Reconstructing the anal sphincters to reverse iatrogenic overstretching following a pull-through for Hirschsprung disease. One-year outcomes.

BACKGROUND: In patients with Hirschsprung disease (HSCR), soiling may be related to anal sphincter damage following the initial pull-through. No optimal treatment has been developed for such patients, although enemas (rectal or antegrade) have been applied with some success. We present the one-year outcomes of a new technique for anal sphincter reconstruction. METHODS: All patients with HSCR referred from other institutions for post pull-through soiling were studied. Seven patients with patulous sphincters underwent sphincter reconstruction. Six had a full preoperative evaluation and were included in the study. Their 12-month outcomes were assessed. RESULTS: All six patients had soiling without voluntary bowel movements (VBMs). One patient was clean on Malone flushes when referred. Three underwent pre- and post-reconstruction non-sedated three-dimensional anorectal manometry, and objectively were able to close their sphincters following the reconstruction. All patients without Down syndrome (4 of 6) showed improvement in the abbreviated Baylor Continence Scale (4.5 vs. 0.75). One patient has achieved total bowel control without antegrade flushes, three now have VBMs which they did not have before but have occasional accidents and use antegrade flushes intermittently. They reported higher productivity, the ability to participate in sports and be away from home with confidence in their regimen. Two of 6 patients have Down syndrome and required a redo pull-through for other indications and underwent empiric sphincter reconstruction. For these two patients we do not have an outcomes assessment. CONCLUSIONS: A new technique for sphincter reconstruction shows promising results in improvement of bowel control at one year. LEVEL OF EVIDENCE: IV.

Functional constipation refractory to medical management: The colon is the problem.

BACKGROUND: Management of refractory constipation in children has not been standardized. We propose a protocolized approach which includes a contrast enema, anorectal manometry and exclusion of Hirschsprung disease (HD). For those without HD or with normal sphincters, an assessment of the colonic motility may be needed. The subgroups of dysmotility include (1) slow motility with contractions throughout, (2) segmental dysmotility (usually the sigmoid), or (3) a diffusely inert colon. We offered a Malone appendicostomy in all groups with the hope that this would avoid colonic resection in most cases. METHODS: Patients with medically refractory constipation were reviewed at a single institution (2020 to 2021). For patients without HD or an anal sphincter problem, assessment of colonic motility using colonic manometry was performed followed by a Malone appendicostomy for antegrade flushes. RESULTS: Of 196 patients evaluated for constipation refractory to medical management, 22 were felt to have a colonic motility cause. These patients underwent colonic manometry and Malone appendicostomy. 13 patients (59%) had a slow colon but with HAPCs throughout, 5 (23%) had segmental dysmotility, and 4 (18%) had a diffuse colonic dysmotility. 19 (86%) responded well to antegrade flushes with 17 reporting no soiling and 2 having occasional accidents. 3 patients (14%) failed flushes and underwent a colon resection within 6-month following Malone procedure. CONCLUSION: We propose a protocol for medically refractory constipation which provides a collaborative framework to standardize evaluation and management of these patients with antegrade flushes, which aids in avoidance of colonic resection in most cases. LEVEL OF EVIDENCE: Level IV.

Per Oral Endoscopic Myotomy (POEM) for pediatric achalasia: Institutional experience and outcomes.

BACKGROUND: The surgical treatment of achalasia by both laparoscopic and endoscopic approaches has been recognized as the definitive management in children. Despite reported low volumes in many centers, there has been an increasing worldwide experience with endoscopic approaches to pediatric achalasia. The aim of this study is to report our institutional experience with per oral endoscopic myotomy (POEM) as first-line or revisional therapy for achalasia. METHODS: An IRB approved retrospective review of all patients who underwent operative procedures for achalasia, specifically with the POEM technique, from July 2015 to September 2021. Data including demographics, intra-operative details, pre and post operative Eckardt scores, complications, outcomes, and follow-up were obtained. RESULTS: During the study period, a total of 43 children underwent 46 operations for achalasia including POEM and laparoscopic Heller myotomy (LHM). Operations included 37 POEMS (33 primary POEMS; 3 POEMS after failed LHM; and 1 POEM after failed POEM). Additionally, 9 LHM operations including, 4 primary LHM; 3 attempted POEMS converted to LHM; 1 attempted POEM after failed LHM converted to redo LHM; and 1 LHM after failed POEM. In the POEM group (n = 37), based on the high resolution esophageal manometry findings Chicago Classification types at diagnosis were as follows: 9 patients were type I (24.3%); 25 patients were type II (67.6%); 2 patients were type III (5.9%) and 1 patient was unknown type (2.7%). Sixteen children (43.2%) had prior endoscopic treatment of achalasia prior to POEM [Pneumatic Balloon Dilatation (PBD), and/or Botox injection (BTI)],), while prior operative intervention occurred in 4 patients (10.8%), 3 LHM and 1 POEM. Age at operation was 2-18 years (mean ± SD age: 11.6 ± 4.5 years). Weight at operation 11.8-100.7 kg (mean ± SD kg; 39 ± 19.9 kg). Range of baseline Eckardt score was 4-10 (mean ± SD: 6.73 ± 1.5). Operative time was 64-359 min (mean ± SD minutes: 138.1 ± 62.2 min). Intraoperative complications occurred in 16 patients (43.2%) but did not require reoperation during index admission including: 4 mucosotomy (11.8%); 9 pneumothoraces (24.3%); 2 pneumomediastinum (5.4%); 10 pneumoperitoneum (27%); 0 sub-mucosal tunnel bleeding (0%); 0 open conversion/death (0%). Post operative complications included: 5 recurrent dysphagia (13.5%); 0 esophageal leak (0%); 3 GERD (8.1%); 1 failed POEM (2.7%). Median length of stay was 2 days (mean ± SD days: 2.4 ± 0.9 day). Follow-up ranged from 1 to 74 months (median 15 months), mean follow-up 22.6 months ± 20 months. Post POEM Eckardt score was 0.6 ± 0.9. Five patients required a single PBD post POEM (13.5%) and 1 patient required a repeat myotomy (LHM) after POEM (2.7%) for a 16.2% reintervention rate. Subsequent normalization of Eckardt scores (≤ 3) and symptomatic relief was achieved in all patients (100%). CONCLUSIONS: POEM as first-line therapy for pediatric achalasia, or as a secondary procedure after failed prior myotomy or POEM, in our experience is safe and effective. We have shown equivalent results to our own prior experience with LHM. Long-term follow-up will be performed to monitor for recurrent symptoms, adequate physical growth, and general development. LEVEL OF EVIDENCE: II.

A technique to reconstruct the anal sphincters following iatrogenic stretching related to a pull-through for Hirschsprung disease.

Soiling and fecal incontinence are troubling complications which can occur after a pull-through for Hirschsprung disease. They can usually be improved with proper medical management, but in some cases are the result of an anatomic defect related to overstretching of the sphincters and/or damage of the anal canal. For such patients the treatment of this true fecal incontinence is limited to a structured bowel management program with ante- or retrograde enemas to achieve social continence. Herein we report two such patients with overstretched sphincters and loss of the dentate line after an initial pull-through and describe a sphincter tightening technique to improve bowel control.

Median Arcuate Ligament Syndrome with Orthostatic Intolerance: Intermediate-Term Outcomes following Surgical Intervention.

OBJECTIVES: To report the intermediate-term outcome following surgical intervention for median arcuate ligament syndrome (MALS) in adolescents and young adults with orthostatic intolerance (OI) to assess clinical improvement in the gastrointestinal and 5 other functional domains and if relief of arterial obstruction is associated with resolution of clinical symptoms. STUDY DESIGN: Thirty-one patients were given 2 dysautonomia-designed questionnaires to assess changes in symptoms following operative intervention in 6 functional domains and underwent postoperative repeat abdominal ultrasound examinations. RESULTS: Average follow-up after surgery was 22.4 ± 14.8 months. Self-assessed quality of health on a Likert scale (1-10 with 10 being normal) improved from 4.5 ± 2.1 preoperatively to 5.3 ± 2.4 postoperatively (P = not significant). Gastrointestinal symptoms of abdominal pain, nausea, and vomiting improved in 63% (P = .007), 53% (P = .040), and 62% (P = .014) of patients, respectively. Cardiovascular symptoms of dizziness, syncope, chest pain, and palpitations improved in 45% (P = not significant), 50% (P = not significant), 54% (P = .043), and 54% (P = .037) of patients, respectively. Transabdominal ultrasound peak supine expiratory velocity decreased from 348 ± 105 cm/s preoperatively to 251 ± 109 cm/s at 6 months or more after a ligament release procedure. Decrease of the postoperative celiac artery Doppler velocity was not associated with an improvement in gastrointestinal symptoms (P = .075). CONCLUSIONS: Adolescent and young adult patients with median arcuate ligament syndrome and OI have a good response to surgical intervention. About two-thirds of patients report significant improvement in symptoms of abdominal pain, nausea, and vomiting. Despite these encouraging data, many patients with MALS and OI continue to have an impaired quality of health.

Utility of Diagnostic Studies for Upper Gastrointestinal Symptoms in Children with Orthostatic Intolerance.

OBJECTIVE: To assess the utility of gastrointestinal (GI) diagnostic studies in the evaluation of patients with orthostatic intolerance. STUDY DESIGN: Medical records of 103 consecutive children/young adults with orthostatic intolerance and gastrointestinal symptoms were reviewed. All patients had undergone antroduodenal manometry in conjunction with the tilt table test, autonomic testing, and upper gastrointestinal endoscopy (EGD). A gastric emptying study (GES) was performed in 81 patients. RESULTS: The median age of the cohort was 17 years (IQR, 15-19) with a female predominance (females:males, 3:1). As expected, the tilt table test was abnormal in all patients. Antroduodenal manometry was abnormal in 83 of 103 patients (81%), showing neurogenic intestinal dysmotility in 50%, rumination in 20%, and visceral hyperalgesia in 10%. The GES results were abnormal in 23 of 81 patients (28.4%), mostly (21 of 23) with delayed GES. None of the tilt table test or autonomic results were predictive of abnormal antroduodenal manometry or GES. Analysis of EGD biopsy samples revealed nonspecific esophagitis and/or gastritis in 16 of 103 patients (15%). CONCLUSIONS: Antroduodenal manometry with the tilt table test were the most insightful investigations in adolescents and young adults with orthostatic intolerance and gastrointestinal symptoms. GES and EGD provided limited information. Gastrointestinal symptoms were related more to functional rather than mucosal or organic etiologies, suggesting a limited role of endoscopy alone in evaluating patients with orthostatic intolerance presenting with gastrointestinal symptoms.

Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series.

The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non-SCD cause of hepatopathy in a patient with viral hepatitis. These complications require a systematic approach to extensive evaluation and coordinated multidisciplinary care.

Surgical management of esophageal achalasia: Evolution of an institutional approach to minimally invasive repair.

BACKGROUND: Surgical management of esophageal achalasia (EA) in children has transitioned over the past 2 decades to predominantly involve laparoscopic Heller myotomy (LHM) or minimally invasive surgery (MIS). More recently, peroral endoscopic myotomy (POEM) has been utilized to treat achalasia in children. Since the overall experience with surgical management of EA is contingent upon disease incidence and surgeon experience, the aim of this study is to report a single institutional contemporary experience for outcomes of surgical treatment of EA by LHM and POEM, with regards to other comparable series in children. METHODS: An IRB approved retrospective review of all patients with EA who underwent treatment by a surgical approach at a tertiary US children's hospital from 2006 to 2015. Data including demographics, operative approach, Eckardt scores pre- and postoperatively, complications, outcomes, and follow-up were analyzed. RESULTS: A total of 33 patients underwent 35 operative procedures to treat achalasia. Of these operations; 25 patients underwent laparoscopic Heller myotomy (LHM) with Dor fundoplication; 4 patients underwent LHM alone; 2 patients underwent LHM with Thal fundoplication; 2 patients underwent primary POEM; 2 patients who had had LHM with Dor fundoplication underwent redo LHM with takedown of Dor fundoplication. Intraoperative complications included 2 mucosal perforations (6%), 1 aspiration, 1 pneumothorax (1 POEM patient). Follow ranged from 8months to 7years (8-84months). There were no deaths and no conversions to open operations. Five patients required intervention after surgical treatment of achalasia for recurrent dysphagia including 3 who underwent between 1 and 3 pneumatic dilations; and 2 who had redo LHM with takedown of Dor fundoplication with all patients achieving complete resolution of symptoms. CONCLUSIONS: Esophageal achalasia in children occurs at a much lower incidence than in adults as documented by published series describing the surgical treatment in children. We believe the MIS surgical approach remains the standard of care for this condition in children and describe the surgical outcomes and complications for LHM, as well as, the introduction of the POEM technique in our center for treating achalasia. Our institutional experience described herein represents the largest in the "MIS era" with excellent results. We will refer to alterations in our practice that have included the use of flexible endoscopy in 100% of LHM cases and use of the endoscopic functional lumen imaging probe (EndoFLIP) in both LHM and POEM cases which we believe enables adequate Heller myotomy.

Diagnostic yield, safety, and efficacy of push enteroscopy in pediatrics.

BACKGROUND: Push enteroscopy has not been compared to standard endoscopy in children. OBJECTIVE: The aim of this study was to determine the feasibility of push enteroscopy in children with suspected proximal small bowel disease, and to compare its diagnostic yield and safety with standard endoscopy. DESIGN/SETTING: Database review. PATIENTS: A database analysis was performed on all children who underwent push enteroscopy at The Johns Hopkins Children's Center from 2001 to 2005. Patient demographics, clinical history, and indication for push enteroscopy were all recorded. Clinical utility was qualified based on the influence of PE on therapy. MAIN OUTCOME MEASUREMENTS: Diagnostic yield and safety of push enteroscopy in children. RESULTS: Push enteroscopy was performed on 44 children (27 M; 17 F) with a median age (range) of 10 (2-18) years. The most common indications for push enteroscopy were suspected proximal small bowel disease based on radiological criteria (21), and bleeding (9). Push enteroscopy confirmed the diagnosis of proximal small bowel Crohn's disease (CD) in 23, polyps in 5, eosinophilic gastroenteritis in 4, celiac disease in 1, microvillous inclusion disease in 1, and lymphoproliferative disease in 1 patient. An isolated non-Crohn's related gastric (1) and jejunal ulcer (1) was also identified. Just 9 of these identifiable lesions were within reach by esophagogastroduodenoscopy (EGD). Seven patients had a normal push enteroscopy. The clinical management was modified in 34 patients. Push enteroscopy was not shown to significantly alter the time of procedure when compared to EGD. CONCLUSIONS: Push enteroscopy is a safe diagnostic tool with proven clinical utility in children with suspected proximal small bowel disease. Larger studies are needed to establish the widespread application of push enteroscopy in pediatrics.

Crohn's jejunoileitis: the pediatrician's perspective on diagnosis and management.

Although uncommon, diffuse jejunoilietis is one of the most difficult areas in Crohn's disease (CD) to treat. Although the exact frequency is unknown, most gastroenterologists believe that its prevalence has been underestimated and that it may have an increased incidence among children and young adolescents. The clinical importance of this clinical disease phenotype is the impact diffuse small bowel disease is expected to have on a child's growth and development. Moreover, patients with jejunoileitis are more likely to experience complications, including fistulization, and most commonly, intestinal obstruction. The associated morbidity and frequent need for surgical intervention renders these patients at risk for intestinal insufficiency. Although stricturoplasty has reduced the incidence of short bowel syndrome, most patients with proximal small bowel CD still require repeated surgical intervention. Jejunoileitis represents a distinct clinical phenotype within the heterogenous family of disease phenotypes considered as CD. Whether a specific genotype will be found to associate with jejunoileitis remains to be determined. Through the development of novel diagnostic techniques, including gadolinium enhanced magnetic resonance imaging (GMRI), enteroscopy, and capsule endoscopy, the mean age at diagnosis is expected to decrease. Coupled with an increase in clinical suspicion, early diagnosis may allow physicians to consider implementing aggressive immunomodulatory therapy. Future studies are needed to determine if the early detection and use of immune modulators in patients with proximal small bowel disease will improve overall quality of life and decrease the risk of nutritional and surgical comorbidity.

The limitations of gastro-jejunal (G-J) feeding tubes in children: a 9-year pediatric hospital database analysis.

BACKGROUND: A gastro-jejunal (G-J) feeding tube is a safe and useful temporizing method of providing enteral access in children. Although G-J tubes are often used to obviate the need for a surgical jejunostomy, their long-term use is often associated with mechanical failure. AIM: To review the clinically effective durability of G-J feeding tubes in providing enteral access in children. METHODS: We performed a retrospective review of 102 patients at the Johns Hopkins Children's Center from 1994-2003 whose underlying diagnosis necessitated the need for postpyloric enteral access. RESULTS: Long-term follow-up was obtained in 85 (48 M; 37 F) patients with a median (range) age of 2.0 (0.1-18.0) yr. The most common indication for G-J tube placement was gastroesophageal reflux with aspiration in 51 patients and feeding intolerance and vomiting in 19 patients. The mean (range) number of tube replacements was 2.2 (1-14) over a median (range) duration of follow-up of 39 (2-474) days. The indication for G-J tube replacement included: tube displacement (58), a clogged tube (41), and a cracked tube or ruptured balloon (35). In 52 cases, the cause for G-J tube replacement was undetermined. CONCLUSIONS: G-J feeding tubes are associated with the frequent need for tube maintenance and replacement and may not be the most feasible clinical option in providing long-term (>1 month) enteral access in children intolerant to gastrostomy tube feeds. Future studies are needed to develop innovative percutaneous jejunostomy tube placement techniques that facilitate long-term enteral access.

Gadolinium-enhanced magnetic resonance imaging: a useful radiological tool in diagnosing pediatric IBD.

BACKGROUND: Recent advances in gadolinium-enhanced magnetic resonance imaging (G-MRI) have been developed to enhance the resolution of the intestinal mucosa and facilitate the differentiation of ulcerative colitis (UC) from Crohn's disease (CD). The objective of this study is to apply this technology in Pediatrics. METHODS: A G-MRI was performed on 58 consecutive children with suspected IBD between 1999 and 2002 using intravenous gadolinium, fat suppression, and respiration-suspended sequences to enhance the resolution of the intestinal wall. The sensitivity and specificity in diagnosing either UC or CD was determined by comparing the G-MRI to the established histologic diagnosis. RESULTS: G-MRI confirmed the diagnosis of either CD (21) or UC (7) with a sensitivity and specificity of 96% and 92%, respectively. Among the 21 patients with CD, 14 showed proximal small bowel involvement by G-MRI. In total, 17 patients were diagnosed with indeterminate colitis (IC) based on histologic criteria alone, and among these patients, G-MRI had a significantly lower non-classification rate (P < 0.02). In comparison, endoscopy was less sensitive (57%), but more specific (100%) than either histology or G-MRI in diagnosing IBD. G-MRI also showed a strong concordance with computed tomography in diagnosing CD (P = 0.001). CONCLUSION: G-MRI is a both a sensitive and specific radiologic tool in diagnosing pediatric IBD. In patients with CD, G-MRI may be useful in identifying proximal small bowel involvement. Longitudinal follow-up studies are needed in those patients diagnosed with IC to determine the predictive value of G-MRI testing.

Epidemiology of primary hepatic malignancies in U.S. children.

The epidemiology of primary hepatic malignancies in U.S. children is poorly characterized. We analyzed the incidence, mortality, and characteristics of primary hepatic malignancies in U.S. residents less than 20 years of age. Fatal primary hepatic malignancies in persons less than 20 years of age, between 1979 and 1996, were identified using the multiple-cause-of-death database (National Center for Health Statistics). Histologically confirmed primary hepatic malignancies occurring between 1973 and 1997 were identified using the Surveillance, Epidemiology, and End Results (SEER) database. Between 1979 and 1996, 918 primary hepatic malignancy deaths (average, 0.7/1,000,000/year) were reported nationally among persons less than 20 years of age; rates were higher among Asians and among foreign-born children. Between 1973 and 1997, 271 primary hepatic malignancy cases were reported to SEER among persons less than 20 years of age, of which 184 (67%) and 83 (31%) were hepatoblastoma and hepatocellular carcinoma, respectively. Among children less than 5 years of age, hepatoblastoma accounted for 91% of primary hepatic malignancy cases, whereas among those 15 to 19 years of age, hepatocellular carcinoma accounted for 87% of cases. Five-year survival for hepatoblastoma was 52%, compared with 18% for hepatocellular carcinoma. In the SEER sites, between 1973 and 1977 and 1993 and 1997, hepatoblastoma rates increased (0.6 to 1.2/1,000,000, respectively), while hepatocellular carcinoma rates decreased (0.45 to 0.29/1,000,000, respectively). In conclusion, histologically confirmed hepatocellular carcinoma was reported in children less than 5 years of age, also, where hepatoblastoma is the predominant primary hepatic malignancy. Hepatocellular carcinoma has worse survival rates than hepatoblastoma, and its incidence has not increased. Better maintenance of databases may provide information about associated factors behind this unexpected occurrence.