Bronchial compression by dilated pulmonary artery. Surgical treatment.

Bronchial compression by a dilated left pulmonary artery complicated the postoperative course in two infants: one after a Mustard operation for transposition of the great arteries, restrictive ventricular septal defect, and moderate pulmonary stenosis, the second after correction of tetralogy of Fallot with absent pulmonary valve. In both cases the bronchial compression was successfully treated by division of the left pulmonary artery and its prolongation by interposition of a conduit.

Tomography in the pre-operative evaluation of ear malformations. Report of 47 cases, 58 ears.

We have studied 47 children (58 ears) with external and middle-ear malformations. Tomography was useful and effective in the pre-operative evaluation of anatomical patterns. We have divided our cases into four groups and described the most characteristic tomographic findings in each. We also discuss problems concerning patients immobilization and X-ray protection.

[Celiac disease: correlation of different diagnostic methods (author's transl)]. / La malattia celiaca: valutazione comparativa delle varie metodiche diagnostiche.

The results of four diagnostic tests have been critically evaluated (xylosemia, serum Ig A level, steatorrhea, X-Ray of the digestic tract) in 80 cases of atrophy of the small intestinal mucosa discovered by biopsy, and in 20 cases in which the biopsy has proved normal. In the celiac subjects the best correlation with the biopsy was found with xylosemia (90%), followed by serum Ig A (87%), by X-Ray of the digestive tract (86%) and finally by steatorrhea (59%). Even in cases without histological alteration of the mucosa, and xylosemia proved abnormal in 70% of the cases, the seric IgA resulted higher greater than 2 delta in 35%, the steatorrhea in 29% while the X-Ray of the digestive tract has never given a false diagnosis. The presence of false negative or positive results in all four tests (except the radiological tests) reconfirms the absolute necessity for biopsy of the digestive tract in the diagnosis of celiac disorder, and has led the AA. Carry out whenever possible the xylosemia, the steatorrhea and the serum IgA level because in none of the 80 cases of celiac disorder has there been a false negative result in all three such tests at the same time.

[A new (?) multiple abnormality complex similar to the Poland syndrome]. / Nuovo (?) complesso malformativo multiplo simile alla sindrome di Poland.

The report describes a case Poland's Syndrome - like in which the hypoplasia of the left major pectoralis muscle was associated with: subtotal aplasia of sternal body, severe hypoplasia of the ipsilateral scapula, humerus and forearm bones, tetradactyly in the ipsilateral hand, partial defect of the diaphragm with thoracic migration of the left hepatic lobe and secondary dextrocardia (false dextrocardia). Syndactyly was absent. The contribution is aimed to join out the radiological characteristics of the syndrome as well as the specific contribution provided in the case by Ultrasounds and aortography. Furthermore, the analysis of the case seems to indicate a strict relationship between the abnormalities of diaphragm, pectoralis muscle and homolateral upper limb.

[Mesomelic dwarfism. Pronounced form in a female infant of a father with dyschondrosteosis and a mother with Madelung's deformity]. / Il nanismo mesomelico. Forma conclamata in bambina nata da padre con discondrosteosi e da madre con deformità di Madelung.

A serious form of mesomelic dwarfism was observed in a 4 1/2-month-old girl whose parents (first cousins) presented dyschondrosteosis and Madelung's deformity respectively. The radiological findings in all three subjects are discussed. It is suggested that the three morbid forms are expressions of the some genotypical alteration, probably at the homozygote status of mesomelic dwarfism.

[2 cases of bronchiectasis caused by an undiagnosed endobronchial foreign body]. / Due casi di bronchiettasie da corpo estraneo misconosciuto endobronchiale.

Two cases in which an unknown foreign body gave rise to bronchiectasia are culled from a series of 54 cases of endobronchial foreign bodies in children and discussed.

Post traumatic leptomeningeal cysts in infancy.

Post traumatic leptomeningeal cysts (P.T.L.C.) are capsulated liquor collections in the sub-arachnoidal space consequent to skull fractures. They occur typically in infancy and their deceitful onset needs an accurate X-ray follow-up in children with a history of cranial trauma. The authors present six cases of P.T.L.C. encountered in the last three years, recall the mechanism of formation, and stress the importance of standard radiological examination, and computed tomography for early diagnosis.

A case of a thoracic vertebral body dislocation without neurological signs in a child with neurofibromatosis.

In a 10-year-old child with neurofibromatosis, dislocation of a thoracic vertebral body was noted almost by accident; a singular dislocation unaccompanied by neurological symptoms.

Constitutional osteochondrodysplasias identifiable at birth. A short review on the state of the art in radiodiagnosis in the late 20th century.

INTRODUCTION: The value of a systematic radiologic analysis in constitutional osteochondrodysplasias remains underestimated by both neonatologists and radiologists. We report the clinical experience of the Department of Neonatology and Neonatal Intensive Care Unit of St. John Hospital in Rome with constitutional osteochondrodysplasias identifiable at birth. MATERIAL AND METHODS: We reviewed 2120 cases of newborns hospitalized in our unit from January 1996 to August 1998 and here submitted to at least one direct radiograph of chest and abdomen (the so-called "babygram"). All the newborns were clinically assisted by the same three neonatologists and radiologically followed by the only pediatric radiologist, the external consultant for diagnostic imaging of the Intensive Care Unit of the Neonatology Department. RESULTS, DISCUSSION AND CONCLUSIONS: We diagnosed 14 cases of constitutional osteochondrodysplasias (.66%): 4 of them (28.27%) belong to the group considered by the European Society of Pediatric Radiology (ESPR) as lethal before or immediately after birth, while the other 10 (71.43%) belong to different groups of the ESPR classification. Thus, we arbitrarily grouped them into a single pathologic condition, based on their two main features: being generally not lethal and always or very often identifiable at birth. These 10 cases were: 1 campomelic dysplasia, 3 achondroplasias, 2 asphyxiant thoracic dysplasias type Jeune, 1 cherubinic dysplasia, 2 osteogenesis imperfectae, 1 osteopetrosis. We justify the relatively high incidence of constitutional osteochondrodysplasias in our study (.66% versus an average incidence of .076% reported in the world population) on the basis of: a) an increasing number of high-risk newborns in our intensive care unit; b) an improvement in our clinical and radiologic diagnostic skills. We conclude that the state of the art of the diagnosis of constitutional osteochondrodysplasias is still based on the first plain X-ray examination performed at birth because of cardiorespiratory and/or abdominal diseases in the newborn.

Intestinal perforation in newborn following intrauterine meconium peritonitis.

A newborn infant, who had suffered intrauterine perforation and had developed peritoneal calcification, showed no sign of pneumoperitoneum on the first radiograph taken 3 hours after delivery. At that time air was present in the stomach only. Subsequently pneumoperitoneum developed as air passed down the gut to the point of perforation. Thus, the absence of pneumoperitoneum on a radiograph taken very early in life does not exclude an intestinal perforation.

Ultrastructural aspects of chondrodystrophia calcificans congenita (syndrome of Conradi-Hünermann).

An ultrastructural study of chondrodystrophia calcificans congenita is reported. Foci of initial calcification of cartilage are characterized by coexistence of three different types of crystals, probably due to abnormal proteoglycan composition of cartilage matrix. The calcification process in chondrodystrophia calcificans congenita is apparently not related to 'matrix vesicles' as it is in normal cartilage.

[Rib notchings in Cooley's anemia]. / Incisure costali nella anemia di Cooley.

In 3 cases of Cooley's anemia rib notchings were found. In the first case the clinical pattern did not allow to exclude postductal type of aortic coarctation and thus was performed a venous digital aortography (SVC) showing the integrity of the aortic arch and the absence of intercostal arterial collateral circulation. Only two previous descriptions of rib notchings in thalassemia were found in literature, and the authors shortly describe the 3 observed cases and suggest some possible pathogenetic interpretations either of rib notchings in thalassemia or of the relatively late appearance (adolescence) of rib notchings.

[High-resolution computed tomography in the diagnosis of branchial otodysplasias. A report on 10 cases (15 ears)]. / La tomografia computerizzata ad alta risoluzione nella diagnostica delle otodisplasie branchiali. Rapporto su 10 casi (15 orecchi).

Computed Tomography (CT) is not yet widely employed in the evaluation of temporal bone diseases in pediatric patients. Our experience is reported in the study of branchial otodysplasias (10 cases, 15 ears) by means of a high-resolution program. Indications, advantages, limitations and risks of this technique are discussed. Some technical details are reported, such as number and type of scans, length of data acquisition, thickness and feed of slices, electronic image reconstruction, and radiation doses given to the lens. The method employed to immobilize younger patients is also described. CT findings in the single cases are summarized in the tables. The most significant images of 5 cases are also reproduced. A careful analysis of the cases in our series highlights the advantages provided by the use of high-resolution CT in the evaluation of the temporal bone in pediatric patients, especially as far as dysplasias are concerned.

Mucolipidosis II: correlation between radiological features and histopathology of the bones.

Twelve cases of Mucolipidosis II (I-cell disease) with a wide range of severity of skeletal involvement were studied. Pathological findings in two cases provided helpful information in understanding the radiographic features of dysostosis multiplex. Inhibition of the growth plate cartilage calcification and rickets-like lesions were observed in the metaphyses. Enhanced subperiosteal remodelling and paratrabecular fibrosis were also evident in the diaphyses. High levels of parathormone were found in one case. This finding supports the hypothesis that bone lesions may be secondary, at least in part, to damage in such viscera as the kidney and/or the liver and that they are mediated by vitamin D and parathormone.