Edmund A. Franken

Gastroesophageal reflux in infants and children-comparative accuracy of diagnostic methods*

To assess the diagnostic accuracy of methods employed for detection of gastroesophageal reflux, 30 infants and children with symptoms of GER were evaluated by upper gastrointestinal series, gastroesophageal scintiscan, measurement of mean resting lower esophageal sphincter pressure, esophageal intraluminal PH measurement (acid reflux test), and endoscopy. Fifteen control patients were also evaluated by the above studies. GER was demonstrated by UGI in 15 and by GE scintiscan in 17 study patients. LESP less than 15 mm Hg was noted in 12 and a positive acid reflux test was obtained in 29 study patients. Esophagitis was detected in two (of 30) study patients radiographically and in 15 (of 21) study patients by upper gastrointestinal endoscopy. Pulmonary aspiration of gastric contents was not detected by the radionuclide method. None of the 15 control patients had GER demonstrated with any of the above methods. These studies indicate that (1) the acid reflux test correlates most closely with symptoms of GER; (2) THE GE scintiscan is complementary to the UGI in the diagnosis of GER, i.e., the combination increases sensitivity; (3) normal LESP does not necessarily exclude GER; and (4) endoscopy is superior to the UGI in detecting the presence of esophagitis.

The radiographic spectrum in neurofibromatosis

N EUROFIBROMATOSIS (von Recklinghausen disease) is a hereditary condition resulting from dysplasia of neuroectodermal and mesoderma1 tissues. Any organ or system of the body may be primarily or secondarily involved in this disease. It is estimated that the incidence of neurofibromatosis is one in 3000 births.g It is transmitted as an autosomal dominant, but 50% of cases arise as spontaneous mutants. There is no known sex or racial predominance. von Recklinghausen gave the first organized description of the disease in 1882,57 although other authors had previously described some of its manifestations. From that time forward, knowledge of the entity has been broadened by hundreds of reports and studies. The etiology is not known. There is marked variation of the histologic pattern of lesions in neurofibromatosis and considerable disagreement as to the cell or cells of origin. It is known that the serum of a high percentage of these patients contains increased nerve growth stimulating activitypg which may account for the apparent multiple cell origin. l6 Primary neuroectodermal and mesodermal dysplasia apparently are responsible for most of the manifestations of neurofibromatosis. Lesions may arise from the neuroectodermal sheath cells of Schwann and mesodermal connective tissue elements.47 The classical clinical signs include cutaneous pigmentation (cafe au lait spots), multiple soft cutaneous tumors, and palpable neurofibromas of peripheral nerves. To this can be added manifestations of dysfunction of every organ system. Some of us were taught early in our medical careers that syphilis is the great imitator of other diseases. This statement is also true of neurofibromatosis. The purpose of this paper is to describe and

Tumors of the chest wall in infants and children.

Chest wall tumors of children may arise from subcutaneous tissues, the bony thorax, or extrapleural region. Review of experience at our institution and the literature indicates that primary bony tumors are most often malignant; Ewings sarcoma is the most frequent in this category. Extrapleural tumors are rare but also usually malignant. Clinical and roentgen features of these lesions are discussed.

Angiographic determination of arterial patency after percutaneous catheterization in infants and small children.

Patency of the femoral artery of infants and children who previously had percutaneous arterial catheterization at a weight of < 25 kg was studied angiographically. During the study period, 118 patients had repeat arterial catheterization, 48 from the opposite leg. Femoral artery occlusion was found in four patients, while 44 of the 48 studied from the opposite leg had complete patency. At the later study, all four with blockage were asymptomatic and possessed good pedal pulses, while two had decreased femoral pulsations. When events surrounding initial catheterization were retrospectively analyzed, three of four had decreased pedal pulsations beyond six hours. It is concluded that 3-8%percnt; of patients weighing < 25 kg have arterial occlusion after catheterization. This complication may be entirely asymptomatic, but these patients will require continued observation for possible late vascular problems.

Intestinal Pseudo-Obstruction in Mucocutaneous Lymph-Node Syndrome

Mucocutaneous lymph-node syndrome (MCLS) is an acute exanthem with specific clinical features, sometimes complicated by involvement of internal organs. Two patients with MCLS had clinical and radiographic evidence of mechanical small-bowel obstruction, probably on the basis of focal vascular insufficiency, as anatomic obstruction was not documented in either instance. The cases indicate that intestinal involvement in patients with MCLS can simulate intestinal obstruction, and conservative management of such patients is appropriate.

Congenital dysplasia of C2--6.

Neural arch dysplasia of the cervical 4 vertebrae is a rare occurrence. The authors present a case with dysplasia of cervical bodies and posterior arch elements at multiple levels. The embryogenesis of these anomalies is discussed.

Roentgenology of colon atresia

The clinical and roentgenologic features of three neonates who had atresia of the colon are described. There were features of obstruction evident on plain roentgen films of the abdomen and contrast enema confirmed this in all patients. The importance of early diagnosis of this lesion is emphasised. The complications and differential diagnosis are discussed.

Roentgenographic Evaluation of Infant and Childhood Trauma

The roentgenographic evaluation of the traumatized child must be appropriate for the injuries present. In the acute phase emergency screening roentgenograms tailored to the type of trauma being investigated are performed. Detailed evaluation and special studies are performed when indicated. In some instances these examinations are needed on an emergency basis; in other cases a more leisurely study can be performed when the patients condition improves.

Natural history of ventricular-septal defects associated with ventricular-septal aneurysms

Abstract The diagnosis of ventricular-septal aneurysm was established by left ventricular cineangiography in forty-four patients. Six of the patients had a ventricular-septal aneurysm without a ventricular-septal defect at the time of their initial catheterization. Twenty-seven patients who had a ventricular-septal defect and ventricular aneurysm as their only significant cardiovascular lesion were followed for a mean of four years and ten months. The left-to-right shunt ratio was less than 1.5:1 in all cases. Shunt size decreased in six out of seven patients subjected to repeat catheterization. In no case were we able to document complete closure of the ventricular-septal defect. Thus, although a ventricular-septal aneurysm may represent a mechanism for partial closure of a ventricular-septal defect, complete closure of the defect appears unlikely if it remains open past the first few years of life.

Angiographic Assessment of Occipital Encephaloceles

Abstract Previously utilized means of assessment of occipital encephaloceles are of limited value in defining the anatomy of the sac contents, determination of surgical candidates, and prognosis. Posterior fossa angiography has been utilized in 4 cases. It was helpful in determining the neural contents of the sac and particularly brain-stem positioning as judged from angiographic visualization of the basilar artery. Variability of the basilar artery ranging from normal to complete extracranial positioning is shown. Surgical candidacy and prognosis have been ascertained by the angiographic anatomy.