Paolo Dodero

A 14-yr-old male with dyspnoea, productive cough and chest pain

A 14-yr-old White male was referred to the Urology Unit of the G. Gaslini Institute because of a post-traumatic urethral stenosis (arising from a bicycle accident) that, during the previous 18 months, had required repeated endoscopic urethral dilation manoeuvres at the patients local hospital. Apart from the urethral stenosis, the patient had been in excellent health until 2 months before admission, when slowly progressive exertional dyspnoea, associated with nonproductive cough and right-sided posterior chest pain, developed. On admission, the patient appeared in good clinical condition. Decreased percussion and auscultatory sounds were noted over the middle and lower portions of the right hemithorax. A summary of the results of the blood tests performed on admission is shown in table 1⇓. View this table: Table 1 Results of blood tests performed on admission Chest radiography (fig. 1⇓) and high-resolution computed tomography (HRCT) (fig. 2⇓) were performed. Fig. 1.— Chest radiograph. Fig. 2.— High-resolution computed tomography of the chest at two different window settings: a) “parenchymal”; and b) “soft tissue”. Thoracocentesis was performed and 500 mL haemorrhagic pleural fluid aspirated. Pleural fluid analysis did not show any cytological changes suggesting malignancies, amylase levels were within the normal range and microbiological evaluation results were negative for bacteria, fungi, mycoplasmata, mycobacteria and viruses. Plasma d‐dimer levels were slightly elevated but ultrasonography of the deep venous system did not show signs of thrombosis in the legs, penis or pelvis. Echocardiographic evaluation did not demonstrate any right ventricular dysfunction. Ultrasonographic examinations showed that there were no abnormalities of the abdominal organs and no peritoneal effusion and ruled out the presence of testicular or thyroid tumours. Fibreoptic bronchoscopy was then performed and did not reveal any airway abnormalities. Bronchoalveolar lavage analysis was nondiagnostic (no siderocytes suggestive of pulmonary haemorrhage, acid-fast bacilli, bacteria, viruses, fungi or malignant cells were identified in the epithelial lining fluid or lavage fluid …

Results of a multicenter retrospective study of a combined medical and surgical approach to pulmonary aspergillosis in pediatric neutropenic patients

Invasive aspergillosis (IA) is a serious problem in patients suffering from hematological malignancies. Surgical resection has been reported to improve disease control and patient survival. There are few reports describing the role of surgery in children with pulmonary IA.

Uneventful outcome of unrelated hematopoietic stem cell transplantation in a patient with leukemic transformation of Kostmann syndrome and long-lasting invasive pulmonary mycosis

Abstract: Kostmann syndrome (KS) is an inherited hematological disorder characterized by an absolute neutrophil count (ANC) <0.2 × 109/L and life‐threatening bacterial infections. Granulocyte‐colony stimulating factor (G‐CSF) makes it possible to reach an ANC of 1.0 × 109/L and consequently to reduce significantly the occurrence of severe infections. Absence of response to G‐CSF, G‐CSF receptor mutation, and leukemic transformation are absolute indications to perform hematopoietic stem cell transplantation (HSCT). Pulmonary mycosis does not represent an absolute contraindication to bone marrow transplantation (BMT), although a relapse rate of 30–50% has been reported, despite adequate medical and surgical treatment. Mycotic pneumonia recurrence shows a mortality rate above 80%, especially in the presence of persisting immunosuppression. We report on a KS patient with long‐lasting fungal pneumonia who developed myelodysplasia and subsequent acute myeliod leukemia (AML) conversion resistant to antiblastic therapy. Despite surgical excision and secondary prophylaxis, recurrence of the pulmonary lesion occurred prior to the unrelated HSCT. In spite of these poor prognostic characteristics, outcome was uneventful and the patient is alive and well in continuous complete remission with no signs of fungal infection.

Eosinophilic chromophobe renal cell carcinoma in a child with hypospadias

Chromophobe renal cell carcinoma (CRCC) is a distinct variant of renal carcinoma generally affecting adults. We report a case of an unusual CRCC, arising in a male child affected by hypospadias. This case demonstrates that CRCC can occur in the pediatric patients and can be associated with genital tract anomalies such as Wilms tumor. Pediatr Blood Cancer 2008;50:413–415.

Images in Anesthesia: Congenital tracheal stenosis in a boy with Rubinstein-Taybi syndrome

RUBINSTEIN-TAYBI syndrome (RTS) is a complex entity, characterized by facial changes, broad thumbs and great toes, post natal growth retardation, cognitive defects and multiple malformations.1 No airway abnormalities are described in RTS other than post-cricoid web2 and tracheoesophageal compression due to vascular ring.3 However, because craniofacial changes often include micrognathia, problems with intubation during anesthesia may occur with great frequency.4,5 We report the first case of congenital tracheal stenosis in RTS. A 46-month-old Caucasian male, diagnosed soon after birth as being affected by RTS, was admitted for surgical correction of bilateral cryptorchidism. At admission, the characteristic face and the typical thumb and toe changes were observed. Laparoscopy followed by orchiopexy was planned, and because of the presence of a high arched palate and marked hypoplastic maxilla, difficulties in intubation were expected. While no problems in passing the vocal cord with the tracheal tube were encountered, an unpredictable resistance thereafter was found. Fibreoptic bronchoscopy was performed and a severe concentric tracheal stenosis with complete cartilaginous O-rings was detected, whose characteristics were better defined by spiral computed tomography scan three-dimensional reconstruction (Figure). No vascular rings or other great vessel abnormalities were detected. CARDIOTHORACIC ANESTHESIA, RESPIRATI N A D AIRWAY