Tracheostomy, respiratory support, and developmental outcomes in neonates with severe lung diseases: Retrospective study in one center.

OBJECTIVES: Pediatric tracheostomy has evolved significantly in the past few decades and the optimal timing to perform it in children with respiratory assistance is still debated. The objective of this study was to describe the indications, timing, complications, and outcomes of infants on respiratory support who had a tracheostomy in a tertiary pediatric intensive care unit (PICU). METHODS: All children younger than 18 months of corrected age requiring respiratory support for at least 1 week and who had a tracheostomy between January 2005 and December 2015 were included. Their demographic and clinical data and their outcomes at 24 months of corrected age were collected and analyzed after approval from the CHU Sainte-Justine ethics committee. RESULTS: During the study period, 18 children (14 preterm infants, 4 polymalformative syndromes, and 2 diaphragmatic hernias) were included. The median corrected age at tracheostomy was 97 days (0-289 days) and 94.4% were elective. The indications for tracheostomy were ventilation for more than 7 days with (61.1%) or without (38.9%) orolaryngotracheal anomaly. The median number of consultants involved per patient was 16 consultants (10-23 consultants). The median hospital length of stay was 122 days (8-365 days) before tracheostomy and 235 days (22-891 days) after tracheostomy. The median invasive ventilation time was 68 days (8-168 days) before tracheostomy and 64 days (5-982 days) after tracheostomy. In terms of complications, there were nine cases of tracheitis and five cases of tracheal granulomas. At 24 months of corrected age, 17 of 18 children survived, one of/17 was still hospitalized, three of 17 were decannulated, three of 17 received respiratory support via their tracheostomy, 11 of 17 were fed with a gastrostomy, and all had neurodevelopmental delay. CONCLUSION: Tracheostomy in infants requiring at least 1 week of ventilation is performed for complex cases and is favored for orolaryngotracheal anomalies. Clinicians should anticipate the need for developmental care in this population.

Langerhans' cell histiocytosis of the temporal bone in children.

OBJECTIVE: Langerhans' cell histiocytosis (LCH) is a rare pathology that implies an abnormal proliferation of these kinds of cells associated with a granular infiltration that affects different structures of the human body, including the temporal bone. The authors present their series of LCH of the temporal bone in children at the Sainte-Justine university hospital. The twofold objective of this study is to illustrate the clinical presentation, management and prognosis of this disease, and to compare these results with previously reported series. METHODS: A retrospective study was conducted between 1984 and 2007 with patients diagnosed and treated for a LCH of the temporal bone at the Sainte-Justine university hospital, a paediatric tertiary care center. A chart review was performed to obtain demographic, clinical, paraclinical, and therapeutic data. They were analysed and compared to other published series. Through a MEDLINE query, we found that since 1966, 50 articles dealing with a LCH of the temporal bone have been published. RESULTS: Fifty-nine cases of LCH were diagnosed and among them, 10 children had temporal bone involvement. They were four females and six males with a mean age of 3.28 years. The two most frequent clinical manifestations were the presence of a mass in the temporal region (70%) and otitis (60%). Two of our patients had a unifocal lesion of the temporal bone implicated. Eight patients had a multisystem involvement among which, two showed evidence of organ dysfunction. In 80% of cases, the diagnosis was made by immunohistochemical findings of the S-100 protein and/or the CD1 antigen. The common radiological finding on a skull CT scan is a lytic lesion in the temporal bone. Seven patients were treated by chemotherapy, two were treated by radiotherapy as a primary treatment, and one received radiotherapy for a recurrence on the pituitary gland. Finally, one patient was treated with local steroid injections. Two patients had a recurrence. All our patients were in total remission on a mean average of 1.6 years after the diagnosis. Our results concord with other studies in which the prognosis of unifocal bone disease is excellent and children with a multifocal disease have a survival rate of 65-100%. CONCLUSION: LCH is a rare disease. A high-index suspicion should be raised in the context of a temporal mass, chronic otitis, and otorrhea. A biopsy is recommended in the presence of a temporal bone lytic lesion. Chemotherapy is the preferred therapeutic modality.

Temporal bone rhabdomyosarcoma in children.

OBJECTIVE: Rhabdomyosarcoma is the most frequent soft tissue sarcoma in the pediatric age group. The authors present their series of rhabdomyosarcoma of the temporal bone in children at Saint Justine Hospital. The twofold objective of this study is to illustrate the clinical presentation, management, and prognosis of this malignant striated muscle tumor, and to compare these results with previously reported series. METHODS: A retrospective study was conducted of patients diagnosed and treated for rhabdomyosarcoma of the head and neck at Saint Justine Hospital, a tertiary pediatric center, between 1970 and 2005. Only cases of temporal bone rhabdomyosarcoma were included in the study. A thorough review of medical and surgical charts was performed to obtain demographic, clinical, paraclinical, and therapeutic data, which were subsequently analyzed and compared to published results. A MEDLINE search yielded 34 studies dealing with temporal bone rhabdomyosarcoma since the year 1966. RESULTS: Thirty-nine patients with rhabdomyosarcoma of the head and neck region were identified, among which only six children had temporal bone rhabdomyosarcoma. The mean age at the time of diagnosis was 4.15 years. Chronic otitis media was the most common clinical presentation. Five children had the embryonal subtype and one had the botryoid subtype on histology. All patients except two received combined chemotherapy and radiotherapy as treatment. Five-year survival rate was 66%. Our results match those reported in the literature 41-81%. CONCLUSION: Rhabdomyosarcoma of the temporal bone is an aggressive tumor that clinically simulates chronic otitis media. A high index of suspicion should be raised in the context of otitis media that is unresponsive to ordinary medical treatment. A biopsy is hence recommended in the presence of polyps in the external auditory canal that are resistant to medical treatment. Early diagnosis and the adoption of multimodal therapy offer the best outcome.

Nontuberculous mycobacterial adenitis of the head and neck in children: experience from a tertiary care pediatric center.

OBJECTIVE: To describe our experience with the diagnosis, surgical treatment, and outcome of nontuberculous mycobacterial (NTM) adenitis of the head and neck in children, and to present a preliminary report about the use of NTM skin tests in our institution. STUDY DESIGN: Retrospective study. METHODS: The medical records of all children diagnosed with cervicofacial NTM adenitis were retrospectively reviewed for the period from January 1, 1995, through December 31, 2000. We also examined the use of intradermal skin tests for the diagnosis of NTM infection. RESULTS: Fifty patients were diagnosed with NTM cervicofacial adenitis. Pertinent demographic information, clinical presentation, investigation, and type of diagnostic procedures were documented. Surgical procedures, complications, and relapses were also noted. One unusual case of retropharyngeal adenitis is illustrated. All patients were treated with complete excision of their lesion at the first operation. No major complications were noted. Only one patient relapsed and required a second operation. Forty-one children were skin-tested with NTM antigens. Of these, 30 patients were dual-tested with Purified Protein Derivative (PPD) also. No adverse reactions were noted with the use of skin tests. Sensitivity of NTM antigens alone is 87%. Sensitivity of dual testing is 78%. No patient had a PPD-dominant reaction. CONCLUSION: Surgical excision is the treatment of choice of NTM adenitis because of the high cure rate with a single procedure, the excellent cosmetic result, and the low complication rate. NTM skin tests are safe and could be useful in early diagnosis of the infection but further investigation is needed.

Tubeless spontaneous respiration technique for pediatric microlaryngeal surgery.

BACKGROUND: Tubeless spontaneous respiration technique for pediatric microlaryngeal surgery may be accomplished using different anesthetic protocols. Two methods, inhalation of volatile anesthetic agents alone and in combination with intravenous propofol, are reviewed with regard to intraoperative airway stability, post-operative morbidity, recovery room course, and halothane concentration required during maintenance anesthesia. DESIGN: Retrospective case series. SETTING: Otolaryngology referral hospital. PATIENTS AND METHODS: Twenty-nine microlaryngeal procedures were performed using tubeless spontaneous respiration technique in children ranging from 2 weeks to 11 years of age. The following 2 anesthetic protocols were used: inhaled volatile anesthetic agents alone in 18 procedures and in combination with intravenous propofol in 11. Anesthesia, surgery, and recovery room times were documented. Specific characteristics of anesthetic maintenance, including total anesthetic gas flow (liters per minute), variations of halothane concentration (percentage), and duration of halothane administration (minutes) were also recorded to calculate the mean concentration of halothane (percentage) delivered to each patient. RESULTS: No statistical differences were observed between the 2 protocols in terms of anesthesia and surgical outcomes. Adjusting for differences in patient age, weight, maintenance duration, and total anesthetic gas flow, the introduction of propofol allowed a statistically significant reduction in the mean concentration of halothane required during maintenance anesthesia. CONCLUSIONS: Both tubeless spontaneous respiration technique protocols proved successful in this study. However, the addition of propofol allowed a significant reduction in the halothane requirement during anesthesia maintenance. This has the potential benefit of decreasing the exposure of operating room personnel to volatile anesthetics during tubeless spontaneous respiration technique.

Oral soft tissue myxoma: report of a case and review of the literature.

The soft tissue myxoma of the oral cavity is a rare neoplasm. We report a case arising from the palate in a 15-year-old male. A review of the literature is presented with clinicopathologic, immunohistochemical, and ultrastructural data confirming the diagnosis. The pathogenesis and differential diagnosis are discussed with reference to documented association with fibrous dysplasia and "Carney syndrome."