Optimal timing for Soave primary pull-through in short-segment Hirschsprung disease: A meta-analysis.

BACKGROUND: The optimal age for endorectal pull-through (ERPT) surgery in infants with short-segment Hirschsprung disease varies, with a trend toward earlier surgery. However, it is unclear if the timing of surgery impacts functional outcomes. We undertook the present study to determine the optimal timing of ERPT in infants with short-segment Hirschsprung disease. METHODS: The NCBI PubMed database was searched for English-language manuscripts published between 2000 and 2019 analyzing functional outcomes for patient following the initial Soave ERPT for short-segment Hirschsprung disease. Raw data from these studies was obtained from the corresponding author for each manuscript. We combined data from these papers with our own institutional data and performed a meta-analysis. RESULTS: A total of 780 infants were included in our meta-analysis. Constipation occurred in 1.0-31.7%, soiling 1.3-26.0%, anastomotic stricture 0.0-14.6%, and anastomotic leak 0.0-3.4%. Regarding age at ERPT, younger infants at the time of initial corrective surgery had higher rates of soiling, stricture, and leak. On sub-group analysis, patients <2.5 months at their initial corrective surgery had higher rates of soiling (25.9% vs. 11.4%, p<0.01), as well as stricture (10.0% vs 1.7%, p<0.01) and leak (5.5% vs 1.3%, p<0.01). CONCLUSION: While age at Soave endorectal pull-through for short-segment Hirschsprung disease has decreased over time, functional outcomes associated with this trend have only recently been examined. Our findings suggest that patients <2.5 months old at the time of endorectal pull-through may have worse functional outcomes, emphasizing the need to consider further study of the timing of surgery in this population.

Thoracic epidural-based Enhanced Recovery After Surgery (ERAS) pathway for Nuss repair of pectus excavatum shortened length of stay and decreased rescue intravenous opiate use.

BACKGROUND: PCA- and block-based enhanced recovery after surgery (ERAS) pathways have been shown to decrease hospital length of stay (HLOS) and opiate use following Nuss Repair for Pectus Excavatum (NRPE). No thoracic epidural-based ERAS pathway has demonstrated similar benefits. METHODS: In this pre-post single-center study, data were retrospectively collected for patients ≤ 21 years undergoing NRPE from May 2015 to August 2019. Univariate and multivariate methods were used to evaluate whether implementation of a thoracic epidural-based ERAS in April 2017 was associated with HLOS, opiate use, or pain scores. RESULTS: There were 110 patients: 35 pre- and 75 post-ERAS. HLOS decreased from median 4.8 (1.1) to 3.3 (0.6) days with ERAS (p < 0.001). Use of rescue intravenous opiates decreased from 35.3% pre- to 9.3% with ERAS (p = 0.013). When adjusted for baseline characteristics, ERAS was associated with a 1.3 ± 0.2 day decrease in HLOS and 0.188 times the odds of rescue intravenous opiate use (p = 0.011). CONCLUSIONS: Pain scores, ED visits, and readmissions did not change with ERAS (p > 0.05). Implementation of a thoracic epidural-based ERAS following NRPE was associated with decreased HLOS and need for any rescue intravenous opiates without a change in pain scores, ED visits, or readmission.

Surgical options in the treatment of ulcerative colitis.

Children and young adults with ulcerative colitis tend to present with more extensive colonic disease than an adult population. The need for surgical intervention in the pediatric population with ulcerative colitis occurs earlier after diagnosis and has a greater incidence than a comparably matched adult population with an estimated need for colectomy at 5 years following diagnosis of 14-20%. Perhaps, even more than the adult population, there is a desire to restore intestinal continuity for the pediatric patient to achieve as healthy and normal quality of life as possible. With surgery playing such a prominent role in the treatment of ulcerative colitis in this age group, an understanding of the surgical treatment options that are available is important. The surgeon's awareness of the complexities of the different operations associated with proctocolectomy and reestablishing intestinal continuity may help to avoid early complications and minimize the risk of less than ideal long-term outcomes.

Role of percutaneous abscess drainage in the management of young patients with Crohn disease.

BACKGROUND: Intra-abdominal abscess is a common complication of Crohn disease in children. Prior studies, primarily in adults, have shown that percutaneous abscess drainage is a safe and effective treatment for this condition; however, the data regarding this procedure and indications in pediatric patients is limited. OBJECTIVE: Our aim was to determine the success rate of percutaneous abscess drainage for abscesses related to Crohn disease in pediatric patients with a focus on treatment endpoints that are relevant in the era of biological medical therapy. MATERIALS AND METHODS: We retrospectively reviewed 25 cases of patients ages ≤20 years with Crohn disease who underwent percutaneous abscess drainage. Technical success was defined as catheter placement within the abscess with reduction in abscess size on post-treatment imaging. Clinical success was defined as (1) no surgery within 1 year of drainage or (2) surgical resection following drainage with no residual abscess at surgery or on preoperative imaging. Multiple clinical parameters were analyzed for association with treatment success or failure. RESULTS: All cases were classified as technical successes. Nineteen cases were classified as clinical successes (76%), including 7 patients (28%) who required no surgery within 1 year of percutaneous drainage and 12 patients (48%) who had elective bowel resection within 1 year. There was a statistically significant association between resumption of immunosuppressive therapy within 8 weeks of drainage and both clinical success (P < 0.01) and avoidance of surgery after 1 year (P < 0.01). CONCLUSION: Percutaneous abscess drainage is an effective treatment for Crohn disease-related abscesses in pediatric patients. Early resumption of immunosuppressive therapy is statistically associated with both clinical success and avoidance of bowel resection, suggesting a role for percutaneous drainage in facilitating prompt initiation of medical therapy and preventing surgical bowel resection.

Mutations in TBX18 Cause Dominant Urinary Tract Malformations via Transcriptional Dysregulation of Ureter Development.

Congenital anomalies of the kidneys and urinary tract (CAKUT) are the most common cause of chronic kidney disease in the first three decades of life. Identification of single-gene mutations that cause CAKUT permits the first insights into related disease mechanisms. However, for most cases the underlying defect remains elusive. We identified a kindred with an autosomal-dominant form of CAKUT with predominant ureteropelvic junction obstruction. By whole exome sequencing, we identified a heterozygous truncating mutation (c.1010delG) of T-Box transcription factor 18 (TBX18) in seven affected members of the large kindred. A screen of additional families with CAKUT identified three families harboring two heterozygous TBX18 mutations (c.1570C>T and c.487A>G). TBX18 is essential for developmental specification of the ureteric mesenchyme and ureteric smooth muscle cells. We found that all three TBX18 altered proteins still dimerized with the wild-type protein but had prolonged protein half life and exhibited reduced transcriptional repression activity compared to wild-type TBX18. The p.Lys163Glu substitution altered an amino acid residue critical for TBX18-DNA interaction, resulting in impaired TBX18-DNA binding. These data indicate that dominant-negative TBX18 mutations cause human CAKUT by interference with TBX18 transcriptional repression, thus implicating ureter smooth muscle cell development in the pathogenesis of human CAKUT.

Management of congenital tracheal anomalies and laryngotracheoesophageal clefts.

Congenital obstructions and anomalies of the pediatric airway are rare problems that may be associated with mild symptoms or critical stenoses that may be life threatening in the first few days of life. This review provides an overview of the embryologic development of the airway, different congenital anomalies associated with airway development, and surgical correction that may be associated with good long-term outcome.

Restorative proctocolectomy with and without protective ileostomy in a pediatric population.

PURPOSE: This study aim was to review outcomes of pediatric patients after restorative proctocolectomy with or without a protective ileostomy in the treatment of ulcerative colitis and polyposis syndromes. METHODS: All patients who underwent rectal mucosectomy with ileal pouch reservoir and hand-sewn ileal pouch anal anastomosis (IPAA) during 19-year period were reviewed retrospectively. RESULTS: Eighty-three patients with ulcerative colitis and 7 patients with polyposis syndromes (ages 2.0-21.8 years) were reviewed. Sixty-eight patients underwent IPAA without diverting ileostomy. Fifty-six patients underwent restorative proctocolectomy as single-stage procedures, and 12 had abdominal colectomy and subsequent definitive IPAA without diverting ileostomy. Nineteen patients had IPAA with diverting ileostomy and subsequent closure of ileostomy. Three-stage procedures were performed in 3 cases. An ileal pouch leak or pelvic abscess occurred in 2 patients. Surgical pouch revision for retraction, efferent limb syndrome, prolapse, pouchitis, or perirectal infections occurred in 19 (6/62 J-pouch, 13/28 S-pouch). Fourteen patients (5/22 with diversion, 9/68 without diversion) developed small bowel obstruction. Overall, daytime and nighttime continence was excellent with rare nocturnal evacuations. CONCLUSIONS: Restorative proctocolectomy without protective ileostomy is not associated with an increased morbidity, even in patients with active colitis, and may be appropriate most patients.

Antegrade colonic enemas and intestinal diversion are highly effective in the management of children with intractable constipation.

PURPOSE: Intractable constipation in children is an uncommon but debilitating condition. When medical therapy fails, surgery is warranted; but the optimal surgical approach has not been clearly defined. We reviewed our experience with operative management of intractable constipation to identify predictors of success and to compare outcomes after 3 surgical approaches: antegrade continence enema (ACE), enteral diversion, and primary resection. METHODS: A retrospective review of pediatric patients undergoing ACE, diversion, or resection for intractable, idiopathic constipation from 1994 to 2007 was performed. Satisfactory outcome was defined as minimal fecal soiling and passage of stool at least every other day (ACE, resection) or functional enterostomy without abdominal distension (diversion). RESULTS: Forty-four patients (range = 1-26 years, mean = 9 years) were included. Sixteen patients underwent ACE, 19 underwent primary diversion (5 ileostomy, 14 colostomy), and 9 had primary colonic resections. Satisfactory outcomes were achieved in 63%, 95%, and 22%, respectively. Of the 19 patients diverted, 14 had intestinal continuity reestablished at a mean of 27 months postdiversion, with all of these having a satisfactory outcome at an average follow-up of 56 months. Five patients underwent closure of the enterostomy without resection, whereas the remainder underwent resection of dysmotile colon based on preoperative colonic manometry studies. Of those undergoing ACE procedures, age younger than 12 years was a predictor of success, whereas preoperative colonic manometry was not predictive of outcome. Second manometry 1 year post-ACE showed improvement in all patients tested. On retrospective review, patient noncompliance contributed to ACE failure. CONCLUSIONS: Antegrade continence enema and enteral diversion are very effective initial procedures in the management of intractable constipation. Greater than 90% of diverted patients have an excellent outcome after the eventual restoration of intestinal continuity. Colon resection should not be offered as initial therapy, as it is associated with nearly 80% failure rate and the frequent need for additional surgery.

Extracorporeal membrane oxygenation for nonneonatal acute respiratory failure: the Massachusetts General Hospital experience from 1990 to 2008.

OBJECTIVE: To determine the efficacy of extracorporeal membrane oxygenation (ECMO) for nonneonatal acute respiratory failure. DESIGN: Single-institution, retrospective medical record review from February 1990 to March 2008. SETTING: Tertiary care hospital. PATIENTS: Eighty-one nonneonatal patients (mean age, 23 years; age range, 2 months to 61 years) with acute respiratory failure who had failed maximal ventilator support received ECMO therapy between 1990 and 2008. Patients were grouped into 6 categories based on diagnosis: sepsis (n = 8), bacterial or fungal pneumonia (n = 15), viral pneumonia (n = 9), trauma or burn (n = 10), immunocompromise (n = 15), and other (n = 24). Main Outcome Measure Survival to hospital discharge. RESULTS: Overall survival was 53%. Survival was highest in patients with viral pneumonia (78%), followed by bacterial pneumonia (53%), sepsis syndrome (44%), and immunocompromise (40%). Patients treated following trauma or burns had the lowest survival (33%). The average age was 19 years for survivors as compared with 27 years for nonsurvivors. Survival was lower in patients with multiple organ failure as compared with those with single organ failure (33% vs 60%, respectively), in patients who experienced mechanical ventilation for longer than 10 days prior to the initiation of ECMO as compared with those who received ventilatory support for less than 10 days prior to the initiation of ECMO (31% vs 57%, respectively), and in patients requiring more than 400 hours of ECMO support as compared with those requiring less than 400 hours of ECMO support (42% vs 55%, respectively). CONCLUSIONS: Therapy with ECMO may provide a survival benefit in carefully selected patients with nonneonatal acute respiratory failure who have failed maximal ventilator support. Nonneonatal survival with ECMO therapy is strongly dependent on diagnosis, with the highest survival seen in those with viral or bacterial pneumonia. Older age, multiple organ failure, prolonged ventilation prior to ECMO initiation, and long ECMO runs are associated with decreased survival.

Botulinum toxin for the treatment of chronic constipation in children with internal anal sphincter dysfunction.

Internal anal sphincter (IAS) dysfunction is a cause of refractory constipation in children. The goal of this study was to determine whether intrasphincteric injection of botulinum toxin is effective in the treatment of constipation in pediatric patients with IAS dysfunction. A retrospective review was performed of 24 pediatric patients with intractable constipation. All patients had abnormal anorectal manometry, with either elevated IAS resting pressure (> or =100 mm Hg) or an absent or diminished rectoanal inhibitory reflex. Patients with Hirschsprung's disease were excluded. All patients underwent botox injection into the IAS and were followed for a minimum of 6 months. Of 24 patients, 22 experienced significant improvement in their constipation lasting greater than 2 weeks. The duration of effect was variable, with 12 patients demonstrating benefit lasting at least 6 months. Transient postoperative incontinence occurred in five patients. Intrasphincteric injection of botox is a safe and effective treatment for intractable constipation in children with IAS dysfunction.

Radiographic reduction of intussusception in patients with cystic fibrosis.

Cystic fibrosis (CF) is a genetic disorder affecting approximately one in 2,500 births in the United States. Nearly 1% of patients with CF will develop intussusception, which is commonly ileocecal and felt to be secondary to inspissated feces. These patients generally present before the age of ten. Once the diagnosis of intussusception is confirmed on ultrasound or CT, surgery has been the mainstay of treatment in this patient group. We propose the use of air and contrast enemas as effective and beneficial non-operative approach in these patients. Clinical and imaging findings in four children with known CF who presented with intestinal intussusception, average age 13.25 +/- 5.3 years (range 8-18 years) were reviewed. Patients were diagnosed using ultrasound (n = 3) or abdominal CT (n = 1). All patients suffered from an ileocolic intussusception. Air and Gastrografin(R) enemas were used in an attempt to reduce the intussusception. There were six separate successful intussusception reductions in four patients. Three patients required multiple attempts (2.3 +/- 0.6). Air enemas were used initially in all cases. Gastrografin(R) was used successfully following the failure of air enema in one patient. One patient suffered three separate intussusceptions over a period of 18 months, which were all successfully reduced using air. There were no complications and the patients tolerated the procedure well. Intussusception is an uncommon but serious complication in children with CF. While surgical reduction has been the mainstay of treatment for these patients, we demonstrated that reduction of an intussusception using air or contrast can be accomplished safely, without anesthesia, and should be the initial treatment option.

The use of white blood cell count and left shift in the diagnosis of appendicitis in children.

BACKGROUND: The use of white blood cell (WBC) count and left shift in the diagnosis of appendicitis in pediatric patients is unproven. It is commonly thought that children with appendicitis have an elevated WBC count with a left shift; however, most data supporting this belief stem from studies conducted on appendicitis in adults, not children. The purpose of this investigation was to determine the value of WBC count and differential in the diagnosis of appendicitis in children presenting to the emergency department (ED) with acute abdominal pain. METHODS: Seven hundred twenty-two pediatric ED patients with a primary complaint of nontraumatic abdominal pain were identified by prospective and retrospective methods. White blood cell count with differential was performed on patients with history and physical examination findings that were felt to warrant laboratory investigation. Results of WBC counts were determined as low, normal, or high, with or without a left shift, based on normal age-related values per laboratory protocol for pediatric patients. RESULTS: The diagnosis of appendicitis was made in 10.2% of all patients presenting to the ED with acute abdominal pain. Thirty percent of toddlers (1-3.9 years) with high WBC counts had appendicitis, whereas 0% of toddlers with low WBC counts and 4.8% of toddlers with normal WBC counts had appendicitis (chi = 6.5, P = 0.04). A normal WBC count did not rule out appendicitis in toddlers; however, the negative predictive value (NPV) for normal or low WBC count was high (NPV = 95.6%). In the child age group (4-11.9 years), high WBC count was both sensitive and specific for the diagnosis of appendicitis in children (sensitivity = 71%, specificity = 72%), and the NPV for normal or low WBC count was high (NPV = 89.5%). Lastly, 43.9% of adolescents (12-19 years) with high WBC counts had appendicitis, whereas 0% of adolescents with low WBC counts and 8.3% of adolescents with normal WBC counts had appendicitis (chi = 37.3, P < 0.001). The NPV for a low or normal WBC count was also high in the adolescent group (NPV = 91.9%). Left shift was also strongly associated with appendicitis. Among toddlers, 40% of patients with a left shift had appendicitis, whereas 1.8% of toddlers without a left shift had appendicitis (chi = 25.7, P < 0.001, NPV = 98.2%). Similarly, left shift was strongly associated with appendicitis in children and adolescents. Among children, 54.3% of patients with a left shift had appendicitis, whereas 5.4% of children without a left shift had appendicitis (chi = 67.8, P < 0.001, NPV = 90.5%). Among adolescents, 53.5% of patients with a left shift had appendicitis, whereas 6.1% of adolescents without a left shift had appendicitis (chi = 72.3, P < 0.001, NPV = 93.9%). In patients with a left shift, 51.2% had appendicitis, whereas 3.7% of patients without a left shift had appendicitis (chi = 226.2; P < 0.001, NPV = 96.3%). In all patients with appendicitis, elevated WBC counts had a sensitivity of 67% and a specificity of 80%. Using left shift alone as an indicator for appendicitis was associated with a sensitivity of 59% and a specificity of 90%. However, when a high WBC count and left shift were combined, the sensitivity climbed to 80%, and specificity remained at 79%. The sensitivity fell to 47% when both a high WBC count and left shift were analyzed, and specificity climbed to 94%. The positive likelihood ratio for a high WBC count and left shift was 9.8. CONCLUSIONS: The determination of WBC count and differential is useful in the diagnosis of appendicitis in children presenting to the ED with nontraumatic acute abdominal pain, regardless of age. High WBC counts and left shift are independently, strongly associated with appendicitis in children aged 1 to 19 years. In fact, for this subset of patients older than 4 years, the most common diagnosis in the setting of an elevated WBC count was appendicitis. The presence of an increased WBC count or left shift carries with it a high sensitivity (79%), and the presence of both high WBC count and left shift has the highest specificity (94%). These values are, therefore, helpful in the diagnosis and exclusion of appendicitis. Although not absolute, the WBC count and left shift can be helpful in the diagnosis and exclusion of appendicitis.

Malrotation with midgut volvulus: CT findings of bowel infarction.

Midgut volvulus, the most common serious complication of malrotation, can be diagnosed using conventional contrast fluoroscopy, US or CT. CT is a quick and comprehensive examination in the evaluation of complex acute abdominal pathology in children. Contrast-enhanced CT can readily help the radiologist recognize perfusion abnormalities of the bowel, which is vital for reducing morbidity and mortality in affected children. Our case emphasizes and demonstrates additional CT features of bowel infarction in a child with a proven malrotation with midgut volvulus.

The diagnosis of acute appendicitis in a pediatric population: to CT or not to CT.

PURPOSE: The aim of this study was to determine if focused appendiceal computed tomography with colon contrast (FACT-CC) increases the accuracy of the preoperative diagnosis of acute appendicitis in children. METHODS: A 5-year retrospective review was conducted of a university hospital database of 283 patients (age 0.8 to 19.3 years; mean, 11.3 years) treated with appendectomy for presumed acute appendicitis. RESULTS: Of the 283 patients in whom appendectomies were performed, 268 were confirmed by pathologic analysis of the specimen to have acute appendicitis for a diagnostic accuracy in our institution of 94.7%. Ninety-six patients (34%) underwent FACT-CC scans as part of their preoperative evaluation. The sensitivity of the computed tomography (CT) scan was 94.6%, and the positive predictive value was 95.6%. In girls older than 10 years, CT imaging was not significantly more accurate in predicting appendicitis than examination alone (93.9% v. 87.5%; P =.46). CONCLUSIONS: Preoperative FACT-CC did not increase the accuracy in diagnosing appendicitis when compared with patients diagnosed by history, physical examination and laboratory studies. If there was a strong suspicion of appendicitis, a negative CT scan did not exclude the diagnosis of appendicitis. However, focused appendiceal CT scan is a sensitive test with a high positive predictive value and may be useful in a patient with an atypical history or examination.