined by placental histology. IL-6, IL-1, and tumor necrosis factor- were quantified by ELISA. Blood pressure, heart rate, cardiac output, stroke volume, fractional shortening, and middle cerebral artery blood flow velocities were measured at 3 ± 1 h after birth. Chorioamnionitis was evident in 22 placentas and was associated with increased IL-6 (p< 0.001), IL-1 (p0.035), and heart rate (p0.027); and with decreased mean and diastolic blood pressure (p0.026 and p0.019, respectively). IL-6 concentration correlated inversely with systolic, mean, and diastolic blood pressures. Right ventricular cardiac output was elevated (p0.028) in infants with fetal vessel inflammation. Maternal temperature ≥38.0°C and newborn immature-to-total white blood cell ratio ≥0.4 were associated with significant decreases in left ventricular fractional shortening (p0.001 and p0.005, respectively). Neither chorioamnionitis nor elevated cytokine concentrations were associated with changes in middle cerebral artery Doppler blood flow velocities. Chorioamnionitis and elevated cord blood IL-6 concentrations are associated with decreased blood pressure in premature newborns. Inflammation of the fetal vessels and nonspecific indicators of infection are associated with disturbances in cardiac function. Infants with chorioamnionitis and elevated cytokine concentrations do not manifest changes in cerebral Doppler indices within the first few postnatal hours. We speculate that cytokine-associated systemic hemodynamic disturbances in premature infants born after chorioamnionitis predispose such infants to perinatal brain injury.

Objective To retrospectively study prenatal ultrasound images of patients with craniosynostosis to determine the extent to which prenatal diagnosis is possible.Method Prenatal ultrasound images of 19 patients with postnatally diagnosed metopic or coronal suture craniosynostosis were retrospectively reviewed. The 26 ultrasound examinations obtained were compared with normal images and tables of gestation.Results It was not possible to diagnose craniosynostosis in the first trimester. In the second trimester, Kleeblattschädel was diagnosed at 20.5 weeks. A multilobular shape to the skull and diastasis of the frontotemporal suture was identified. In a second child with Kleeblattschädel, the cephalic index was above normal 86.4 (normal range 70 to 86), and the head circumference to abdominal circumference was increased. In the third trimester, the head shape deformation was more obvious. Brachycephaly diagnosis was made in the second trimester. In Crouzon syndrome the hypertelorism was identified at 19.9 weeks. Plagiocephaly was diagnosed at 21.4 weeks. In trigonocephaly the reduced cephalic index was noted at 18.8 weeks. In the third trimester, the deformity was diagnosed in all cases.Conclusion No diagnosis of craniosynostosis was made in the first trimester. In the second trimester, it was possible to diagnose Kleeblattschädel, tri-gonocephaly, brachycephaly (bilateral coronal suture craniosynostosis), and plagiocephaly (unilateral coronal suture craniosynostosis) in nine of the examinations. In the third trimester and at term, it was possible to diagnose previously listed conditions from six of the seven examinations obtained. Klee-blattschädel was suspected during original examination. A total of 15 examinations of 26 were correctly diagnosed during this investigation.

A retrospective study of 101 children who underwent tracheotomy at the Children's Hospital of Pittsburgh from 1993 to 1996 was performed. The following criteria were reviewed in each patient: age, gender, race, prematurity, weight during tracheotomy, presence of preoperative airway support, duration of tracheotomy, nature (emergent versus elective), tracheotomy tube size, reason for tracheotomy, accompanying medical diagnoses, chest x-ray (CXR) findings, surgical service, postoperative symptoms (up to 3 days), and operative mortality rate. Of these criteria, our results show that CXR-screenable complications occurred in patients who underwent emergent recannulation, as well as those who exhibited ventilatory distress (oxygen saturation level of <90%) and specific changes in postoperative symptoms. Pneumothorax developed after tracheotomy in 3 of the 101 patients; each had one of these risk factors. We conclude that CXR of all pediatric patients after tracheotomy may be unnecessary with the use of flexible endoscopy and screening restrictions that are both health-conscious and cost-effective.

Interventional techniques have become well established and offer safe and effective alternatives for management of the sick patient in the 1CU setting. Most likely, the radiologist will become more involved in the management of sick children and adults, thus reducing the need for open surgical procedures and the complexity of surgery, and treating complications.

This report describes the clinical course, diagnostic evaluation and management of six children with bilateral renal artery stenosis (RAS) and concurrent narrowing of the abdominal aorta. Except for one child with active arteritis, the others were asymptomatic. There were no clinical or laboratory features suggesting the etiology of hypertension in four of six patients, and diagnostic procedures, including Doppler duplex ultrasound and captopril scintigraphy, were unreliable in screening for such hypertension. Abdominal aortography and selective renal angiography confirmed the diagnosis of bilateral RAS and associated anatomical alterations of the aorta and its branches. The hypertension was severe and minimally responsive to antihypertensive agents. It was cured or improved after percutaneous transluminal angioplasty (PTA) of three vessels in two children with mid-vessel stenoses, while hypertension persisted after PTA of two mid-vessel stenoses in a third child and one vessel with ostium stenosis in a fourth child. Autotransplantation of seven kidneys in four children resulted in cure or significant improvement of the hypertension. Renal function was preserved in all children during a mean follow-up time of 41 months. Based on illustrative data from these six children, as well as information from a review of the literature, this report discusses the key diagnostic issues and stresses the potential advantages of renal autotransplantation in selected children with this disorder.

A child with nephropathic cystinosis developed seizures and coma. CT showed prominent sulci and slight ventricular enlargement. Nuclear cisternogram was normal. Despite successful renal transplantation and treatment of hypothyroidism, neurologic recovery was poor. CT and nuclear cisternogram 5 months later showed moderate panventricular and subarachnoid space enlargement and abnormal ventricular isotope retention. Ventriculoperitoneal shunt placement was followed by improved intellectual function, resolution of pyramidal tract signs, and control of seizures. Anisotropic crystals consistent with cystine were demonstrated in biopsy samples of arachnoid and cerebral cortex. Nonabsorptive hydrocephalus may have resulted from deposition of cystine in the meninges.

Neurocapsular growth is highly heritable and determines neurocranial form. Although craniosynostosis alters brain growth direction, resulting in compensatory changes in the neurocranium, it is believed that such compensations occur without reduction in intracranial volume. This hypothesis was tested in a rabbit model with nonsyndromic, familial coronal suture synostosis. Skulls of 56 rabbits 20 normals, 20 with delayed onset synostosis, and 16 with complete synostosis were scanned using threedimensional computed tomography at 6 and 18 weeks of age. Intracranial contents were reconstructed, and indirect intracranial volume was calculated. Qualitatively, reformations of intracranial contents from completely synostosed rabbit skulls exhibited the typical “copper beaten” morphology. Quantitatively, intracranial volume was significantly p< 0.05 reduced in rabbit skulls with complete synostosis compared with both control rabbit skulls and rabbit skulls with delayed onset synostosis at 6 weeks by 11 percent and 14 percent, respectively. By 18 weeks, intracranial volume in rabbit skulls with synostosis was significantly p< 0.05 reduced by 12 percent in complete synostosis and 8 percent in delayed onset synostosis compared with normal rabbits. Results suggest that in rabbits with uncorrected craniosynostosis, compensatory changes in the neurocranium were not adequate to allow normal expansion of the neurocapsular matrix. Further research is needed to determine whether reduction in intracranial volume was a result of neural tissue deficiency or cerebrospinal fluid i.e., ventricular or subarachnoid space compression in this model. Plast. Reconstr. Surg.100 1121, 1997.

Tuberous sclerosis is characterized by the development of hamartomas in many organs, particularly the brain, skin, heart, and kidney. We report a child with typical features of this disease who also had a severe generalized vascular dysplasia of both muscular arteries and veins and an abdominal aortic aneurysm in association with obliterative dysplasia of the aortic vasa vasorum. This is the first report of such a widespread vascular abnormality in association with tuberous sclerosis. This case along with several reports of more isolated vascular abnormalities in these patients indicates that vascular dysplasia represents another manifestation of the generalized tendency to hamartoma formation seen in this disorder.

Although craniosynostosis alters brain growth direction resulting in compensatory changes in the neurocranium, it has been suggested that such compensations occur with little reduction in intracranial volume (ICV). This hypothesis was tested in a rabbit model with nonsyndromic, familial coronal suture synostosis. Cross-sectional three-dimensional computed tomographic head scans were obtained from 79 rabbits (25 normal, 28 with delayed-onset synostosis, and 26 with early-onset synostosis) at 25, 42, and 126 days of age. Intracranial contents were reconstructed and indirect ICV was calculated. Results revealed that by 25 days of age the intracranial contents from early-onset synostosed rabbit skulls showed rostral (anterior) constrictions and a “beaten copper” morphology in the parietal and temporal regions compared with the other two groups. These deformities increased in severity with age. Quantitatively, ICV was significantly reduced (p< 0.05) by 7 in rabbits with early-onset synostosis compared with both control rabbits and rabbits with delayed-onset synostosis at 25 days of age. By 126 days of age, ICV in rabbits with synostosis was significantly reduced (p< 0.05) by 11 in early-onset synostosis and by 8 in delayed-onset synostosis compared with normal rabbits. Results suggest that in rabbits with uncorrected craniosynostosis, compensatory changes in the neurocranium were not adequate to allow normal expansion of the neurocapsular matrix. Further research is needed to determine if ICV reduction is correlated with cerebral atrophy or cerebral spinal fluid (i.e., ventricular or subarachnoid) space compression in this model.

Preoperative aortograms were reviewed in six children with thoracic myelomeningocele and lumbar kyphosis at an average age of 25 months. In all children, the abdominal aorta bridged the lumbar kyphosis. In four children, the upper renal poles were close to the spine and were at potential risk during kyphectomy. All children had aortic shortening with an increased variability in origin of the major abdominal aortic branches. Abnormal lumbar arteries were noted in four children.