Purpose: We conducted a questionnaire survey regarding surgical treatment of children and adults with severe motor and intellectual disabilities (SMID) at the Kyushu Pediatric Surgery Study Group and investigated the practice of gastrostomy.

Methods: The subjects were patients in whom gastrostomy was performed under general anesthesia between January 2018 and December 2022. The survey items included age, concurrent surgery, surgical procedure, gastropexy method, postoperative nutritional management, and complications.

Results: 579 gastrostomy cases were collected from 22 institutions. The most common age group was 1–5 years old (37%), but patients aged 16 years or older accounted for 30%. 321 cases involved gastrostomy alone, and the most common concurrent surgery of the other patients was fundoplication (163 cases). Regarding cases with gastrostomy alone, the most common procedure was laparoscopically assisted gastrostomy without gastroendoscope (37%), followed by laparoscopically assisted PEG (LA-PEG) (26%). A Funada-style gastropexy device was used in 80% of the patients using a gastroendoscope. Postoperative fluid administration via gastrostomy was initiated by the first postoperative day; however, enteral nutrition was initiated from one to four days after surgery. Blenderized tube feeding (BTF) was used in 17% of the patients. The postoperative infectious complication rate was significantly lower in the PEG and LA-PEG groups than in the laparoscopically assisted gastrostomy groups.

Conclusions: Pediatric surgeons performed gastrostomy in not only children but also adolescents and adults with SMID. Although laparoscopically assisted gastrostomy was the most common procedure, PEG and LA-PEG were superior in reducing postoperative complications. The initiation of postoperative enteral nutrition was not early, and the early introduction of BTF was rare.

Purpose: Clinical practice in pediatric tracheostomy decannulation varies widely because evidence-based guidelines are limited. We reviewed a decade of experience in our department to describe current procedures and outcomes, and to establish a standardized protocol with clear indications and steps.

Methods: We included patients aged ≤15 years who underwent decannulation attempts in our department between January 2013 and November 2023. Patients who underwent laryngotracheoplasty were excluded. Our standard approach consists of a staged downsizing of the tracheostomy tube, capping trials, and rigid bronchoscopy, followed by inpatient decannulation. Since 2022, speech cannulas have been used to support aspiration prevention and speech practice. However, the order and duration of each step were not standardized. We retrospectively reviewed medical records to evaluate procedures and decannulation outcomes, and performed logistic regression to identify potential risk factors for intratracheal granulation that may hinder decannulation.

Results: Thirty-seven decannulation attempts were performed in 33 patients. The median age at decannulation was 5 years, and the success rate was 86.5%. Rigid bronchoscopy was performed before decannulation in 91.9% of attempts. Tracheal granulation was treated in 23.5% of those patients, and speech cannulas were used in 32.4%. The median capping duration, post-decannulation observation, and time to decannulation were 6, 4, and 90 days, respectively. No significant factors were associated with tracheal granulation.

Conclusions: Our findings indicate that implementing a protocol is essential for efficient and timely decannulation. On the basis of these findings, we developed a new pediatric decannulation protocol incorporating a 6-day hospitalization along with the routine use of a speech cannula and speech valve.

A 14-year-old boy with suspected neurofibromatosis type 1 (NF1) presented with abdominal pain and a large retroperitoneal mass accompanied by right hydronephrosis. A malignant tumor, such as malignant peripheral nerve sheath tumor (MPNST) or embryonal rhabdomyosarcoma (ERMS), was suspected. Two open biopsies revealed only reactive inflammatory tissue and failed to establish a diagnosis. Following postoperative bowel obstruction, tumor resection was performed, enabling a definitive diagnosis. Histopathological and immunohistochemical analyses (positive for desmin, myogenin, and MyoD1; negative for S-100; complete loss of H3K27me3) confirmed malignant triton tumor (MTT), a subtype of MPNST. Despite postoperative chemotherapy, the tumor progressed, and the patient died 15 months after surgery. Tumor heterogeneity, including necrosis and inflammation, likely contributed to sampling error. This case highlights the fact that when a biopsy fails to yield a definitive diagnosis despite a clinical course suggestive of a malignant tumor such as NF1, it is necessary to perform biopsies from multiple and deeper sites, taking into account the heterogeneity of the tumor. Furthermore, in cases where diagnosis is difficult, it is considered important to evaluate the clinical findings suggestive of malignancy comprehensively and then consider diagnostic tumor resection.

A 4-year-old boy presented with a 5-day history of abdominal pain and vomiting. He had initially been treated for acute gastroenteritis at a previous hospital; however, the vomiting subsequently became bilious. Contrast-enhanced abdominal CT revealed intestinal volvulus associated with a cystic lesion, leading to his transfer to our hospital. During emergency surgery, an 18 × 13 cm cystic lesion in the small-bowel mesentery was identified, which had caused a 900-degree counterclockwise volvulus of the small intestine. After detorsion, the intestine was found to be viable without necrosis; the cyst was resected along with a 10 cm segment of the small intestine. Histopathological examination confirmed lymphangioma. This case represents a rare instance in which intestinal necrosis did not occur despite a 5-day clinical course and a severe 900-degree volvulus. One possible contributing factor was the subacute clinical course suggested by the chylous cyst contents. Additionally, the massive cyst within the confined abdominal space may have supported the torsion base from the caudal side. This mechanical support may have reduced stress on the mesentery, thereby preventing excessive stretching and compression of the mesenteric vessels.

A 5-year-old boy with a history of repair of a low-type anorectal malformation was transferred to our institution for suspected bowel obstruction after presenting with abdominal pain and vomiting. On arrival, he exhibited abdominal distension and tenderness. Contrast-enhanced CT revealed clustered small bowel loops in the right upper quadrant with distal dilatation. These findings were suggestive of strangulated bowel obstruction caused by a right paraduodenal hernia (PDH), and emergency laparotomy was performed. Intraoperatively, the small intestine was found to be incarcerated through a hernia orifice formed by incomplete fusion between the right colonic mesentery and the retroperitoneum. After enlargement of the orifice, ileal torsion around Meckel’s diverticulum was identified. Detorsion and partial ileal resection, including the diverticulum, were performed. The jejunum was located on the right without the formation of the ligament of Treitz, indicating malrotation. In right-sided PDH, the hernia sac is often opened to prevent recurrence. However, in cases complicated by intestinal malrotation, the superior mesenteric artery and ileocolic artery may run close to the hernia orifice; therefore, careful assessment of the mesenteric vasculature is essential to avoid vascular injury during surgical manipulation. This case is notable for the preoperative suspicion of right PDH based on contrast-enhanced CT findings and by the rare mechanism of a strangulated bowel obstruction caused by ileal torsion around Meckel’s diverticulum within the hernia sac. These findings highlight the importance of accurate imaging diagnosis and an appropriate surgical strategy in the management of right-sided PDH.

Fetal lung interstitial tumor (FLIT) was reported by Dishop et al. in 2010. We report a case of FLIT resected by right middle lobectomy through a hemi-clamshell approach (HCA) and review the literature. A nine-day-old boy was transferred to our hospital because a huge FLIT occupied his right thorax and caused respiratory failure. His respiratory condition seemed to gradually improve, and a contrast computed tomography revealed that the huge tumor originated from the right middle lobe and displaced the other lobes. Right thoracotomy through the hemi-clamshell approach was performed. The tumor adhered to the mediastinal pleura, pericardium, and diaphragm; however, there were no signs of invasion. Right middle lobectomy was performed without any intraoperative complications. Partial wound disruption occurred as a postoperative complication; however, the patient recovered with negative pressure wound therapy. The patient was discharged without requiring any medical care at home. HCA seems to be a considerably useful thoracotomy technique to secure a commanding operative field in cases where a huge tumor occupies the hemithorax and the pediatric patient is symptomatic.

The patient was a 21-year-old male who was diagnosed with congenital malformation on the basis of peculiar facial features, thoracic kyphosis, and mental retardation. A tracheostomy with an anterior cricoid cartilage resection and the final correction of kyphosis were performed at the ages of 13 and 20, respectively. He was referred to our department for tracheostomy closure. The diameter of the tracheostomy orifice was 14 × 16 mm. Respiration was maintained even after the tracheostomy was temporarily closed with medical tape, ascertaining the legitimacy of the tracheostomy closure. During the operation, a hinged skin flap was created by leaving an island of epidermis cranial to the tracheostomy orifice, with the surrounding epidermis removed. The epidermal portion of the hinged flap was folded caudally and suture-fixed to the orifice. The hinged skin flap was further strengthened by a right sternohyoid muscle flap. He was discharged on the seventh postoperative day without aspiration pneumonia or respiratory distress on the adjusted diet and has been doing well for two years. Tracheostomy closure with a hinged skin flap to reconstruct the anterior tracheal wall can be a safe and useful approach for patients with seemingly “permanent” tracheostomy with anterior cricoid cartilage removal.