Current characteristics and management of congenital esophageal stenosis: 40 consecutive cases from a multicenter study in the Kyushu area of Japan

Purpose

Congenital esophageal stenosis (CES) is rare, and the available clinical data are limited. We explored the current diagnosis, treatment and outcomes of CES.

Methods

A questionnaire survey was performed using medical records at pediatric surgical centers in the Kyushu area.

Results

Over 10 years, 40 patients (24 males) had CES. The incidence of associated anomalies was 52.5% (21/40), and that of esophageal atresia was 20.0% (8/40). The mean age at the diagnosis was 12.0 months (range, 1 day–8.8 years). Seven (17.5%) patients were diagnosed in the neonatal period. Ten (25.0%) developed CES due to tracheobronchial remnants, 27 (67.5%) due to fibromuscular stenosis (FMS) and 1 (2.5%) due to membranous stenosis + FMS. Thirty-six (90.0%) were treated by balloon dilatation (mean, 3 times; range, 1-20). Perforation at dilatation occurred in 7 (17.5%) patients, and all were diagnosed with FMS. Eighteen (45.0%) patients underwent radical operation (3 primary, 15 secondary to dilatation).

Conclusions

Our study clarified the characteristics and outcomes of CES, including neonatal diagnoses. CES occurred in 1 in every 33,000 births in the Kyushu area. Careful attention should be paid, even in cases of dilatation for FMS. CES requires long-term follow-up for symptom persistence after adequate and repeated treatment.

     

Steroid pulse therapy for Kawasaki disease complicated with myocarditis

Background

The clinical management of intravenous immunoglobulin-resistant Kawasaki disease shock syndrome (KDSS) is obscure.

Case characteristics

Three children presented with intravenous immunoglobulin-resistant KDSS complicated with myocarditis.

Outcome

All cases were successfully managed with steroid pulse therapy.

Message

Steroid pulse therapy is effective in immunoglobulin-resistant KDSS.

     

Preservation of native esophagus in infants with pure esophageal atresia has good long-term outcomes despite significant postoperative morbidity

Purpose

To evaluate long-term outcomes of pure esophageal atresia (EA) repair with preservation of native esophagus.

Methods

Infants with pure EA treated at our institution (2000–2010) and with minimum 5-year follow-up were reviewed (REB:1000046653). Data analysed included demographics, management and outcomes and are reported as mean ± SD/median (range).

Results

Of 185 infants with EA, 12 (7 %) had pure EA (gestational age: 36 ± 2.4 weeks, birth weight: 2353 ± 675 g). Ten had associated anomalies, including trisomy-21 (n = 2) and duodenal atresia (n = 1). Surgery: 1 patient (short gap) underwent primary thoracoscopic anastomosis, 11 had gastrostomy (Stamm, n = 5; image-guided, n = 6) as initial procedure. At definitive repair (age: 128 ± 91 days; weight 5.5 ± 2.3 kg): ten had primary anastomosis and 1 had Collis gastroplasty. No patient had esophageal replacement surgery. Outcomes: three patients had gastrostomy dehiscence requiring re-operation. At post-operative esophagram, seven had anastomotic leak successfully treated conservatively. Seven patients developed strictures requiring balloon dilatations (median two dilatations, range 1–10), six received antireflux surgery. At 7-year follow-up (range 5–15 years), all patients had the gastrostomy closed and were on full oral feeds.

Conclusions

The management of pure EA continues to be challenging. The preservation of native esophagus is possible with significant morbidity. The long-term outcomes are favourable.

     

Favorable Renal Outcome of Japanese Children with Severe IgA Nephropathy With Nephrotic Syndrome

Background

Nephrotic syndrome is a rare but severe feature of IgA nephropathy.

Case characteristics

Nine Japanese children with severe IgA nephropathy with nephrotic syndrome.

Intervention

All received low-dose intravenous methylprednisolone (IVMP) within five weeks after the disease onset. Eight out of nine patients achieved resolution of proteinuria without severe adverse events.

Message

Early low-dose intravenous methylprednisolone may be safe and effective for children with severe IgA nephropathy with nephrotic syndrome.

     

Esophagoesophagopexy technique for assisted fistulization of esophageal atresia

Purpose

We describe our experience using a modified suture fistula technique for addressing tension in longer gap esophageal atresia (EA). Esophagoesophagopexy (EEP) is the tacking of the proximal and distal ends of esophageal pouches without formal anastomosis. In this retrospective cohort, we review the outcomes of patients with EA after EEP.

Methods

We reviewed the operative reports of EA cases treated at our institution from 1997 to 2016 and identified all patients described as having EEP.

Results

Of 129 EA cases, five patients underwent EEP. Formal anastomosis was not done due to patient’s instability, prematurity, or long gap. Median birth weight was 1.4 kg (0.6–2.2 kg), and median gestational age at birth was 29 weeks (25–34 weeks). Age at time of EEP ranged 0–5 months. Esophagoesophageal fistula was confirmed in three patients. All three had strictures requiring weekly dilations. One of these patients died. The two surviving patients underwent fundoplication.

Conclusion

We describe an alternative technique for esophageal anastomosis in patients for whom a standard anastomosis is not possible. EEP can lead to a functional anastomosis through fistulization and avoid the morbidity of multiple thoracotomies and lengthening procedures. Families should be educated on the potential need for dilations and antireflux procedures.

     

Management of congenital tracheal stenosis in the neonatal period

Purpose

Few reports have focused on the management of congenital tracheal stenosis (CTS) in the neonatal period. The aim of this study was to determine appropriate management strategies for CTS in the neonatal period.

Methods

The medical records of eight neonatal patients with CTS at a single institution between January 2007 and December 2016 were retrospectively reviewed.

Results

Three patients with frequent ventilatory insufficiency despite assisted ventilation underwent surgical intervention (balloon tracheoplasty: n = 1, slide tracheoplasty: n = 2). Ventilatory insufficiency improved after surgery in all three patients. One patient who underwent slide tracheoplasty died due to non-airway-related causes. Observation or conservative management was performed in five patients with minimal respiratory symptoms or stable ventilation under assisted ventilation. All five patients were safely managed non-operatively in the neonatal period.

Conclusion

Depending on the severity of ventilatory insufficiency, there are two management strategies for CTS in the neonatal period. Surgical intervention, such as balloon tracheoplasty or slide tracheoplasty, is indicated for patients with unstable ventilatory status despite assisted ventilation. Observation or conservative management is a more suitable option for neonates with stable ventilation.

     

Surgical techniques for esophageal replacement in children

Purpose

Surgical techniques for esophageal replacement (ER) in children include colon interposition, gastric tube, gastric transposition, and jejunal interposition. This review evaluates the merits and demerits of each.

Method

Surgical techniques, complications, and outcome of ER are reviewed over last seven decades.

Results

Colon interposition is the time-tested procedure with minimal and less serious complications. Long-term complications include reflux, halitosis, colonic segment dilatation, and anastomotic stricture, sometimes requiring surgical interventions especially for dilatation and reflux. Gastric tube is technically more risky, and associated with early serious complications like prolonged leak in neck or mediastinum, graft necrosis, and ischemia leading to stricture of the tube. Long-term results are good. Gastric transposition is much simpler, can be performed in emergency and in newborns. It involves a single anastomosis in the neck. Post-operative complications include gastric stasis, bile reflux, restricted growth, and decreased pulmonary functional capacity. Jejunal interposition has not been used extensively due to short mesentery but long-term results are good in expert hands.

Conclusion

Colon is the most preferred and safest organ for ER. Stomach is a vascular and muscular organ with lower risk of ischemia. Gastric tube is a demanding technique. Jejunum or ileum is alternative for redo cases.

     

Dilations of anastomotic strictures over time after repair of esophageal atresia

Aim of the study

Anastomotic strictures commonly occur in patients undergoing surgery for esophageal atresia (EA). The primary aim of this study was to determine the age distribution of dilation procedures for anastomotic strictures over the patient’s childhood after reconstruction of EA. The secondary aim was to evaluate the effect of postoperative proton pump inhibitors (PPIs) on the frequency of dilations.

Methods

This observational study was conducted at a single tertiary center of pediatric surgery. The times that dilations of strictures were performed were assessed during three study periods: 1983–1995, 2001–2009, and 2010–2014. PPIs were not used during the first period, and then, respectively, for 3 and 12 months postoperatively. The indications for dilation were signs of obstruction and/or radiological signs of stricture.

Primary results

A total of 131 children underwent esophageal reconstruction, and of those, 60 (46%) required at least 1 dilation procedure for strictures. There were no differences in the frequencies of dilation procedures between the three study periods (28/66, 18/32 and 14/33, respectively; P = 0.42). The overall median number of dilations per patient was 3 (range 1–21) with no differences between the study periods. The differences between ages at which the first dilation was performed during each study period were significant, as follows: 7, 2, and 8 months, respectively (P = 0.03). Fiftyone percent of all dilation procedures were performed during the first year of life, 16% during the second year, and 33% during years 2–15. Four children (2%) underwent >12 dilations.

Conclusion

The first year of life was the time of greatest need for dilation of AS after reconstruction of EA; however, dilations were also performed several years later. PPIs did not affect the frequency of dilations during the first year of life.

     

Deep sedation in pediatric imaging: efficacy and safety of intravenous chlorpromazine

Background

The quality of MRI and CT depends largely on immobility of the patient during the procedure, which is often difficult to achieve without sedation in children below the age of 6 years.

Objective

To assess the efficacy and safety of intravenous chlorpromazine sedation for repeated imaging in young children treated for cancer.

Materials and methods

From July 2003 to January 2007, information on children younger than 6 years of age having MRI or CT was prospectively collected. Forty-five minutes before the scan, a 10-min infusion of chlorpromazine 0.5 mg/kg was administered and managed by non-anesthetic staff. Patient monitoring included continuous measurement of pulse, respiration, oxygen saturation and arterial blood pressure. Procedure-related parameters and adverse events were documented. Sedation was considered successful when the procedure was completed and at least 95% of images were usable.

Results

One-hundred-one procedures (82 MRI, 19 CT) were evaluated in 62 children, 3–74 months old. Adequate sedation was achieved in 96% of cases, with mean induction time, 22 min; mean duration of sleep, 72 min, and mean duration of procedure, 33 min. Mean time spent in the radiology unit was 104 min. Ninety-six percent of imaging procedures were successfully completed. No cardiac, respiratory, neurological or allergic complication occurred.

Conclusion

Intravenous chlorpromazine is safe and effective for procedural sedation in young children with cancer undergoing MRI and CT.

     

Airway reconstruction using decellularized tracheal allografts in a porcine model

Purpose

Tracheal cartilage reconstruction is an essential approach for the treatment of tracheal congenital abnormalities or injury. Here, we evaluated the use of allogeneic decellularized tracheas as novel support scaffolds.

Methods

Six weaned pigs (4-week-old domestic males) were transplanted with allogeneic tracheal graft patches (three decellularized and three fresh tracheal scaffolds) onto artificial defects (approximately 15 × 15 mm). After 11 weeks, the tracheas were evaluated by bronchoscopy and histological studies.

Results

No pigs displayed airway symptoms during the observation period. Tracheal lumen restored by fresh graft patches showed more advanced narrowing than that treated with decellularized grafts by bronchoscopy. Histologically, fresh grafts induced typical cellular rejection; this was decreased with decellularized grafts. In addition, immunohistochemistry demonstrated regenerating foci of recipient cartilage along the adjacent surface of decellularized tracheal grafts.

Conclusion

Decellularized allogeneic tracheal scaffolds could be effective materials for restoring impaired trachea.

     

Surgical intervention strategies for congenital tracheal stenosis associated with a tracheal bronchus based on the location of stenosis

Purpose

The aim of this study was to determine the appropriate surgical intervention strategies for congenital tracheal stenosis (CTS) associated with a tracheal bronchus based on the location of stenosis.

Methods

The medical records of 13 pediatric patients with CTS associated with a tracheal bronchus at a single institution between January 2006 and December 2015 were retrospectively reviewed.

Results

Type 1: tracheal stenosis above the right upper lobe bronchus (RULB) (n = 1). One patient underwent slide tracheoplasty and was successfully extubated. Type 2: tracheal stenosis below the RULB (n = 7). Tracheal end-to-end anastomosis was performed before 2014, and one patient failed to extubate. Posterior–anterior slide tracheoplasty was performed since 2014, and all three patients were successfully extubated. Type 3: tracheal stenosis above the RULB to the carina (n = 5). One patient underwent posterior–anterior slide tracheoplasty and was successfully extubated. Two patients with left–right slide tracheoplasty and another two patients with tracheal end-to-end anastomosis for the stenosis below the RULB could not be extubated.

Conclusion

Tracheal end-to-end anastomosis or slide tracheoplasty can be selected for tracheal stenosis above the RULB according to the length of stenosis. Posterior–anterior slide tracheoplasty appears feasible for tracheal stenosis below the RULB or above the RULB to the carina.

     

The value of contrast studies in the evaluation of bowel strictures after necrotising enterocolitis

Purpose

Strictures of the bowel are a frequent complication post-necrotising enterocolitis (NEC). Contrast studies are routinely performed prior to stoma closure following NEC. The aim of this study was to evaluate the ability of these studies to detect strictures and also directly compare them to operative and histological findings.

Methods

Two hundred and fourteen neonates who had a diagnosis of NEC (Bell stage 2 or greater) in a single unit (2007–2011) were analysed. Their case notes, radiology, and histology were reviewed.

Results

One hundred and sixteen neonates underwent an emergency laparotomy and 77 had stomas fashioned. Sixty-six patients had a contrast study prior to stoma closure (distal loopogram 18, contrast enema 37, both studies 11). Colonic strictures were reported in 18 patients and small bowel strictures were reported in two patients. Fourteen of these colonic strictures were confirmed at operation and on histology but three colonic strictures were missed on contrast studies; one patient had had both contrast studies and the other two only a distal loopogram. Two small bowel strictures reported were confirmed and an additional small bowel stricture missed on distal loopogram was also detected at the time of operation. The incidence of post-op strictures was 19 out of 68 patients (27.9 %) and 16 (84.2 %) of these strictures were found in the colon. Contrast enemas had a much higher sensitivity for detecting post-NEC colonic strictures than distal loopograms; 93 versus 50 %, respectively; however, they are more likely to give a false positive result and therefore their specificity is lower; 88 versus 95 %, respectively.

Conclusion

Colon is the commonest site for post-NEC stricture and contrast enema is the study of choice for detecting these strictures prior to stoma closure.

     

Age Specific Peak Expiratory Flow Nomogram in Children

Objective

To determine peak flow rate nomogram in children between 5 to 12 y of age in suburban population.

Methods

This was a cross-sectional study randomly done on 5000 healthy school children. Mini-Wright flow meter was used for measurement of peak expiratory flow rate (PEFR). The range of age, weight and height were defined and linear and multiple regression analysis were performed.

Results

Correlation between PEFR and height was more significant and between PEFR and weight was lower. On the basis of height, the regression equation for both sexes were drawn; Boys: PEFR = 3.29 (Height in cm) - 218.38; Girls: PEFR = 3.25 (Height in cm) - 216.49.

Conclusions

Thus, region specific nomograms for PEFR for assessing the severity and monitoring of airway obstruction in relation to age, sex, weight and height of children can be determined.

     

Pediatric Budd-Chiari Syndrome: A Case Series

Objective

To study the diagnostic methods and treatment outcomes in children with Budd-Chiari syndrome.

Methods

Case records of 25 patients with Budd-Chiari syndrome were evaluated retrospectively. These patients were investigated with imaging techniques and underwent balloon angioplasty or surgical management.

Results

21 patients underwent balloon angioplasty, of which 17 had good medium- to long-term results, while only one out of four patients who underwent a portocaval shunt survived.

Conclusion

The balloon angioplasty has satisfactory outcome in the treatment of acute Budd-Chiari syndrome. In failed cases, the surgical therapy may be attempted, but the outcomes do not appear rewarding.

     

Psychosoziale Gesundheit von Kindergartenkindern im Urteil von Eltern und Pädagogischen Fachkräften

Background

Psychosocial health is of major importance for kindergartener’s development.

Objective

The aim of the study was to examine correlations between developmental and health problems and behavioral problems. Additionally, we examined inter-rater reliability between parent’s and kindergarten teacher’s ratings on developmental and health problems. The total sample consisted of 1036 kindergarteners aged 3 to 6 years.

Materials and methods

A questionnaire was assessed by both parents and kindergarten teachers. The developmental and the health status, as well as the child’s behavior over the past four weeks, were addressed.

Results

Statistically significant correlations were found between developmental and health problems and behavioral problems. Inter-rater reliability varies by questionnaire scales.

Conclusions

Results stress the need for integration of various judgments of different information sources and plead for a multiperspective elicitation of psychosocial health in kindergarteners.

     

Outcomes of laparoscopic nissen fundoplications in children younger than 2-years: single institution experience

Background

Variation exists in the diagnostic testing for gastroesophageal reflux (GER) in infants and in the application of surgical therapy. There has been an increase in antireflux surgery (ARS) since the development of laparoscopy but the outcomes in high-risk infants is unclear. This study examines the results of laparoscopic fundoplication in infants less than 2 years.

Methods

The results of infants less than 2 years undergoing laparoscopic Nissen fundoplication (Lap-F) from 2012 to 2015 were retrospectively reviewed and outcomes were followed until 2017.

Results

There were 106 patients, median gestational age 32.50 weeks?±?6.35 SD and non-corrected age at operation 23.0 weeks?±?19.0 SD, mean weight of 4.81 kg?±?2.10 SD. One of the most common reasons for surgical consultation was improvement in respiratory status after insertion of nasoduodenal feeding tube. Of the Lap-F, 100 were with gastrostomy tube (GT). There were no conversions to open or intraoperative complications. The complication rate was 4.71%, and the reoperation rate was 5.66%, one fundoplication revision and the others gastrostomy revisions. The median time for feeds and to reach goal were 1 (1–14) and 4 (2–279) days, respectively. The 30-day mortality was 0.9% and long-term it was 4.71%. The long-term mortality was related to the underlying medical problems. The median follow-up was 113 (3–286) weeks. One patient required revision of the fundoplication and none required esophageal dilatation during the follow-up period.

Conclusion

Fundoplication is effective for relief of symptoms of GER in children younger than 2 years. The procedure has a low morbidity and mortality in this population.

     

Predictive factors affecting the prognosis and late complications of 73 consecutive cases of esophageal atresia at 2 centers

Purpose

While the diagnosis and outcomes of esophageal atresia (EA) have improved, associated anomalies, the management of late complications and growth remain major issues. We analyzed factors that affected the prognosis, late complications and growth.

Methods

We retrospectively reviewed EA patients treated at two centers from 1984 to 2016. Patient characteristics, complications (gastroesophageal reflux [GER], anastomotic stenosis, tracheomalacia, dysphagia) and growth were evaluated.

Results

Seventy-three EA patients were treated (overall survival rate:80.8%). The mean birth weight was 2514?±?509 g in the surviving group, and 2453?±?567 g in the fatal group excluded chromosomal abnormality (p?=?0.76). Cardiac and chromosomal anomalies significantly affected mortality. Postoperative GER and anastomotic stenosis each occurred in 39% of the patients. Only GER was significantly affected by the Gross classification. The standard deviation (SD) values of the EA patients’ growth were all lower than in the normal population. The SD of body weight was significantly lower in patients with extremity anomalies.

Conclusions

Associated cardiac and chromosomal anomalies significantly affected the prognosis. GER and anastomotic stenosis were the most common late complications. The growth of the surviving cases was insufficient. These factors will help optimize the therapeutic strategies and postoperative management for EA.

     

Impact of botulinum toxin A injection on esophageal anastomosis in a rabbit model

Purpose

The management of esophageal atresia is established, but the rate of postoperative complications remains high. We focused on a new, recently reported method of esophageal elongation using botulinum toxin type A (BTX-A) and evaluated the efficacy of BTX-A injection around esophageal anastomoses with tension in a rabbit model.

Methods

Twenty rabbits aged 8–10 weeks and weighing 1.27–1.72 kg underwent resections of the esophagus measuring 1.5 cm long using an anterior cervical approach. Esophagoesophagostomies were performed after intramural administration of Xeomin? (3 U/body) in the BTX-A group and saline in the control group. Morphological and histological evaluations were examined on postoperative day 14.

Results

Six rabbits in each group survived. The BTX-A group showed significantly less postoperative anastomotic stricture and less fibrosis than the control group. Changes in wall thickness on both sides of the anastomotic areas were equivalent between the two groups, and no muscle fracturing was observed.

Conclusion

Local administration of BTX-A for esophagoesophagostomy significantly reduced postoperative anastomotic stricture with less fibrosis than that observed in the control group. Reduced anastomotic tension with BTX-A presumably contributed to better anastomotic healing. Determining the optimum dose of BTX-A is necessary for clinical application.

     

Comparison of Four Days Versus Seven Days Duration of Antibiotic Therapy for Neonatal Pneumonia: A Randomized Controlled Trial

Objective

To compare the effect of 4 day course (study group) with 7 day course (control group) of antibiotic treatment in neonatal pneumonia, on treatment success rate.

Methods

This randomized controlled trial was conducted in a tertiary teaching hospital. Seventy, term and near-term neonates with pneumonia who had clinical remission by 48 h of antibiotic therapy were included. The neonates were randomized to receive a total of 4 d of antibiotics (Group 1) or 7 d of antibiotics (Group 2). The outcome measure was treatment failure in each group within 3 d of discharge.

Results

The treatment success rate of both the groups was 100%. There was a significant reduction in the duration of hospital stay (p?<?0.001), antibiotic usage (p?<?0.001), and cost (p?<?0.001) in the 4 d group. On follow up till 28 d of enrollment, no infective morbidity was found in either group.

Conclusions

For term and near-term neonates who become clinically asymptomatic within 48 h of antibiotic therapy, 4 d of antibiotic therapy is as effective and safe as 7 d of antibiotic therapy, with significant reduction in hospital stay, antibiotic usage and cost.

     

Neonatal Mucormycosis with Gastrointestinal and Cutaneous involvement

Background

Mucormycosis of the gastrointestinal tract is a rare fungal infection of neonates.

Case characteristics

48-hours-old term neonate presented with intestinal obstruction and perforation. No significant risk factors were present. Histopathological examination of the resected gangrenous bowel revealed mucormycosis. Cutaneous involvement due to systemic spread led to dermal necrosis in toes.

Outcome

Though cutaneous lesions responded promptly to antifungal therapy, gastrointestinal manifestations required multiple antifungal therapy for prolonged period apart from surgical debridement.

Message

Precise histopathological diagnosis and early appropriate therapy can prevent dismal outcomes in neonatal mucormycosis.