Fine-needle aspiration cytology in children with superficial lymphadenopathy.

INTRODUCTION: In pediatric population Fine-Needle Aspiration Citology (FNAC) is slowly gaining acceptance in clinical management of Superficial Lymphadenopathy (SL). Our experience adds some data about the usefulness of this technique in diagnosing the cause of a SL and therefore guiding further treatment. PATIENTS AND METHODS: 238 FNAC were performed in 217 patients with SL, observed at our Institution from 2002 to 2006. The neck was the most frequent localization. The results were available within few hours. In cases of granulomatous findings, the samples were processed for microbiological and PCR test, in order to identify Mycobacteria. RESULTS: 174 were reactive lesions, 38 granulomatous lymphadenopathies, 24 malignant lesions, 2 specimens inadequate for diagnosis. Among the 174 reactive SL, 22 required an incisional biopsy after 1 month follow-up. Among the granulomatous lymphadenopathies, 13 children with Cat-Scratch Disease recovered, 25 with Mycobacteria infection underwent surgical excision. For 24 malignant lesions, the diagnosis was confirmed by further biopsy. Two false negative and no false positive were detected (sensitivity 92%, specificity 100%). No complications were encountered. CONCLUSIONS: In our experience FNAC, performed by experienced cytopathologist, has revealed to be a fast, safe, non invasive and inexpensive method to achieve diagnosis in persistent SL. The use of FNAC gave us the possibility to select patients for further investigation and/or surgical treatment. Incisional biopsy remains necessary to confirm the diagnosis in case of malignancy or doubtful lesions.

[Congenital and acquired chylothorax]. / Il Chilotorace congenito e acquisito.

BACKGROUND/PURPOSE: Chylothorax in paediatric age is a life-threatening clinical entity that cause serious respiratory, nutritional and immunologic complications. Chylothorax in the absence of trauma or tumour is uncommon and lymphangiomatosis of the bone, although extremely rare, has been associated with these condition. The authors describe the case of a two-year-old girl who presented with a massive chylothorax associated with hip and paravertebral lymphangioma and spread lymphangiomatosis of the spine. The authors also review the literature and their experience of congenital and postoperative chylothorax in order to establish guidelines for the diagnosis and management of both primary and postoperative chylothorax in paediatric age. METHODS: From 1990 and 1999, 14 children had chylothorax. 9 patients had pleural effusion after surgical procedure, 5 patients had congenital chylothorax (both in prenatal and neonatal time), one of whom with bone lymphangiomatosis associated. RESULTS: Postoperative chylothorax has been successfully treated by conservative approach (starvation, total parenteral nutrition and chest tube) in 6 out of 7 cases (two patients died because of complex cardiac malformation). Conservative approach is useful in case of congenital chylothorax, but not with bone lymphangiomatosis associated. CONCLUSIONS: Postoperative and congenital chylothorax is well managed with conservative treatment. Chylothorax with bone lymphangiomatosis associated needs early and aggressive surgical approach.

Lung volume reduction surgery in lieu of pneumonectomy in an infant with severe unilateral pulmonary interstitial emphysema.

A male infant with a prenatal diagnosis (at 20 weeks' gestation) of cystic adenomatoid malformation was delivered after 38 weeks' gestation (birth weight, 3 kg) and admitted to the neonatal intensive care unit. During the first few days of life, he developed mild respiratory distress; a chest radiograph and computed tomography scan showed multiple cystic areas in the left lower lobe with hyperinflation and herniation of the upper lobe across the midline. At 3 weeks of age, a left lower lobectomy was performed for presumed cystic malformation. To our surprise the pathology reports revealed pulmonary interstitial emphysema. The postoperative chest radiograph was unchanged, and mechanical ventilation was necessary and required progressively increasing ventilatory settings to provide adequate support. High-frequency oscillatory ventilation and selective right bronchus intubation failed to improve lung function. After 3 weeks, a left thoracotomy was repeated and lung volume reduction was performed with removal of 50' of the peripheral hyperinflated parenchyma. Postoperative recovery was rapid; the child was weaned from the ventilator after 3 days and discharged after 3 weeks. Follow-up chest X-rays showed a normally expanded right lung with mediastinal structures back to midline and a small left lung. Favorable results persisted at 3 years of follow-up. This first and successful experience with lung volume reduction in a neonate suggests that infants who need removal of a large portion of lung parenchyma to achieve adequate ventilation and gas exchange, lung volume reduction surgery should be considered as an alternative to pneumonectomy.

Is surgical treatment of lipoblastoma always necessary?

Lipoblastoma is an uncommon, benign mesenchymal tumor with an excellent prognosis despite its potential to local invasion and rapid growth. However, in the literature, a spontaneous resolution has never been reported, and, consequently, the need for a complete surgical excision has never been questioned. The authors report a case of a 2-day-old boy with congenital diffuse lipoblastoma in the left thigh, which forced us to withhold from surgical treatment to avoid the risk of mutilation in a patient so young. The lesion was followed-up by imaging, and a complete spontaneous resolution of the diffuse lipoblastoma was shown by magnetic resonance imaging (MRI) at 1-year follow-up. In the literature, a complete surgical excision is recommended. The results of this case suggest that a "wait and see" approach is justified at least in infants with huge invasive lesions requiring a mutilating excision.

Inflammatory pseudotumors of the lung in children: conservative or aggressive approach?

BACKGROUND: Inflammatory pseudotumors (inflammatory myofibroblastic tumors) of the lung are myofibroblastic lesions of controversial nosology. The concept that these are benign lesions has been recently questioned given their capacity for local invasion and recurrence. PROCEDURE AND RESULTS: We observed five children with inflammatory pseudotumors of the lung in whom the tumor was resected using the most limited excision possible. Unusually, in all five cases the tumor was localized in the right upper lobe: Three were parenchymal, and the others involved the bronchus. The three parenchymal masses underwent wedge resection, whereas the two bronchial lesions required ex-cision followed by a bronchoplasty and an up-per lobectomy, respectively. In all, the pathology showed a complete resection, and the patients had uneventful recoveries. Follow-ups, including CT and bronchoscopies, ranged from 4 to 8 years (mean of 6.4 years) with no signs of recurrence. CONCLUSIONS: Our limited experience supports the idea that excisional surgery is the treatment of choice for inflammatory pseudotumors of the lung. Such excisions, both diagnostic and curative, carry minimal risks and avoid unnecessary mutilation. Closely monitored follow-up is mandatory, as the natural history of this lesion is not yet well understood.

Renal angiomyolipoma in children: diagnositc difficulty in 3 patients.

PURPOSE: Because angiomyolipoma is less common in children than in adults, its diagnosis can be difficult. We present 3 cases of pediatric angiomyolipoma in which diagnostic problems resulted due to the presenting characteristics. MATERIALS AND METHODS: We report on 3 children with unilateral renal angiomyolipoma. Computerized tomography (CT) and ultrasonography revealed 3 large renal masses, 20, 7 and 8 cm. in diameter, respectively. A correct diagnosis was not made preoperatively in any case by CT, ultrasound or fine needle biopsy. Wilms tumor was suspected in the first patient who received preoperative chemotherapy. Imaging was inconclusive in the other 2 cases. RESULTS: All patients underwent surgical exploration and subsequent nephrectomy due to the large size of the tumor. At followup 33, 23 and 13 months postoperatively all children were well without signs of recurrence. CONCLUSIONS: It has been reported that the demonstration of fat on renal ultrasound and CT can diagnose angiomyolipoma in 95% of the cases. Most radiologists rely solely on CT demonstration of lipid density in the renal mass to diagnose angiomyolipoma but the identification at imaging of lipid tissue may be difficult in small tumors. In our cases the fat content of the tumors was less than 10% despite the large size. This low fat content results in misdiagnosis, since fatty tissue is also present in other renal tumors, such as lipoma, liposarcoma, teratoma and Wilms tumors. We recommend conservative surgery when tumor size permits in pediatric patients with angiomyolipoma to avoid chemotherapy.

Endoscopic dilation of acquired tracheobronchial stenosis in infants.

Acquired tracheobronchial strictures in infants are associated with significant morbidity. Their treatment requires different types of therapeutic procedures depending on the severity and location of the stenosis. We successfully dilated five children (ages between 1 and 23 months) with acquired tracheobronchial (stenoses two carinal and three of the mainstem bronchi) using a new technique. Gruentzig balloon catheters of different caliber were introduced into the tracheal lumen of apneic children under endoscopic vision. Three infants required four dilations and in the other two cases only two procedures were necessary. One complication occurred during dilation and resulted in a pneumothorax caused by a partial-bronchial disruption. At follow-up (range, 18-36 months; mean, 23.4 months) all five children were clinically improved. In the four cases without complications the chest x-rays were normal and the last bronchoscopy showed tracheobronchial trees with normal calibers. The child who developed a pneumothorax during attempted dilation recovered. In this child, dilation of the right upper lobe bronchus was unsuccessful. Dilation was attempted without visualization of the bronchus and the stricture. Our limited experience confirms that pneumatic balloon catheters can successfully treat acquired tracheobronchial stenoses in infants. To avoid complications, strict criteria need to be applied in the selection of cases with tracheobronchial stenoses.

The safety of home tracheostomy care for children.

Home tracheostomy care avoids prolonged hospitalization for tracheostomized children who must remain cannulated for a long time; however the safety aspects of this form of management are still under discussion. Since 1987 the authors have tutored parents in tracheostomy care. Once parental training was complete and the necessary equipment (aspirator, humidifier, etc) provided, children in stable condition were discharged and had period follow-up as outpatients. Thirty-four children had a total of 710 months in home care; 11 of them had decannulation. The only reported complications were two partial obstructions of the cannula and two accidental decannulations. The low number of documented accidents and the parents' profound appreciation in having their children at home indicate that patients with a tracheostomy in need of long-term care can be safely managed at home.