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.

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.

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.

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.

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.

Serial intralesional triamcinolone acetonide injections for acquired subglottic stenosis in premature infants.

PURPOSE: Long-term intubation of premature infants sometimes induces acquired subglottic stenosis (SGS), causing glottic or supraglottic problems. These kinds of SGS often require tracheostomy and subsequently make decannulation difficult. The aim of our study was to clarify the efficacy of repeated intralesional steroid injections to the stenosis. METHODS: Six children with acquired SGS, who were treated with triamcinolone acetonide injections to the subglottic space just below the vocal folds between September 2015 and December 2017 were retrospectively reviewed. RESULTS: The patients' mean age was 4.3 (range 1.3-4.4) years, the mean gestational age at birth was 25 (23-28) weeks, and the mean birth weight was 591 (456-734) g. The degree of SGS was grade II in one patient and III in five patients, with both tracheostoma and glottic or supraglottic abnormalities. They generally underwent ten procedures, every 3-4 weeks. In most cases, the patency of the injected space improved by 25-220%, and the symptoms were relieved. One patient achieved decannulation, and another one underwent laryngotracheal reconstruction and decannulation. Two patients started using a speech cannula. There were no severe complications. CONCLUSION: Serial intralesional steroid injections are likely to be effective in improving the patency of acquired SGS.

New prognostic classification and managements in infants with esophageal atresia.

PURPOSE: The aim of this study was to investigate the risk factors for in hospital mortality in infants with esophageal atresia (EA) without critical chromosome abnormality disorders and develop a new prognostic classification to assess the influence of external risk factors on the prognosis of EA, which could provide more effective treatment strategies in clinical practice. METHODS: A retrospective chart review of all consecutive patients between January 1994 and December 2017, which included 65 EA infants who were admitted to Shizuoka Children's Hospital, was conducted. Based on multivariate analysis data and ROC analysis, the discrimination of the new prognostic classification was quantified and compared with that of the Spitz classification using the area under the ROC curve (AUC). RESULTS: Multiple logistic regression analysis showed that birth weight of < 1606 g (adjusted OR, 13.16; 95% CI, 1.16-352.75), and complex cardiac anomalies (adjusted OR 22.39; 95% CI 2.45-569.14) were significant risk factors for death. We have created a new classification close to Spitz classification using the presence of complex cardiac anomalies and birth weight. The mortality rates were 0% for class I (n = 0/40), 7.1% for class II (n = 1/14), 33.3% for class III (n = 3/9), and 100% for class IV (n = 2/2). The AUC of the new classification was better than that of the Spitz classification (0.939 vs 0.812, respectively; p = 0.04). CONCLUSION: New prognostic classification can improve the stratification of EA patients and be a useful predictor of survival.

The fetal lung-to-liver signal intensity ratio on magnetic resonance imaging as a predictor of outcomes from isolated congenital diaphragmatic hernia.

PURPOSE: We investigated the developmental changes in the unaffected contralateral lungs of patients with isolated left-sided congenital diaphragmatic hernia (CDH) using signal intensity ratios on prenatal magnetic resonance imaging (MRI) and determined whether these changes correlated with clinical outcomes. METHODS: We performed 47 fetal MRI screens on 30 patients with isolated left-sided CDH. A cohort of 88 fetuses was selected as the control. We calculated the lung-to-liver signal intensity ratio (LLSIR) using region of interest analysis and compared LLSIR between the groups and between those in the CDH group with good and poor prognoses. RESULTS: In the control group, LLSIR increased as pregnancy progressed [regression line = 2.232 + 0.135 × (GW-23), r = 0.669]. In the CDH group, especially in the poor prognosis group, LLSIR did not significantly increase as pregnancy progressed [regression line for good prognosis = 1.827 + 0.092 × (gestational week-23), r = 0.733; regression line for poor prognosis = 1.731 + 0.025 × (gestational week-23), r = 0.634]. CONCLUSION: Fetal LLSIR on T2-weighted MRI is an accurate marker of fetal lung maturity that correlates with postnatal survival and can potentially be used as a prognostic parameter in CDH management.

Evaluation of developmental prognosis for esophageal atresia with tracheoesophageal fistula.

PURPOSE: This study investigated risk factors for developmental disorders after the repair of esophageal atresia with tracheoesophageal fistula (EA/TEF). METHODS: The clinical charts of all infants with EA/TEF (gross type C) treated at our institution between 1993 and 2013 were analyzed. Patients with chromosomal aberrations were excluded. Forty-seven patients were divided into groups according to the presence (D) or absence (N) of developmental disorders. Patients were assessed with appropriate developmental examinations at age >3 years. RESULTS: Group D comprised 13 patients. The 34 patients in the group N had received a standard education. In groups D and N, gestational age was 36.5 versus 38.5 weeks, birth weight was 1808 versus 2662 g, and congenital cardiac anomalies occurred in 76.9 versus 23.5%. These differences reached significance. Operative data did not differ significantly. Overall complications occurred in 69.2 versus 29.4%, gastroesophageal reflux (GER) requiring fundoplication in 46.1 versus 8.8%, and oral ingestion difficulty in 61.5 versus 14.7%, which were significantly different. The frequencies of anastomotic leakage, anastomotic stricture, and recurrent fistula did not differ significantly. CONCLUSION: Gestation, birth weight, and cardiac anomalies could be risk factors for developmental disorders. Moreover, overall complications, GER, and oral ingestion may affect development.

Risk factors and prevention for surgical intestinal disorders in extremely low birth weight infants.

PURPOSE: Surgical intestinal disorders (SID), such as necrotizing enterocolitis (NEC), focal intestinal perforation (FIP), and meconium-related ileus (MRI), are serious morbidities in extremely low birth weight (ELBW, birth weight <1000 g) infants. From 2010, we performed enteral antifungal prophylaxis (EAP) in ELBWI to prevent for SID. The aim of this study was to identify disease-specific risk factors and to evaluate the efficacy of prevention for SID in ELBW infants. METHODS: A retrospective chart review of all consecutive patients between January 2006 and March 2015, which included 323 ELBW infants who were admitted to Shizuoka Children's Hospital, was conducted. RESULTS: The number of infants with NEC, FIP, and MRI was 9, 12, and 13, respectively; 28 in 323 ELBW infants died. The control group defined the cases were not SID. In-hospital mortality was higher in infants with NEC relative to those in the control group. On logistic regression analysis, low gestational age and cardiac malformations were associated with increased risk of NEC. IUGR were associated with increased risk of MRI. EAP decreased risk of NEC and FIP. Low gestational weight and NEC were associated with increased risk of death. CONCLUSION: Survival to hospital discharge after operation for NEC in ELBW infants remains poor. EAP decreased risk of NEC and FIP in ELBW infants.

Laparoscopic resection for retroperitoneum ganglioneuroma with Supine hypotension syndrome.

BACKGROUND: Supine hypotension syndrome (SHS) has been reported to occur due to compression by a giant tumor such as ovarian tumor. We herein report a case of retroperitoneal ganglioneuroma with SHS treated with laparoscopic resection. CASE PRESENTATION: The patient was an 11-year-old male with right-sided abdominal pain. He had a pale complexion and tachycardia while falling asleep. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a giant mass lesion (60 × 35 mm) with compression of the inferior vena cava (IVC) and duodenum ventrally and the right kidney caudally. The IVC was flattened by mass compression. Abdominal ultrasonography (US) revealed narrowing of the IVC due to the mass and accelerated blood flow after IVC stenosis in the supine and left lateral recumbent position. His pale complexion and tachycardia while falling asleep was thought to be due to decreased venous return caused by the tumor compressing the IVC, resulting hypotension. 123I-MIBG scintigraphy revealed no abnormal findings. Tumor markers were normal. He was diagnosed with SHS due to a right adrenal gland tumor. The tumor compressed the IVC from the dorsal side, and hemostasis was expected to be difficult during bleeding. Therefore, a guidewire was inserted from the right femoral vein into the IVC for emergency balloon insertion during bleeding. A laparoscopic tumor resection was performed. A histopathological examination confirmed the diagnosis of primary retroperitoneal ganglioneuroma. CONCLUSIONS: The treatment of symptomatic retroperitoneal tumors requires a multidisciplinary approach.

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.

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.

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.