Cystic adenomatoid malformation in children: CT histopathological correlation.

PURPOSE: This study was performed to assess the accuracy of computed tomography (CT) in classifying the various types of cystic adenomatoid malformation (CAM) of the lung, as described by Stocker et al., taking histopathology as the gold standard. MATERIALS AND METHODS: We retrospectively reviewed six cases of histologically proven CAM. Chest radiography, chest CT and histopathology results were available for all patients. The CT images were reviewed blinded to the histological findings, and attention was paid to the number and size of cysts so as to classify the lesions into the three groups described by Stocker et al. The classification of lesions based on the CT images was then correlated to the histopathological findings. RESULTS: Areas with small-sized cysts (<2 cm) were detected by CT in two patients (33.3%), areas with large cysts (>2 cm) were seen in three cases (50%) whereas in the remaining case, the diagnosis was mixed type I and type II CAM. In one patient with type I CAM, an area of low-density consolidation around the cysts was interpreted as CAM in a context of pulmonary sequestration. The CT classification based on Stocker et al.'s categories was in agreement with the histopathological findings in four cases, whereas in the remaining two cases, the lesions were classed as type I or II on CT and as mixed (type I and II) lesions at histopathology. In one case, the CT classification was correct, but the histopathology revealed the coexistence of pulmonary sequestration. CONCLUSIONS: In our study, there was concordance between CT and histopathology in 66.7% of cases, whereas in 33.3% histopathology revealed areas with mixed grade lesions. CT proved to be accurate in identifying and characterising CAM and provided important information on lesion site and extension.

Haemothorax caused by isolated costal exostosis.

We report an unusual case of spontaneous haemothorax in a 13-year-old girl with isolated costal exostosis. Surgical excision of the exostosis was performed with complete resolution. Costal exostosis should be considered in the differential diagnosis of spontaneous haemothorax in children in order to avoid unnecessary investigation and to establish an adequate treatment plan.

[Ocular ultrasonography in pediatrics: persistence of hyperplastic primary vitreous]. / Ecografia oculare in pediatria: persistenza del vitreo primitivo iperplastico.

AIM OF THE STUDY: To assess the diagnostic viability of ultrasound and color Doppler ultrasound in a particular segment of paediatric ophthalmology-persistent primary hyperplastic vitreous, in the presence of leucocoria, retrospectively evaluated in patients observed over the last two years. MATERIAL AND METHODS: We re-evaluated four patients (two new-born, one unweaned and one nine-years-old) who at ophthalmoscopic examination were suspected having persistent primitive hyperplastic vitreous. The follow-up included an ultrasound examination, basic color-Doppler ultrasound and re-evaluation during sedation both using an operative microscope and ultrasound. The examinations were performed with linear 7.5 Mhz probes and a Doppler frequency of 3.7 Mhz, while the sedation examination was performed with an anular 13 Mhz probe. The scans were sagittal and axial to correctly localize the lesion with respect to the lens, to ciliary bodies and to the optic nerve head. RESULTS: In the first patient ultrasound revealed a hyperechogenic inhomogeneous structure bilaterally in the vitreous structure; this extended from the posterior wall of the lens to the optic nerve head and retina, and was found to be highly vascolarized at the subsequent color-Doppler ultrasound. In the second patient there was an echogenic band extending from the posterior wall of the lens to the optic nerve head and to the retina, as the fourth patient showed a series of echogenic bands extending from the temporal ciliar of bodies to the temporal retina; in both cases no significant vascolarization was found by color Doppler ultrasound. In the third patient ultrasound showed a lesion involving nearly all of the vitreous body, with inhomogeneous structure with small calcifications and intense vascolarization. DISCUSSION AND CONCLUSIONS: Ultrasound supplies essential information for the diagnosis of persistent hyperplastic primary vitreous as it determines the presence of the lesion, its extension and retinal and optic nerve head involvement. As confirmed by operative microscopy the first patient had bilateral lesions involving the optic nerve head, as well as the retina, the latter which appeared raised. The second and fourth patients had typically posterior lesions; the lesion of the third patient was difficult to interpret even by operative microscopy. To conclude,Ultrasound proved to have an optimal sensitivity and specificity for precisely locating the site and extension of the lesion. By contrast, the difficulties and need for sedation with color Doppler ultrasound unlikely to be useful with paediatric patients.

[Percutaneous renal biopsy in children: use of a fine needle]. / Biopsia renale percutanea in età pediatrica: uso di un ago sottile.

Percutaneous renal biopsies were performed on native kidneys in 40 children and adolescent, aged 1.7-13 (mean 6.7) years. Bleeding diatheses were excluded by the determination of Hb, blood platelets, PT, PTI and fibrinogen. Biopsies were performed under ultrasound imaging, using a semiautomated and thin needle (20 gauge in children with age under 5 years and 18 gauge for those over 5 years). All the patients were lightly sedated, except for 3 ones who received a general anesthesia. Diagnostically adequate tissue was retrieved in 38 of 40 biopsy procedures (95%). A macro-haematuria was observed with elimination of haematic coagula in 3 children (7.5%) and 24-h post-biopsy ultrasonography disciosed a small haematoma of the biopsied kidney in 2 one (5%). No mayor complications occurred. We conclude that the use of ultrasound imaging and a semiautomated needle is a safe and efficient method for performing renal biopsies in paediatric patients. The use of smaller (18 or 20-gauge) cutting needles would reduce the complications rate while allowing retrieval of sufficient tissue for histologic diagnosis.

Child with oral, facial, digital, and skeletal anomalies and psychomotor delay: a new OFDS form?

We report on a male infant with oral, facial, digital, and skeletal anomalies in association with severe psychomotor delay. This may represent a new oral-facial-digital syndrome.

[Neonatal hemoperitoneum: role of ultrasonography. Experience with 4 cases]. / Emoperitoneo neonatale: ruolo dell'ecografia. Esperienza in quattro casi.

This study was aimed at investigating the role of US in the newborn with a clinical suspicion of hemoperitoneum. Hemoperitoneum is a dramatic clinical event which may be caused by some obstetrical and fetal factors--breech or complicated delivery, hepatomegaly, macrosomy, anoxic liver congestion being the most frequent causes. Direct or indirect trauma of an abdominal organ during delivery represents the major pathogenetic factor. Clinical symptoms are correlated with the appearance of shock whose intensity is proportional to trauma severity: symptoms may appear even 48 hours after delivery. The authors report their experience with 4 consecutive cases of neonatal hemoperitoneum which underwent US of the abdomen 24 to 72 hours after birth. In the first 3 newborns, US was performed on the basis of a clinical picture of bleeding shock, while in the extant newborn US was performed after a sudden increase in blood transaminase level associated with an obstetrical paralysis of the left arm. The US examinations were performed using a 7.5-MHz sectorial probe unit (Siemens). In all patients, US demonstrated the presence of a liquid effusion in the abdomen which could be referred to hemoperitoneum. In two patients, it was associated with a traumatic focal alteration of the liver parenchyma, in the third with a diffuse subcapsular liver hematoma and, finally in the fourth patient with a disconnected splenic vascular pedicle which was at surgery demonstrated. In conclusion, US is indicated as the most reliable and valuable diagnostic technique in the study of neonatal hemoperitoneum. The authors believe abdominal US to be essential in all the neonatal cases where hemoperitoneum is suspected and in selected cases presenting specific risk factors, to allow a rapid diagnosis and better treatment planning.

Respiratory distress in a newborn with primary ciliary dyskinesia, situs inversus and Turner syndrome.

A report is given of a newborn girl with situs inversus and Turner syndrome that presented respiratory distress. The patient had a mosaic karyotype 45,X/46,X + mar (80%/20%). Ciliary motion analysis demonstrated a total absence of ciliary motion whereas, ultrastructural studies revealed typical features of primary ciliary dyskinesia (PCD) (absence or short outer/inner dynein arms in 90% of the cilia). We regard this rare combination (PCD, situs inversus and Turner syndrome) as a coincidental occurrence.

Successful interferon gamma therapy in a patient with X-linked chronic granulomatous disease, McLeod syndrome and hyper-IgE. Case report.

Recent studies have shown clinical benefit resulting from recombinant interferon gamma (rIFN-gamma) therapy in patients affected by chronic granulomatous disease (CGD), which represents an important adjunct to conventional therapy. In order to evaluate the effect of interferon gamma therapy, we investigated clinical and haematological parameters in a child with X-linked CGD, McLeod phenotype (kell negative) and hyper-IgE, before and after 8 months of therapy. Our results show no significant effect of rIFN-gamma on the respiratory burst of peripheral polymorphonuclear leukocytes. This notwithstanding, we observed improved clinical and haematological conditions. These results support the view that interferon gamma may benefit these subjects by influencing oxygen-independent antimicrobial activity or other immunological parameters.

[Critical analysis of the diagnostic imaging of focal hepatic lesions]. / Analisi critica della diagnostica per immagini delle lesioni epatiche focali.

The aim of this paper is to evaluate the number and type of consecutive initial diagnoses of focal hepatic lesions obtained by abdominal US and CT. The diagnoses were coded according to the Index for Radiological Diagnoses (ACR). From January 1990 through April 1991, US and CT diagnosed focal hepatic lesion in 16.4% and 9.1% of cases, respectively. A lower number of focal hepatic lesions was diagnosed by CT, which however was more accurate as to the nature of the lesion itself. This is due to the fact that CT is often used to stage hepatic neoplasms already confirmed by US-guided fine-needle biopsy (FBN). The correlation between the initial diagnosis and actual clinical status demonstrates a high rate of occasional findings, especially with US. The rate of questionable diagnoses relative to primary or secondary malignancies was very high. This could be explained by caution in making a severe diagnosis, by awareness of the limits of macroscopic diagnostic techniques and by the immediate availability of US-guided FNB. In conclusion, the coding of consecutive initial diagnoses, by US and CT, could contribute to a practical evaluation of diagnostic imaging techniques, especially when correlated with the respective anamnestic and clinical data. Further studies are necessary to confirm this hypothesis.

[Gastromiro vs gastrografin vs prontobario CAT for computerized tomography. Multicenter study]. / Gastromiro vs gastrografin vs Prontobario TAC per Tomografia Computerizzata. Studio multicentrico.

The opacification of intestinal loops is mandatory, during CT examination of the abdomen, to prevent the gut from being mistaken for a pathological process or vice versa. The authors compared Gastrografin and Prontobarium-CT with a new, orally administrable, non-ionic contrast medium (Gastromiro) to investigate whether the non-ionic molecule presents any advantages over the other contrast media usually employed with CT. Five hundred ml of water solution/suspension at 11.1% (Gastromiro or Gastrografin) and 1.7% (Prontobarium-CT) were administered 20 minutes before examination to 818 consecutive patients. Different parameters were considered--i.e., patient tolerance as far as "taste and vomiting" were concerned, gut opacification, and any reaction which might have occurred to the patients within 24 hours after examination. The results, statistically analyzed, are the following: no significant difference in the quality of opacification of stomach, duodenum, and large bowel; significant difference in the quality of opacification of small bowel, where Gastromiro proved to have the highest contrast resolution; Gatromiro stimulated diarrhea significantly less than Gastrografin and Prontobarium-CT.

Rotational disorders in intramedullary fixation of the lower limbs.

The routine employment of intramedullary fixation in fractures of the femur and tibia has led the authors to thoroughly evaluate malunions. With the aid of CT scan, precise multilevel quantification of residual rotational deformity was possible by comparison with the contralateral limb after fracture healing. The results of this study confirm the effectiveness of the method, filling an interpretative gap in the literature.

[Mercury dichloride in lung pathology. Study of 241 patients]. / Il bicloruro di mercurio nella patologia del polmone. Studio su 241 pazienti.

In 241 patients suffering from various of lung pathology and subjected to lung scanning with mercury bichloride (Hg 197), it was found that 90% of the neoplastic masses pick up the isotope in significant fashion; 40% of the masses of other nature also pick up the compound. This results means that scanning with radioactive mercury bichloride is of little diagnostic usefulness.

[Normal values of T3-index and of thyroxine (T4) (author's transl)]. / Valori normali del T3-indice e della tiroxina (T4).

The normal values of T3-index and of thyroxine (T4) have been evaluated comparing a binomial distribution to frequential curve of all the values obtained. The findings are: T3-index = 89 divided by 112; T4 = 3.7 divided by 15.3 microgram/100 cm3 of serum.