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A 2-year-old, male patient with a mediastinal cystic mass causing tracheal stenosis underwent complete resection of the mass via a partial median sternotomy. The risk of left recurrent laryngeal nerve (RLN) injury seemed high because the mass was located just between the trachea and the esophagus. To reduce the risk of injury to the RLN, intraoperative nerve monitoring (IONM) was used with an adhesive laryngeal electrode, which was wrapped around an endotracheal tube with a 4.0-mm inner diameter. Electromyography of the vocal cords was successfully performed, allowing the left RLN to be clearly detected intraoperatively. The patient's postoperative course was uneventful, and he had no postoperative symptoms of RLN palsy. Esophageal duplication cyst was pathologically diagnosed.IONM can be applied even to infants and may be very useful for improving procedural safety and avoiding damage to the RLN during rection of a mediastinal mass.

Abstract Background Patients with severe motor and intellectual disabilities often suffer from tracheal stenosis due to chest deformation and brachiocephalic artery compression, which sometimes leads to serious complications, such as dying spell and tracheobrachiocephalic artery fistula. We herein described our experience of performing a novel and simple thoracoplastic procedure combined with brachiocephalic artery transection in two patients with severe chest deformation and tracheal stenosis. Case presentation The patients were a 12-year-old female with cerebral palsy due to periventricular leukomalacia and a 21-year-old male with subacute sclerosing panencephalitis stage IV in the Jabbour classification following a laryngotracheal separation. Both patients showed severe chest deformation and symptoms of airway stenosis resulting in dying spells. The sternum was laterally transected between the manubrium and the sternal body, and a manubriotomy was performed longitudinally, ending with an inverse T-shaped sternotomy. Since the clavicle and the first rib remained attached to the halves of the divided manubrium, the sternum was allowed to be left open, resulting in improvement of the mediastinal narrowing and tracheal stenosis. Postoperative computed tomography (CT) showed that the distance between the halves of the manubrium was maintained at 10–11 mm, and that the mediastinal narrowing in both patients improved; the sternocervical spine distance increased from 20 mm to 22 and 13 mm to 16 mm, respectively. The patients’ tracheal stenosis below the sternal end of the clavicle and the manubrium and respiratory symptoms improved, and the patients are currently at home in a stable condition with no chest fragility and no upper limb movement disorder 1 year after surgery. Conclusions Our observations suggested that the inverse T-shaped sternotomy combined with brachiocephalic artery transection may relieve symptoms of tracheal stenosis due to severe chest deformation in patients with severe motor and intellectual disabilities.

Abstract Background Our group previously proved that the human enteric nervous system can be visualized with confocal laser endomicroscopy after topical application of cresyl violet using surgically resected intestine specimens. The present report documents the first in vivo visualization of the human enteric nervous system with confocal laser endomicroscopy using local cresyl violet staining. The aim of this study was to evaluate the technical feasibility and clinical efficiency of confocal laser endomicroscopy in patients with Hirschsprung’s disease and allied disorders in vivo. Methods Confocal laser endomicroscopy was performed in vivo in two patients to confirm the presence of the enteric nervous system during surgery in patients with Hirschsprung’s disease and allied disorders. Cresyl violet was gently injected from the serosal side into the muscular layer of the intestine, and scanning was performed within 30 min. Then, the scanned intestines were resected, and the visualized area of the specimens was pathologically evaluated. Results The ganglion cell nuclei and the enteric nervous system network were clearly visualized intraoperatively in both cases. The morphological findings were similar to the pathological findings of the enteric nervous system in both cases although the period of visibility was brief. Conclusion This study demonstrated the first, real-time observation of the enteric nervous system in humans using confocal laser endomicroscopy and suggest the potential to identify the enteric nervous system intra-operatively during surgery for Hirschsprung’s disease and allied disorders.

Primary rhabdomyosarcoma (RMS) of the diaphragm, especially alveolar RMS, is extremely rare. Because of its rarity, no treatment for it has yet been established, but previous studies have reported long-term survival in recipients of a combination therapy with local control, including a total resection and/or radiation therapy concomitant with multiagent chemotherapy. We experienced a case of primary alveolar rhabdomyosarcoma of the diaphragm surrounding the gastric cardia in a 15-year-old, female patient. After administering chemotherapy consisting of vincristine, actinomycin D, and cyclophosphamide (VAC) to shrink the tumor, tumor resection was performed with a proximal gastrectomy and partial resection of the diaphragm, pericardium, and left lung. After receiving postoperative adjuvant chemotherapy with VAC and radiotherapy at a total of 41.4 Gy, the patient has been relapse-free for 18 months. The tumor-induced stenosis at the hilum was corrected, allowing oral intake and markedly improving the patient's nutritional status. Tumor resection with a proximal gastrectomy for primary diaphragmatic RMS surrounding the lower esophagus is recommended both for its efficacy in local control and for allowing the preservation of postoperative gastrointestinal function.

Background: In our institution, we avoid emergency nighttime appendectomies, instead performing the surgery during daylight hours the following day. We examined whether emergency or early appendectomies affect the outcome of patient morbidity. Materials and Methods: Medical records of children treated for appendicitis between 2010 and 2012 were retrospectively reviewed. Outcomes were compared between Group 1, defined as those patients who presented to the hospital during the day and underwent appendectomy on the same day and Group 2, defined as those patients who presented to the hospital at night and underwent appendectomy the next day. Incidences of perforation at surgery, operative time, complications and length of stay were analysed. Cases with perforation were also analysed to determine if the perforations could have been identified preoperatively. Results: A total of 74 patients met the study criteria, including 41 and 33 in Groups 1 and 2, respectively. There were no significant differences in the incidence of perforation at surgery, operative time, complications and length of stay. A total of nine cases of perforation were identified during surgery, and there were no significant differences in the pre-operative characteristics between perforated and non-perforated cases. Conclusions: The results of this study indicate that early appendectomy is safe and did not increase patient morbidity. We, therefore, recommend performing appendectomies in the daytime.

Aim: The current study aimed to assess the perinatal risk of congenital cystic lung diseases (CCLD) and to establish a suitable surgical strategy in consideration of postoperative lung growth and problems during adulthood.Methods: Among 874 CCLD patients identified in a nationwide survey, 428 patients were born between 1992 and 2012 and treated at 10 high-volume centers were retrospectively reviewed with statistical analysis.Results: In the 194 patients who were prenatally diagnosed to have CCLD, 16.7% presented with fetal hydrops as observed using ultrasonography. The Apgar score (5 minutes) was lower than 5 in 5.4%. As of postnatal day 30, 14.0% of the neonatal patients required respiratory support, and 3.3% (8/243) had died because of pulmonary hypoplasia. Among those who were asymptomatic immediately after birth, 33.6% of the patients developed the respiratory infectious symptoms during their first year of life, and 22.1% did so between the age of 1 and 2 years. The postoperative percent vital capacity among the prenatally diagnosed patients was significantly higher than that among the postnatally diagnosed patients (98.3 ± 11.9 vs. 81.7 ± 9.7, p < 0.0222). Late complications included thoracic deformity in 30 patients and persistent lung cyst in 4 patients, whereas malignancy was not observed in the present series.Conclusions: Approximately 10 to 15% of prenatally diagnosed CCLD patients may carry a high risk of perinatal respiratory distress. Early operation before developing episodes of lung infection, seem to be associated with a better development of the reserved lung during later life. The incidence of carcinogenesis among patients with CCLD may be extremely low. [ABSTRACT FROM AUTHOR]

A 4-year-old boy presented with pneumonia. Computed tomography demonstrated a multicystic mass at the posteromedial bilateral lower lobe segments, which were connected by a bronchus behind the heart. Enhanced computed tomography revealed that an anomalous artery arose from the left gastric artery and inserted into the left sequestrated lung and branched to the right one. The diagnosis of bilateral intralobar sequestration (ILS) with a bridging isthmus was made. After removal of the bilateral ILS, radiologic and pathologic approaches were undertaken to reconstruct the vascular and bronchial architectures. The following observations were made: (1) histologically, the region near the anomalous artery insertion site contained bronchial structures, which looked like an ectopic pulmonary hilus. This bronchial structure was continuously observed in the isthmus and its opening of the right sequestrated lung; (2) radiologically, the shape and course of the bronchi within the ILS indicated a distinct bronchial origin that arose from the pulmonary hilus-like structure, and the anomalous artery that ran along with those bronchi, resembled a pulmonary artery. These features suggested that this bilateral ILS might have originated from an accessory lung tissue. [ABSTRACT FROM AUTHOR]