Anterior mediastinal tracheostomy for severe tracheal stenosis in a child with severe motor and intellectual disabilities: a case report.

BACKGROUND: Owing to recurrent aspiration pneumonia and airway stenosis secondary to thoracic deformities, tracheostomy or laryngotracheal separation are often necessary in children with severe motor and intellectual disabilities. However, these procedures are associated with the risks of tracheal stenosis due to tracheal granulation and tracheoinnominate artery fistula formation. We report a case of a child with severe motor and intellectual disabilities treated with an anterior mediastinal tracheostomy. CASE PRESENTATION: The patient was a 15-year-old boy with severe motor and intellectual disabilities due to intractable epilepsy. Due to thoracic deformity and tracheomalacia, the patient had a flattened and narrowed trachea. Accordingly, laryngotracheal separation was performed 4 months before admission to avoid aspiration pneumonia. Due to a common cold, the patient required frequent sputum suctioning, which exacerbated the tracheal stenosis. Bronchoscopy revealed tracheal stenosis 4-5 cm caudal to the tracheostomy site, tracheal mucosal ulcers, and pulsation of the innominate artery on the anterior wall of the trachea. We performed an anterior mediastinum tracheostomy to release the tracheal stenosis and prevent tracheoinnominate artery fistula formation. CONCLUSIONS: Anterior mediastinal tracheostomy has several advantages. Including sufficient release of bony compression, release of tracheal hyperextension, and relief of tracheal and innominate artery contact ensures a cannula-free tracheostomy, and there is no need to dissect the brachiocephalic artery. It is the procedure of choice in cases of head and neck malignancies requiring extensive tracheal resection and could be a good surgical option for severe tracheal stenosis and tracheoinnominate artery fistula in children with severe motor and intellectual disabilities.

A model study for the manufacture and validation of clinical-grade deciduous dental pulp stem cells for chronic liver fibrosis treatment.

BACKGROUND: Human deciduous pulp stem cells (hDPSCs) have remarkable stem cell potency associated with cell proliferation, mesenchymal multipotency, and immunosuppressive function and have shown beneficial effects in a variety of animal disease models. Recent studies demonstrated that hDPSCs exhibited in vivo anti-fibrotic and anti-inflammatory action and in vivo hepatogenic-associated liver regeneration, suggesting that hDPSCs may offer a promising source with great clinical demand for treating liver diseases. However, how to manufacture ex vivo large-scale clinical-grade hDPSCs with the appropriate quality, safety, and preclinical efficacy assurances remains unclear. METHODS: We isolated hDPSCs from human deciduous dental pulp tissues formed by the colony-forming unit-fibroblast (CFU-F) method and expanded them under a xenogeneic-free and serum-free (XF/SF) condition; hDPSC products were subsequently stored by two-step banking including a master cell bank (MCB) and a working cell bank (WCB). The final products were directly thawed hDPSCs from the WCB. We tested the safety and quality check, stem cell properties, and preclinical potentials of final hDPSC products and hDPSC products in the MCB and WCB. RESULTS: We optimized manufacturing procedures to isolate and expand hDPSC products under a XF/SF culture condition and established the MCB and the WCB. The final hDPSC products and hDPSC products in the MCB and WCB were validated the safety and quality including population doubling ability, chromosome stability, microorganism safety, and stem cell properties including morphology, cell surface marker expression, and multipotency. We also evaluated the in vivo immunogenicity and tumorigenicity and validated in vivo therapeutic efficacy for liver regeneration in a CCl4-induced chronic liver fibrosis mouse model in the final hDPSC products and hDPSC products in the WCB. CONCLUSION: The manufacture and quality control results indicated that the present procedure could produce sufficient numbers of clinical-grade hDPSC products from a tiny deciduous dental pulp tissue to enhance clinical application of hDPSC products in chronic liver fibrosis.

Laparoscopic-assisted Stamm-gastrostomy: technical modifications to ease suturing inside the minimal trocar site.

Laparoscopic gastrostomy tube placement has been increasingly adopted by pediatric surgeons. We herein report our experience with the performance of a laparoscopic-assisted Stamm-operation inside the minimal trocar site without the extension of the trocar site incision or mini-laparotomy. We present some technical modifications that facilitate suturing inside the minimal trocar site. METHODS: A retrospective chart review was conducted of cases involving patients who underwent laparoscopic-assisted gastrostomy, using a simple extracorporeal method inside the trocar site from April 1998 to March 2018. RESULTS: One hundred five gastrostomy tubes were placed in a laparoscopic-assisted operation. All but two of the cases involved patients with neurological impairment. The mean age was 12.5 years; 28 cases were > 16 years of age. Seventy-five cases underwent gastrostomy during laparoscopic fundoplication. All gastrostomy procedures were completed without intraoperative difficulties; however, 8 cases, which involved a thick abdominal wall, required extension of the trocar site. No cases required conversion to open gastrostomy. No major complications were observed. Two patients developed continuous peristomal cellulitis after surgery, due to the mismatch of the site position and an unsuitable button device size. CONCLUSION: We demonstrated that laparoscopic gastrostomy with fully extracorporeal suturing within the trocar site is feasible and beneficial, especially for the most neurologically impaired pediatric cases. Technical modification, changing the order of the process, and suturing technique in the minimal space, made it easier to perform the procedures inside the minimal trocar hole.

Portal venous gas in intestinal malrotation with mild midgut volvulus.

BACKGROUND: Portal venous gas has been considered as a radiological sign requiring urgent operative intervention; however, the reports concerning portal venous gas associated with favorable outcome are recently increasing. CASE PRESENTATION: We describe a 9-month-old boy with acute onset high fever and vomiting. The ultrasonography demonstrated micro-gas bubbles continuously floating in the intrahepatic portal vein. Contrast-enhanced CT, performed 1 h later from echography, revealed a whirlpool sign suggesting an intestinal malrotation with midgut volvulus, but with no signs of residual intrahepatic gas. Operative findings showed a mild volvulus with neither congestion nor ischemic change of the twisted bowel. Detorsion and Ladd's procedure were completed laparoscopically. CONCLUSIONS: Transient portal venous gas bubbles may be generated even in the mild intestinal volvulus with no bowel ischemia. Ultrasonography can be a sensitive detector to visualize such small amounts of gas.

Blowhole tangential cecostomy and transanal tube insertion for neonatal cecal perforation in a patient with Hirschsprung's disease in the earlier definitive operation era.

BACKGROUND: Although uncommon and seldom experienced, intestinal perforation is a well-known complication of Hirschsprung's disease (HD). A literature review revealed that the cecum, including the appendiceal base, is a site of perforation. The cecum is not suitable for making an ordinary loop colostomy, and the optimal operative strategy remains to be established. CASE PRESENTATION: We present a combination technique composed of tangential cecostomy at the perforated portion and postoperative care with a transanal indwelling tube, which was used in the treatment of a 3-day-old boy with cecal perforation with long-segment Hirschsprung's disease. A temporary simple blowhole stoma and continuous decompression with daily irrigation via a transanal indwelling tube in the distal colon achieved a secure recovery and was followed by a definitive operation in the early period. The combination of tangential cecostomy and transanal indwelling catheter management led to the preservation of the ileocecal valve. CONCLUSIONS: We review the Japanese literature and emphasize the usefulness of this combination technique by blowhole tangential cecostomy and transanal tube insertion for neonatal cecal perforation in patients with HD in today's early definitive operation era.

Immunostaining for Hu C/D and CD56 is useful for a definitive histopathological diagnosis of congenital and acquired isolated hypoganglionosis.

Isolated hypoganglionosis (IHG) has been proposed as a distinct entity with two subtypes: congenital IHG (CIHG) and acquired IHG (AIHG). However, due to the rarity of the disease and the lack of defining histological criteria, the concept of IHG is not widely accepted. We studied paraffin-embedded intestinal specimens from 79 patients diagnosed with Hirschsprung's disease (HD) (n = 49), CIHG (n = 25), and AIHG (n = 5) collected between January 1996 and December 2015. Histopathological diagnosis of HD, CIHG, and AIHG was confirmed by hematoxylin and eosin staining and immunohistochemical staining using Hu C/D and CD56. We evaluated (immuno)histopathological findings, counted the number of ganglion cells, and measured the size of Auerbach's plexus. Hu C/D labeled neuronal cell bodies, whereas CD56 was detected in all neuronal components. In HD, all ganglion cells in Auerbach's plexus in the normoganglionic segment (NGS) were immunoreactive for Hu C/D, whereas in the aganglionic segment (AGS), these were replaced by CD56-positive extrinsic nerve fibers and bundles. The number of ganglion cells in AIHG and CIHG was significantly lower than in the NGS of HD (p < 0.05). Auerbach's plexus was significantly smaller in CIHG (p < 0.05) but in AIHG equivalent to the NGS in HD. In summary, immunostaining for Hu C/D and CD56 is useful for definitive histopathological diagnosis of IHG.

The evaluation of rectal mucosal punch biopsy in the diagnosis of Hirschsprung's disease: a 30-year experience of 954 patients.

PURPOSE: For 30 years, we have consecutively performed rectal mucosal punch biopsy to diagnose Hirschsprung's disease. The aim of this study was to evaluate the safety of our technique. METHODS: Patients with suspected Hirschsprung's disease who underwent punch biopsy, including our original "K-PUNCH" method using an S-moid forceps and non-specific blood-collecting tube at our department and branch hospital between April 1986 and March 2016 were included in the present study. Our punch biopsy technique is characterized by excellent visibility and a direct grasping sensation. The backgrounds and complications of the patients were retrospectively investigated. RESULTS: During this period, 954 patients (median age 4 months; range 1 day-73 years) underwent punch biopsy. Although there were no cases of severe complications (i.e., rectal perforation, infection or full-thickness biopsy), one (0.1%) of the 954 cases in the early period showed liver dysfunction and required transfusion due to bleeding. In addition, inappropriate specimens were obtained in 37 patients (3.9%). CONCLUSION: Punch biopsy including the "K-PUNCH" method is considered safe and feasible and is associated with a low rate of complications and inappropriate specimen harvesting among patients of all ages. Comorbidities, including the potential for hemorrhage, should always be considered.