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This report describes a unique clinicopathologic entity characterized as a malignant small cell tumor of the thoracopulmonary region in 20 children and adolescents (average age 14.5 years). There was a female predilection (75%) for this tumor which appeared to originate in the soft tissues of the chest wall or the peripheral lung. The neoplasm tended to recur locally and did not seem to disseminate as widely as some of the other small cell tumors of childhood (rhabdomyosarcoma, Ewing's sarcoma, neuroblastoma and malignant lymphoma). However, the median survival was only 8 months. Electron microscopy of 3 cases suggested a neuroepithelial derivation, but, at the present, the histogeneis remains a subject for further investigation.

Although a variety of metaphyseal and spondylometaphyseal dysplasias have been identified, the natural course of the skeletal defects in these disorders has not been well characterized. We describe an 8-year-old girl with spondylometaphyseal dysplasia, most closely resembling the Kozlowski type, in whom rachitic metaphyseal involvement underwent dramatic radiographic healing when she wore leg braces; discontinuation of the braces was followed by recurrence of the growth plate abnormalities. A generalized disturbance in mineral metabolism or skeletal remodeling to explain these radiographic changes was excluded by extensive biocheḿical studies and histologic examination of an undecalcified iliac crest specimen. Our observations suggest that the defect in endochondral bone formation in the Spondylometaphyseal dysplasias may be responsive to mechanical factors and that interventions that alter stress and strain on the skeleton, such as immobilization or use of orthopedic braces, may considerably modify the radiographic appearance of the growth plate disturbance.

Three cases of premature infants with obstruction of the ileum and proximal colon in the absence of cystic fibrosis are presented. Meconium inspissation complicated low-grade bowel obstruction in 2 infants. In the third, the bowel was anatomically normal, with the tenacious meconium the apparent primary cause of obstruction. Distinguishing features of this unusual type of blockage and other meconium syndromes are also discussed. This condition may simulate necrotizing enterocolitis, requiring surgery for differentiation. It should be considered in the differential diagnosis of low small bowel or colonic obstruction in the premature infant who is several days of age.

Eight patients with an anomaly of the iliac bone consisting of a protuberance projecting into the greater sciatic notch are discussed. The anomaly may be associated with sciatic nerve compression and atrophy; sacral, buttock, or intradural lipomata; tethered spinal cord; and ipsilateral lower extremity weakness. Surgical treatment can result in improvement of neurologic symptoms and signs.

Calyceal diverticulum is a relatively rare lesion that is usually asymptomatic but can assume clinical significance if drainage is impaired. Three patients are presented with unusual complications directly referable to a calyceal diverticulum. One child had a mobile calculus. In 2 others, intrarenal inflammatory masses developed in the area previously occupied by a calyceal diverticulum. One of these children had proved xanthogranulomatous pyelonephritis, and the other had an infected diverticulum.

A young infant undergoing balloon angioplasty for pulmonic stenosis developed global right renal infarction as a complication of cardiac catheterization via the femoral vein. A second patient developed segmental infarction of a recently transplanted kidney due to occlusion of a polar artery. Although routine renal sonography of these patients was normal, duplex Doppler sonography documented the absence of renal arterial and venous flow.

The clinical and radiographic findings of eight low-birth-weight neonates (mean, 900 g) with perforations of the duodenum or jejunum associated with transpyloric feedings are reported. In four patients, the perforations occurred distal to any known tube position. In experimental studies in young rabbits, Silastic or polyvinyl chloride tubes were sewn in place with the tube tips in the proximal duodenum or proximal jejunum, and either normal oral feedings or feedings through the tubes were given. Perforations and gross or microscopic abnormalities of the bowel mucosa in the area of the tubes and beyond were more frequent in rabbits with tube feedings than those given normal oral feedings. It appears that tube feedings induce some degree of mucosal damage and contribute to bowel perforation.

The identification of intracerebellar hemorrhage in a living premature infant by real-time ultrasound scan and confirmation of the findings at autopsy are described. This represents the first demonstration of the value of this noninvasive, convenient, and safe means of brain imaging in diagnosis of this lesion. Previous studies have described the role of the computed tomography (CT) scan in identification of intracerebellar hemorrhage in the newborn. Because infants with intracerebellar hemorrhage are usually critically ill, a means of identification of the lesion that could be utilized at the bedside rather than an approach that requires transport to a CT scanner is needed. This study indicates that portable real-time ultrasound scanning can satisfy that need.

A total of 334 nonreferred grade-school boys were examined for measures of physical growth, I.Q., and reading proficiency. SES was determined by the Hollingshead-Redlich index. Results of the study indicate: (1) for the total sample all physical measures significantly correlated with at least two of the three psychometric test scores; (2) SES alone accounted for 29 to 38% variance in psychometric scores; (3) mean scores of all psychometric and some physical variables significantly separated social classes: the higher the social class the greater the psychometric test scores (I.Q. and reading), HC, and height of the child; (4) holding constant SES decreased all correlations between intellectual performance and somatic growth; but (5) significant correlations remained between all physical measures and one or more psychometric scores; and (6) within individual social classes HC was the best physical predictor of WISC I.Q. and was significantly correlated with it in Classes I through IV.

One year's experience with MAS in a neonatal intensive-care unit is analyzed with follow-up information. Seventeen pationts or 3.7 per cent of all admissions had MAS. Four patients (23.5 per cent) died of acute respiratory failure. Two patients with MAS and persistence of the fetal circulation required cardiac catheterization to exclude cyanotic congenital heart disease. No survivors had persistent chronic lung disease. However, two of three patients with MAS and seizures had significant psychomotor retardation at follow-up examination.

Complications of cystography include infections, especially those associated with intrarenal reflux; complications related to bladder filling; allergic reactions; catheter problems; inflammatory response of the bladder to contrast media; peritoneal spillage of contrast medium via the vagina; transurethral reflux of organisms from cleansing procedures; pain from reflux or incorrect catheter placement; radiation effects; temporary anuria; and intrarenal foreign body reaction to contrast media. Fourteen cases are presented, including two patients who died from sepsis. Some considerations for reduction of the complications are discussed.

Surveys were sent to family physicians in Illinois to determine knowledge and attitude concerning optometry. The respondents were knowledgeable in certain aspects of optometry. However, many need to become more aware of the optometrist as a health care provider.

The clinical and radiographic features of paranasal sinus mucoceles in 6 pediatric patients are discussed. These lesions may be associated with clinical signs of a periorbital or intracranial mass. Characteristic radiographic findings can suggest the preoperative diagnosis, although a sphenoid sinus mucocele may simulate more serious conditions.

An 8-year-old patient with clinical features of mucolipidosis III (pseudo-Hurler polydystrophy) andbiochemical features of mucolipidosis II (I-cell disease) is presented. Clinical findings included short stature, slightly delayed psychomotor development, progressive joint stiffness beginning at the age of three years, moderately severe Hurler-like radiologic changes (dysostosis multiplex), and cardiac murmurs indicating aortic valve disease. Urinary mucopolysaccharides were normal. Biochemical findings in cultured fibroblasts included abnormal accumulation of 35 S-acid mucopolysaccharides, deficiencies for multiple acid hydrolases, and excessive levels of these enzymes in the cell culture medium. Phase microscopy of cultured fibroblasts revealed typical I-cell-like inclusions. The patient's serum showed 7 to 70 fold elevations of several acid hydrolases. Serum hydrolase measurements are recommended as the simples diagnostic test for multiple lysosomal hydrolase deficiency in any patient whose short stature, joint stiffness, and dysostosis multiplex suggest a Hurler-like condition but whose urinary mucopolysaccharides are not elevated.

Antral webs and diaphragms are considered a rare cause of gastric outlet obstruction in infants and children. A radiographic diagnosis of antral web was made in 28 children during a 26-mo period. Nonbilious, often projectile vomiting was the predominant presenting symptom in 24 infants who were less than 6 mo of age. The older children complained of pain, vomiting, fullness after eating, and eructation. The characteristic radiologic finging was a wirelike transverse septum 1--2 cm proximal to the pylorus. Poor antral filling was an important early clue in the radiographic search for the webs. Pyloroplasty was performed in 20 patients, frequently after failure of medical management of symptoms. Coexistent congenital abnormalities were present in 28% of patients. A search for environmental and familial factors has failed to elucidate any teratogenic influences in this population.

The incidence of malignant tumors in the primary immunodeficiency diseases is dramatically increased. Four patients with primary immunodeficiencies who developed fatal malignancies are reported. Lymphoreticular tumors and leukemia predominate in most conditions, but epithelial neoplasms are the most common tumors in selective Iga deficiency, and they comprise over one-fourth of malignancies in common variable immunodeficiency. With the exception of common variable immunodeficiency and the Wiskott-Aldrich syndrome, hyperplasia of lymphoid tissue usually does not occur. Lymph node enlargement in any of the other immunodeficiencies is therefore most likely secondary to malignancy. Benign gastrointestinal nodular lymphoid hyperplasia occurs frequently in common variable immunodeficiency and in some instances may be impossible to differentiate roentgenologically from lymphoma.

The Kniest syndrome is a newly defined chondrodystrophy consisting of disproportionate dwarfism with kyphoscoliosis, flat facies with prominent eyes, myopia, cleft palate, hearing loss and limited joint motion. As a result of a collaborative study of 12 patients, the roentgenographic features of this syndrome have been found to be diagnostic.The major roentgenographic features consist of:1. Flat, markedly elongated irregular vertebral bodies, which have superior-inferior defects in their midportion during infancy and early childhood.2. Dumbbell shaped long bones which in later life show epiphyseal ossification delay and irregularity, as well as expanded metaphyses with a cloud-like effect on both sides of the epiphyseal plate.3. Characteristic flattened and squared off epiphyses of the hands with joint space narrowing.Biopsies of costochondral junction and iliac crest reveal identical histologic and ultrastructural abnormalities which differ from all other chondrodystrophies. Throughout the cartilage are ...

Radiographic, surgical and pathological features of 33 paediatric patients with bronchogenic cysts are reviewed. These congenital lesions usually presented as spheroid mediastinal masses, near the carina or right paratracheal area, 2-3 cm in diameter with sharply delineated borders. Occasionally, the masses were lobulated or triangular in shape. Some cysts produced considerable airway distortion. All intrapulmonary cysts had connections, sometimes patent, with the trachea or main-stem bronchi. Thin-walled, completely aerated cysts became thick-walled with infection. A few air-filled and solid cysts grew rapidly. Cystic lung disease distal to central bronchogenic cysts was observed.

Because the efficiency of vitamin D absorption or hepatic uptake and 25-hydroxylation appears decreased in very premature infants, the routine use of 25-hydroxycholecalciferol (25-OHD3) supplementation has been suggested. Absorption studies of a 3 micrograms/kg orally administered dose of 25-OHD3 showed peak serum 25-hydroxyvitamin D2 and -vitamin D3 (25-OHD) concentrations at 4 to 8 hours similar in timing but of lesser magnitude to those seen in adults. Administration of 1 microgram/kg birth weight/day of 25-OHD3 corrected moderately low, but not very low serum (25-OHD) concentrations, and 2 micrograms/kg BW/day resulted in rapid and sustained increase in serum 25-OHD. Administration of 800 IU ergocalciferol (D2) also produced significantly higher serum 25-OHD concentrations than those in infants given 400 IU vitamin D2, but increases in serum 25-OHD were more gradual than in infants given 25-OHD3. In treatment trials with infants weighing less than 1500 gm, those given 800 IU D2, compared with those given 400 IU D2, had higher serum calcium concentrations and less frequent moderate or severe hypomineralization. Infants given 2 micrograms/kg BW 25-OHD3 had a significant increase in serum phosphorus values, but a decrease in serum calcium and magnesium concentrations, and parathyroid hormone also was suppressed to low normal values. The frequency of moderate to severe hypomineralization remained the same as in infants given 400 IU D2. In a subgroup of infants, serum 1,25-dihydroxyvitamin D was elevated over adult values, both in infants given 25-OHD3 (68.5 +/- 8.4 pg/ml) and in infants given vitamin D2 (60 +/- 6.7 pg/ml). Serum vitamin D concentrations were undetectable in four of six infants receiving 25-OHD3, but were elevated (5 to 31 ng/ml) in four infants receiving vitamin D2. Although 800 to 1000 IU D2 can be recommended as routine vitamin D supplementation in very premature infants fed standard formula, the use of 25-OHD3 requires further study.

The authors reviewed 49 double-contrast and 27 single-contrast barium-enema examinations (BE) in 71 children with suspected inflammatory bowel disease. The diagnosis was proved in 41 patients, of whom 36 underwent proctoscopy and 5 had advanced disease which was treated surgically within 3 to 30 days after BE; the other 30 children served as controls. BE and proctoscopy were compared with regard to sensitivity (detection of disease) and accuracy (categorization as ulcerative or granulomatous colitis). Sensitivity was 93% for double-contrast and 82% for single-contrast BE. Both detected all cases of advanced disease; in early disease, double-contrast BE detected 91% of cases and single-contrast BE 70%. Colitis was correctly categorized as ulcerative or granulomatous in 93% with double-contrast BE and 86% with single-contrast BE. Specificity within the control group was 100% with both techniques. The authors conclude that both types of BE exhibit similar sensitivity in advanced disease, but the double-contrast technique is more accurate in detecting early disease.

Prognostic criteria to be utilized in the selection of neonates with myelomeningoceles for early treatment have been proposed by Lorber.1 Stein et al. suggested modification of the selection factors based on the presence or absence of lacunar skull deformity (LSD) which they thought to be a good predictor of subsequent intellectual development.2 However, disagreement exists regarding the value of LSD as a useful criterion in early selection for treatment because Lonton et al. found only minor differences in intelligence among groups of patients with varying degrees of the deformity.3 In this report, a population of children with myelomeningoceles was studied in an effort to reach definite conclusions regarding the association between LSD and intelligence. See table in the PDF file

Is free intraperitoneal air in the postoperative patient a residual of the operative procedure, or does it indicate a perforation or disruption of an anastomosis? This is a problem that has often plagued both radiologists and surgeons for, generally, postoperative abdominal films are obtained only when a complication is suspected. A study by Bryant, Wiot, and Kloecker (2) showed that, in the adult, body habitus is the most important factor affecting the occurrence and duration of postoperative free air. They pointed out that the time required for disappearance of the residual air depended only on the amount initially trapped and was not influenced by peritonitis, type of surgical procedure, or any other factor except the presence of drains, along which, in rare instances, air entered the peritoneum. The obese patient tended to trap only small amounts of air initially and hence, almost invariably, the abdomen was free of air by the third day. The thin patient, on the other hand, often showed large amounts ...

The authors studied the histologic effects of subcutaneous and intramuscular injections of Conray 60%, Conray 400, Hypaque, and Renografin 60 alone and diluted with saline, water, procaine, and hyaluronidase. All four media produced an acute inflammatory response which was minimal at six hours and peaked between twenty-four and forty-eight hours. Chronic inflammatory cells, muscle atrophy, and scar formation were present at eight weeks. Despite clinical impressions, Renografin produced a heightened histologic reaction. Hyaluronidase appreciably increased the inflammatory reaction with more coagulation necrosis and scarring. No advantage resulted from diluting the medium with water or saline, or adding procaine.

Three cases of leukemia originally diagnosed by peripheral blood and bone marrow studies as aplastic anemia are reported.They had roentgenographic skeletal lesions, nonspecific of, but compatible with leukemia when initially seen. One patient also had enlarged kidneys with distorted calyceal anatomy from leukemia infiltrates. Fifteen true aplastic anemia patients had normal skeletal series.A roentgen survey of the skeleton and kidneys may be of value in differentiating true aplastic anemia from the aplastic phase of leukemia.The optimal site for skeletal biopsy can also be determined.

Three cases are reported in which the thymus gland was anomalously positioned, leading to confusion with pathologic mediastinal or neck masses.In one patient the thymus was lodged between the superior vena cava and ascending aorta and impinged upon the right mainstem bronchus, producing obstructive emphysema of the right middle lobe and right lower lobe. In another patient the thymus displaced the trachea anteriorly and the esophagus to the left. An incompletely descended thymus in the remaining child presented as a substernal mass which enlarged during expiration or when intrathoracic pressure was increased.A diagnostic trial of adrenocorticosteroids can be employed to help distinguish aberrantly positioned thymus from other mediastinal masses.

A classification of renal cysts is presented with a discussion of the clinical, pathologic anti roentgenologic features.

Arteriography of the gastrointestinal tract is still basically an experimental procedure. It has not assumed the role of peripheral arteriography in the study of tumors of the extremities (6, 9), of aortography in the evaluation of diseases of the kidney, or of carotid angiography in cerebral abnormalities (1). The explanation is simple and largely a technical one. It is difficult to inject selectively the superior (10) or inferior mesenteric arteries. When such an injection is performed, the resulting arteriogram shows only a maze of superimposed vessels coursing in all directions, following the many loops of the intestine. Fine differences in vascular patterns are not discernible. Previous studies by Margulis and Heinbecker (7) showed the value of operative mesenteric arteriography and demonstrated that it is possible to differentiate inflammatory from neoplastic and benign from malignant tumors of the stomach and colon. Arteriography offers advantages over conventional studies of the gastrointestinal t...

In 24 sacrococcygeal teratomas, three, or 13 per cent were malignant before the age of two. All three of the patients with malignancy had bowel or bladder dysfunction, and two of the three developed pulmonary and hepatic metastases. The operative mortality rate was less than four per cent. Survival rate in the benign tumors was 100 per cent. There were no survivors in the malignant group. Sacrococcygeal tumors in infants can usually be resected, even if extremely large, with appropriately aggressive surgery. Intraoperative monitoring is vital in these procedures. The coccyx from which these teratomas originate should be excised en bloc with the mass. Certain cases require a combined abdominosacral approach.

Kenny, Linarelli, and Caffey described an infant and mother with transient hypocalcemia, hyperphosphatemia, and unique skeletal abnormalities (5, 2). The syndrome was composed of proportionate dwarfism, skeletal maturation retardation, narrow long-bone shafts with stenosed medullary cavities, large anterior fontanelle and lack of differentiation of the calvaria into a diploic space and outer and inner tables. An unrelated infant with similar roentgen findings and convulsions secondary to hypocalcemia has been identified and is reported to substantiate this clinical entity. Case Report L. L. was born at term by precipitous delivery and suffered biparietal cephalhematomas. At seven days of age jerking movements of all extremities began. Each episode lasted several minutes, and there were repeated episodes each day. Following admission to St. Louis Children's Hospital at ten days of age, generalized seizures were observed and could not be controlled with phenobarbital and Dilantin. The calcium was 6.0 mg per...

THE CLINICAL and roentgenographic diagnosis of acute mesenteric vascular occlusion is difficult (2) and frequently delayed, with a resultant high mortality (1). A reliable method is required for the early detection and differentiation of mesenteric venous and arterial obstruction. The purpose of this paper is to report the angiographic findings in dogs subjected to superior mesenteric arteriography after experimental induction of thrombosis of the superior mesenteric vein. Material and Methods Seven mongrel dogs, each weighing 14 to 24 kg, were fasted for twelve hours and then anesthetized with 25 mg of intravenous sodium pentobarbital per kilogram of body weight. An endotracheal tube was inserted, and the animals were ventilated with a Harvard piston respirator (Model 607A). A celiotomy was performed, and in the first 3 dogs a small polyethylene catheter was introduced into the superior mesenteric artery from an arteriotomy in the adjacent aorta. In subsequent dogs the preferable technic was to pass a cu...

The addition of tannic acid to barium sulfate suspensions employed in the roentgenographic examination of the colon has been recommended by a number of authors (1, 5, 7, 12, 16) and its use is widespread. The rationale is based on the following considerations: Tannic acid as an astringent stimulates contracture of the colon, inhibits the secretion of mucin, and causes precipitation of the protein of the superficial layers of the mucosa. It also makes barium sulfate adhere to the bowel wall (5, 7, 16) and is said to inhibit absorption of substances from the colon (16). The concentration of tannic acid recommended for use in barium enema examinations varies between 0.25 and 3.0 per cent (16). A variety of tannic acids are found in nature. They are water-soluble polymers of various hydroxybenzoic acids (10). Tannic acid in the form of baths, dressings, or jellies was utilized in the treatment of cutaneous burns for almost twenty years before its use was abandoned. Clinical evidence of its hepatotoxicity accu...

Ingestion of iron-containing tablets ranks high as a cause of poisoning in children in the United States. In spite of various therapeutic regimens, the mortality rate approaches 50 per cent (1). As little as one gram may be fatal to a child (5). In children with iron intoxication vomiting, diarrhea, drowsiness, shock, and acidosis usually develop within thirty to sixty minutes after ingestion. Death follows within the first six hours in 20 per cent of the patients. In the remainder there is either gradual recovery or a period of apparent recovery lasting eight to sixteen hours, followed by shock, coma, convulsions, and death. One to two months later scarring of the stomach or bowel may cause obstruction (2). Abdominal roentgenograms have been recommended to establish the presence of iron in the gastrointestinal tract (3) and to determine the effectiveness of measures employed to remove it from the stomach and colon (3, 4). The following studies were undertaken to ascertain the roentgenographic appearance ...

In a previous communication (3), studies of the vascular patterns of five malignant tumors in mice were reported, disclosing that these neoplasms have specific and characteristic vascular arrangements. Growth studies of the tumors showed the basic vascular pattern to remain the same. When certain tumors became large, their growth rate slowed and necrosis became a prominent feature. Several neoplasms had identical angiograms despite transplantation into different strains of mice. With the above material as controls, experiments were designed to observe the vascularity and vascular patterns in the tumor and its bed by angiography. A study was undertaken to investigate: 1. Similarities or differences in the response of these tumors to irradiation, with determination of any correlation with radiosensitivity. 2. Serial changes in five tumors (sarcoma 37, hepatoma, lymphosarcoma, adenocarcinoma of the breast, and rhabdomyosarcoma) , following varying amounts of irradiation. This would include instances where ...

The value of sinus tract injection is discussed and illustrative cases are given.

A young infants is presented with a mass partially obstructing the distal stomach. Sonography demonstrated the superficial origin of the lobulated lesion which proved to be focal foveolar hyperplasia, the most common cause of a gastric polypoid mass in adults but rare in children.

Sinus histiocytosis with massive lymphadenopathy is an unusual disease characterized by prominent lymph node enlargement, especially in the cervical region. The clinical course is benign although prolonged, and no specific treatment is required. The etiology and pathogenesis are unknown. Four cases of sinus histiocytosis are reported, one in association with mediastinal lymph node enlargement and two in patients with orbital enlargement. One other patient had cervical masses. The radiologic features are reviewed to increase awareness of this entity as a cause of lymph node and orbital enlargement.

Case material was reviewed to determine the value of the rectosigmoid index in the diagnosis of both segmental and total-colon aganglionosis in infants less than one month old. Data indicate that a rectosigmoid index higher than 0.9 may rule out a diagnosis of rectosigmoid aganglionosis but may be a misleading indicator of longer segment or total-colon aganglionosis.

A young woman died following bronchography. The bilateral bronchogram done under general anesthesia without fluoroscopic control was associated with extensive small airway filling or "alveolarization" of the contrast medium. The oily Dionosil used had been heated in an autoclave. Experiments showed that the viscosity of oily Dionosil decreases with heating. The lowered viscosity could have contributed to the increased "alveolarization" of the contrast agent and death of the patient.

Pleural disease is an unusual manifestation of childhood leukemia. Three cases are presented which illustrate a definite association between leukemia and pleural thickening. In two patients, there was bilateral diffuse pleural thickening and, in the other, there were unilateral pleural-based masses. Pleural disease may be the initial or only thoracic manifestation of leukemia, or it may occur in combination with other intrathoracic evidence of leukemia.

A case of an obliterated, distal hypogastric (umbilical) artery partial1y obstructing the lower left ureter of a male infant is reported. Surgical resection of the offending vessel resulted in decreased uretero-pyelo-caliectasis. The urographic demonstration of a transverse filling defect of the distal ureter above the ureterovesical junction with some visualization of the ureter distal to the defect is the most valuable roentgenographic finding for ureteral obstruction secondary to pelvic blood vessels. The previously reported cases are reviewed and the differential diagnosis is discussed.

The histologic effects of 30% Cystokon, Hypaque 25%, and Renografin-30, placed into the bladders of rats, were studied. When 1 ml of contrast medium was injected by hand, a moderately severe inflammatory response was evoked which peaked at forty-eight hours and was still present at one week. Inflammatory reaction appreciably decreased when 1 ml of medium was administered by a gravity drip method or when 0.5 ml of medium was given. Dilution of the media by 50% also resulted in less inflammatory response by the bladder. There was little difference among the 3 media.

Radiographic findings in two patients with uncommon manifestations of epidermolysis bullosa dystrophica are described. A girl with recurrent urinary tract infections had scarring of the external genitalia producing chronic vaginal and uterine reflux and retention of urine. A second patient with long-standing stridor was found to have subglottic narrowing due to localized subglottic edema associated with an inflammatory membrane. The second case illustrates the occasional involvement of columnar epithelial surfaces which can occur in both epidermolysis bullosa dystrophica and epidermolysis bullosa hereditaria letalis.