Safety evaluation of a stepwise tracheostomy decannulation program in pediatric patients.

PURPOSE: In the event of failed tracheostomy decannulation, patients might have a tragic course of events. We retrospectively evaluated our stepwise tracheostomy decannulation program and examined its safety. METHODS: A 12-year retrospective study of pediatric patients was conducted. The decannulation program was performed on patients who had airway patency by laryngobronchoscopy and whose cannula could be capped during the day. A stepwise decannulation program was performed: continuous 48-h capping trial during hospitalization (Phase 1), removal of the tracheostomy tube for 48 h during hospitalization (Phase 2), and outpatient observation (Phase 3). If a persistent tracheocutaneous fistula existed, the fistula was closed by surgery (Phase 4). RESULTS: The 77 patients in the study underwent 86 trials. The age at the first time of the decannulation program was 6.5 ± 3.6 years. Sixteen trials failed (18.6%): 8 trials in Phase 1, 2 trials in Phase 2, 4 trials in Phase 3, and 2 trials in Phase 4. Most decannulation failures were due to desaturation in Phase 1/2 and dyspnea in Phase 3/4. The time to reintubation after decannulation was 15-383 days in Phase 3/4. CONCLUSIONS: Patients could fail at every phase of the program, suggesting that a stepwise decannulation program contributes to safety.

The Association between the First Cry and Clinical Outcomes in CDH Neonates: A Retrospective Study.

Congenital diaphragmatic hernia (CDH) is a life-threatening condition characterized by the herniation of abdominal organs into the thorax, resulting in hypoplastic lungs and pulmonary hypertension. The impact of the first cry, a crucial event for lung transition during birth, on CDH patients remains unclear. This study investigated the impact of the first cry during birth on CDH patient survival, along with other prognosis factors. A multi-institutional retrospective study assessed CDH patient characteristics and survival rates by analyzing factors including the first cry, disease severity, birth weight, Apgar scores, oxygenation index (OI) and surgical closure. Among the CDH patients in the study, a positive first cry was linked to 100% survival, regardless of disease severity (p < 0.001). Notably, the presence of a positive first cry did not significantly affect survival rates in patients with worse prognostic factors, such as low birth weight (<2500 g), high CDH severity, low Apgar scores (1 min ≤ 4), high best OI within 24 h after birth (≥8), or those who underwent patch closure. Furthermore, no significant association was found between the first cry and the use of inhaled nitric oxide (iNO) or extracorporeal membrane oxygenation (ECMO). In conclusion, this study suggests that the first cry may not have a negative impact on the prognosis of CDH patients and could potentially have a positive effect.

Pancreaticobiliary maljunction without biliary dilatation in pediatric patients.

PURPOSE: Pancreaticobiliary maljunction (PBM) without biliary dilatation is a condition in which dilatation of the bile duct is not seen in patients with PBM. Recently, the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) published new diagnostic criteria for PBM. In these criteria, biliary dilatation is defined according to the standard diameter at each age. We reviewed cases of pediatric patients with PBM without biliary dilatation. METHODS: From 1992 to 2019, 134 patients with PBM were treated in our institution. Among these, 7 patients were retrospectively diagnosed with PBM without biliary dilatation. The clinical information was retrospectively assessed in these patients. RESULTS: Of the seven patients, six were female. All patients had symptoms similar to those of patients with congenital biliary dilatation. In all seven patients, the diagnosis of PBM was made before definitive surgery. Six patients had type B PBM, and one had type D PBM. All patients underwent extrahepatic bile duct resection and hepaticojejunostomy, and their symptoms resolved. One patient experienced postoperative complications of anastomotic leakage followed by anastomotic stricture. CONCLUSION: The present report revealed important clinical features of this entity. However, there are still some issues that need to be discussed, and further research is needed.

Subtle Crucial X-Ray Findings in Pediatric Foreign Body Aspiration.

Foreign body aspiration (FBA), with potentially life-threatening outcomes, is not unusual in the pediatric population. We report two cases of lobar bronchial radiolucent foreign bodies. Chest X-ray (CXR) showed a slight but significant finding of lobar emphysema without a significant mediastinal shift. This is possibly a key to suspecting foreign bodies. In the clinical field, a stepwise approach to detecting foreign bodies is commonly performed, from less invasive options such as CXR to computed tomography (CT). In this context, clinicians should scrupulously check CXRs when pediatric patients complain of respiratory symptoms, especially with potential FBA history.

Obstructive Fecalomas in an Infant Treated with Successful Endoscopic Disimpaction.

A fecaloma is a mass of accumulated feces with a consistency much harder than that of a fecal impaction. It is most frequently observed in the rectum and sigmoid area, and associated complications include colonic obstruction, ulceration, bleeding, and perforation. A one-year-old, previously healthy boy with no history of chronic constipation was admitted because of vomiting and abdominal distension. An abdominal computed tomography scan showed small and large bowel distension due to multiple obstructive fecalomas in the transverse colon. As the fecalomas could not be resolved by laxatives, enemas, or colonic lavage, endoscopic disimpaction under general anesthesia was attempted. Repeatedly shaving the fecalomas with biopsy forceps finally resulted in gradual fragmentation with subsequent passage. Gastrointestinal food allergy was later suggested as the cause because eosinophilic infiltration was found in a biopsy specimen of the colon wall. Endoscopic disimpaction is an effective treatment approach for addressing fecalomas to avoid more invasive surgical intervention.

Risk factors of dumping syndrome after fundoplication for gastroesophageal reflux in children.

PURPOSE: In postoperative cases of fundoplication, the gastric emptying ability is promoted and sometimes exhibits dumping syndrome. Dumping syndrome often goes unrecognized in children. Furthermore, the risk factors for postoperative dumping syndrome are unknown. This study aimed to investigate the risk factors of developing dumping syndrome after fundoplication. METHODS: A retrospective chart review of all consecutive patients between January 2003 and March 2018 (190 patients) who had fundoplication at our clinic was conducted. Regarding the risk factors of dumping syndrome, gender, age and body weight at the time of surgery, neurological impairment, severe scoliosis, microgastria, chromosomal abnormalities, complex cardiac anomalies, gastrostomy, and laparoscopic surgery were retrospectively studied. RESULTS: 17 patients (9%) developed dumping syndrome post-operatively. Multivariate analysis showed that significant risk factors for dumping syndrome included: undergoing surgery within 12 months of age (adjusted OR 10.3, 95% CI 2.6-45.2), severe scoliosis (adjusted OR 19.3, 95% CI 4.4-91.1), and microgastria (adjusted OR 26.5, 95% CI 1.4-896.4). CONCLUSIONS: We identified that: age at fundoplication being within 12 months of age, severe scoliosis, and microgastria were risk factors for dumping syndrome after fundoplication, and that this information should be explaining to the family before conducting the fundoplication.

A 5-year-old boy with acute neurological disorder from anteflexion-induced cervical cord compression after tracheal surgery: Radiological findings similar to Hirayama disease.

Constant neck flexion has been considered crucial to reducing anastomotic tension after tracheal resection. However, in rare cases, anteflexion can cause cervical cord damage, leading to acute neurological disorders such as tetraplegia. Here, we report a case of 5-year-old boy presenting with acute neurological disorder triggered by a chin-to-chest position over 4 days of deep sedation after cricotracheal resection. The radiological findings would suggest a mechanism similar to Hirayama disease, in which a shift of the dura leads to chronic muscular weakness and atrophy in young populations.

Long-Term Outcomes After Pediatric Tracheostomy-Candidates for and Timing of Decannulation.

BACKGROUND: Although pediatric tracheostomy has been a widely performed, life-saving procedure, its long-term outcomes have remained unclear. This study aimed to review outcomes after tracheostomy at a Japanese tertiary hospital and clarify candidates for and timing of decannulation. MATERIALS AND METHODS: Hospital records of critically ill children who underwent tracheostomy from 2001 to 2014 were retrospectively reviewed, subsequently analyzing outcomes according to demographics, complications, and decannulation. After excluding those who were lost to follow-up or had irreversible neuromuscular impairment, the remaining patients were divided into the decannulation (D group) and nondecannulation (ND group) groups and compared. RESULTS: In total, 184 patients who underwent tracheostomy were analyzed (median age at operation: 0.5 y). The major indication for tracheostomy was irreversible neuromuscular impairment (46%). Surgery-related and overall mortality rates were 1% and 25%, respectively, while the successful decannulation rate was 21%. No significant difference in surgical indications or comorbidities was observed between the D (n = 39) and ND (n = 50) groups, except for infection (7 in D group versus 0 in ND group; P = 0.002) and chromosome-gene disorder (15% versus 34%; P = 0.04). The ND group had a significantly higher mortality rate than the D group (46% versus 3%; P < 0.0001). The median time to decannulation was 3.6 years, while that for infection was 0.7 y. CONCLUSIONS: Patients who underwent tracheostomy at our institution due to temporary infections achieved more successful and earlier decannulation compared to other indications. Chromosome-gene disorder as a comorbidity can negatively affect decannulation.

Idiopathic pneumoperitoneum without gastrointestinal perforation in a low-birth weight infant: A rare type of air leak syndrome.

Neonatal free air on X-ray images is generally due to intestinal perforation, and requires surgical intervention. However, some cases without intestinal perforation show free air on X-ray images. Pneumoperitoneum without perforation is caused by an air leak syndrome. We present here the case of a low-birth-weight infant with free air on X-ray images, who had no evidence of intestinal perforation intraoperatively.

Long-term follow-up for anorectal function after anorectoplasty in patients with high/intermediate imperforate anus: a single center experience.

PURPOSE: The aim of this study was to evaluate the clinical outcomes and postoperative anorectal function in the patients with high/intermediate imperforate anus (HIA/IIA) treated in our institution. In addition, we report our novel procedure, laparoscopically assisted anorectoplasty with anterior perineal incision (LAARP-API), which allows direct approach to the anterior edge of the puborectal sling and muscle complex, and is useful for the creation of a pulling-through route for the rectal pouch. METHODS: From 1976 to 2016, 22 patients with HIA and 43 patients with IIA underwent sacroperineal/sacroabdominoperineal pull-through anorectoplasty (SP/SAP), Potts procedure (Potts), SP with API (SP-API) or LAARP-API. Clinical data and anorectal function of those patients were retrospectively evaluated using the Japanese clinical score. RESULTS: Of the 22 cases of HIA, 15 were treated by SAP, 2 were SP and 5 were LAARP-API. Of the 43 cases of IIA, one was treated by SAP, 31 were SP, two were Potts and nine were SP-API. The mean score of anorectal function of HIA/IIA both increased with age. In IIA, the score after SP-API was significantly higher than the score after SP. CONCLUSION: Long-term outcomes of our anorectoplasty for HIA/IIA are good with excellent anorectal function score.

Unexpected gap between intraoperative caliber change of the intestine and normoganglia in patients with intestinal aganglionosis.

PURPOSE: Intestinal aganglionosis (IA) is so rare that the entity remains unclear. The aim of the present study was to compare the outcomes of patients with IA and those with total colonic aganglionosis (TCA). METHODS: The hospital records were retrospectively reviewed from 1977 to 2018. Outcomes were analyzed for the IA group and the TCA group, including clinical presentation, initial management, and operative details. RESULTS: There were six patients were managed in IA (all male) and seven patients in TCA (4 male). The median age at the first operation was significantly younger in IA than TCA (2 days vs 24 days, p = 0.01). The gap between the intraoperative caliber change (CC) of the intestine and the initial stoma location was not significantly different (7.5 cm vs 12 cm, p = 0.61), but the rate of stoma dysfunction was significantly higher in IA (83% vs 0%, p = 0.005). The gap between the CC and the ganglionated bowel was significantly longer in IA (85 cm vs 10 cm, p = 0.003). CONCLUSION: Patients with IA appear to have a high risk for stoma dysfunction after the first operation because of the unexpected gap between the CC and normoganglia. The initial location of the stoma requires careful consideration.

Non-operative management of congenital tracheal stenosis: criteria by computed tomography.

OBJECTIVES: Whether to perform surgical or conservative treatment for congenital tracheal stenosis (CTS) is controversial. Thus, the computed tomography (CT) criteria for conservative treatment of CTS were investigated. METHODS: From 2005 to 2017, 28 CTS cases were included. The operative cases and preoperative death cases constituted the required intervention group (group I), and the non-operative surviving cases constituted the observation group (group O). The diameter of the tracheal narrowest part (DTNP) on CT was evaluated as a criterion for non-operative follow-up. RESULTS: Chest CT was performed 19 times in 19 group I cases and 18 times in 9 group O cases. The median age of the patients that underwent CT scan examinations was 3.4 months (range 0-25 months) in group I and 22 months (range 0-60 months) in group O. The cut-off values of the non-operative criteria were 40.8% (AUC: 0.82, p < .01) normal for age of the trachea's narrowest part, and 41.6% normal for body weight (AUC: 0.92, p < .01), respectively. CONCLUSIONS: DTNP is 40% and more of the normal diameter appears necessary for non-surgical management. The present study suggests that the criteria for conservative management of CTS are that the DTNP is not less than 40% of the normal tracheal diameter, with a few symptoms.

Is a nasogastric tube necessary after transumbilical laparoscopic-assisted appendectomy in children with perforated appendicitis?

BACKGROUND: This study assessed the necessity of postoperative nasogastric tube (NGT) use in acute pediatric cases of perforated appendicitis. METHODS: All cases of acute pediatric perforated appendicitis managed with transumbilical laparoscopic-assisted appendectomy at our hospital from 2011 to 2017 were retrospectively reviewed. Sixty-two cases were selected and divided into two groups based on NGT placement. RESULTS: There were no significant differences between the two groups in most parameters of patient demographics, or surgical data. Notably, the mean time to first oral intake and to regular diet was significantly shorter in no-NGT group (1 day vs 3 days, P < 0.0001; and 4 days vs 7 days, P = 0.003, respectively). Postoperative length of stay was significantly shorter in no-NGT group (7 days vs 9 days, P < 0.0001). CONCLUSION: Considering the results of our analysis, we believe that routine NGT placement is not always necessary in these situations.

Single-incision laparoscopic gastropexy for mesentero-axial gastric volvulus.

BACKGROUND: Mesentero-axial gastric volvulus (MAGV) is a rare but critical condition as delay in treatment can lead to lethal situations. Although the report of the surgical treatment with laparoscopic approach for MAGV has recently come to be seen, no standard procedures have been established. We aim to describe our operative technique of single-incision laparoscopic gastropexy (SILG) for MAGV and review the relevant literature to ascertain the most appropriate treatment option in these patients. CASE PRESENTATION: Three patients were referred to our hospital because abdominal pain and vomiting suddenly occurred. Acute MAGV was diagnosed by upper gastrointestinal study. After overnight gastric decompression with a nasal tube, the GV was resolved spontaneously. Elective SILG was planned. Single incision at the umbilicus was made and three 5-mm trocars were inserted. The anterior wall of the body of the stomach was sutured to the peritoneum using 6 × 4-0 non-absorbable sutures for prevention of recurrence of GV and occurrence of internal hernia through the space created between the sutures. Two boys and a girl with mean age 4 ± 2 years underwent SILG. The mean time of the operation was 48 ± 23 min. All of the procedures were completed safely, and there were no postoperative complications. The mean time of postoperative hospitalization was 4 ± 1 days. All patients had good cosmetic and clinical results. CONCLUSION: We found SILG is a safe, technically feasible, and minimally invasive approach with low incidence of postoperative complication and the best cosmetic result for the patients with MAGV.

Characteristics, management, and outcomes of congenital biliary dilatation in neonates and early infants: a 20-year, single-institution study.

BACKGROUND: The aim of the present study was to investigate the characteristics, management, and outcomes of congenital biliary dilatation (CBD) in neonates and infants (<1 year old) in a single institution over the past 20 years. METHODS: From 1997 to 2016, 21 patients <1 year old underwent definitive surgery for CBD. Open surgery (OS) was performed between 1997 and 2008, and laparoscopic surgery (LS) has been performed since 2009. RESULTS: The bile duct showed cystic dilatation in all patients. Sixteen (76.2%) of the 21 patients were diagnosed prenatally, and the incidence increased with time (OS 63.6%, LS 90%). Fourteen patients (66.7%) were symptomatic before surgery, with jaundice in 11 (52.4%), acholic stool in seven (33.3%), and vomiting in three (14.3%). There were no significant differences in operation time and blood loss, but the postoperative fasting period and hospital stay were significantly shorter in the LS group (P < 0.05). There were no intraoperative complications, but there was one postoperative early complication in one LS group patient, who had bile leakage and was treated with redo hepaticojejunostomy. CONCLUSION: The incidences of prenatally diagnosed and asymptomatic patients increased with time. Although longer follow-up is needed, LS for CBD could be safely performed even in neonates and early infants.

Microlaryngoscopic surgery for pyriform sinus fistulas in children: a report of two cases.

BACKGROUND: A pyriform sinus fistula (PSF) is a rare branchial anomaly that causes recurrent cervical infections. Open neck surgery has widely been accepted as a definitive treatment for PSFs, and endoscopic surgery has been reported in recent years. However, both approaches are not satisfactory because of high recurrence rates and postoperative complications. Microlaryngoscopic surgery (MLS) is a transoral surgical technique involving the use of an operating microscope. In this report, we present a new procedure involving MLS for resection and closure of a PSF without a skin incision. CASE PRESENTATION: Technique: MLS was performed under general anesthesia with endotracheal intubation. The patient was placed in the supine position, and a direct laryngoscope was inserted to expose the pyriform sinus, which was then magnified using an operating microscope. The mucosal layer was carefully resected using scissors and cupped forceps with sharp edges. The fistula was securely sutured using absorbable suture material. Case 1: A 9-year-old boy with recurrent neck abscesses since 8 years of age presented to our hospital after receiving antibiotics and undergoing drainage in other hospitals. After admission to our hospital, barium esophagography and oral contrast coronal computed tomography showed a PSF on the left side, and open neck surgery was performed to resect the fistula. He was eventually discharged from the hospital without any problem. However, a PSF recurred 2 weeks later. As reoperation with the cervical approach was considered difficult owing to severe adhesions, we adopted MLS as a definitive operation. The postoperative course was uneventful. No recurrence was observed during an 18-month follow-up. Case 2: A 10-year-old girl presented to our hospital with recurrent left-sided neck swelling since 6 years of age. After inflammation control, a PSF was identified on the left side on barium esophagography and computed tomography. MLS was performed safely, and the postoperative course was uneventful. No recurrence was observed during a 10-month follow-up. CONCLUSIONS: MLS allows excellent visualization and effective closure for PSFs, and this approach is suitable for recurrence after open neck surgery. Therefore, MLS might become a first-line treatment for PSFs in children.