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1.
J Surg Res ; 2024 Aug 31.
Artigo em Inglês | MEDLINE | ID: mdl-39218774

RESUMO

INTRODUCTION: Dr. Asa G. Yancey published a surgical technique describing a pull - through of normal colon through a cuff of aganglionic colon to treat Hirschsprung disease in 1952, 12 y before Dr. Franco Soave whose name is attached to the procedure. Yancey and his pioneering operation went unrecognized for over half a century because of discriminatory segregation in the publishing practices of academic medicine dating back to the 1950s. MATERIALS AND METHODS: We performed a literature review on the surgical therapies for Hirschsprung disease. This history was supplemented with first-hand accounts provided by Yancey's children. Further information by leaders of the American Pediatric Surgical Association Hirschsprung interest group regarding the future of surgical nomenclature for the endorectal pull-through procedure was acquired through interviews. RESULTS: A review of the literature revealed that Yancey's description of the pull-through technique for Hirschsprung disease was published 12 y prior to Soave's publication and yet, Yancey received little to no recognition for his work. Yancey's children describe a surgeon who was persistent in his endeavor to create a more inclusive field of academic surgery as well as a man who prioritized his family and the education of future surgeons. Conversations with the American Pediatric Surgical Association Hirschsprung interest group suggest active changes to give posthumous credit to Yancey, including renaming the procedure to the Yancey - Soave pull-through technique. CONCLUSIONS: Here, we provide a historical review of Yancey's career as well as insights on the man behind the surgery and how he persevered in academic surgery despite experiencing discrimination during the civil rights movement.

2.
J Pediatr Surg ; : 161697, 2024 Aug 18.
Artigo em Inglês | MEDLINE | ID: mdl-39256064

RESUMO

BACKGROUND: Anorectal malformations (ARMs) commonly result in constipation and fecal incontinence following primary surgical reconstruction. This study investigates global variations in postoperative care and resources. METHODS: A survey was distributed via the International Pediatric Endosurgery Group (IPEG) and snowball sampling. Geographically, respondents were categorized into high-income countries (HICs) and low-or middle-income countries (LMICs). RESULTS: 233 surveys were received, 64% from LMICs and 36% from HICs. Of these, 51% reported monitoring ARM patients for over a year, while 23% utilized digital technologies. Access to anesthesia for anorectal exams was available to 70% of respondents. Only 29% had established a one-week Bowel Management Program (BMP). Collaboration with urologists and gynecologists was more prevalent in HICs (59%) compared to LMICs (37%, p < 0.01). In HICs, nurses and advanced practice providers were significantly more involved in BMP (71% vs. 39% in LMICs; p < 0.01), and abdominal radiographs for regimen adjustments were used more frequently (80% vs. 69% in LMICs; p = 0.03). Treatment regimens were more varied in HICs, and quality of life tracking was more consistent (19% vs. 9% in LMICs; p = 0.02). LMICs reported significant shortages of medications and equipment (75% vs. 58% in HICs; p = 0.01), inadequate sanitation (48% vs. 24%; p < 0.01), and insufficient insurance coverage (58% vs. 44%; p = 0.04). CONCLUSIONS: There are notable global disparities in the postoperative care of ARM patients, particularly in BMP protocols and treatment regimens, with LMICs facing severe socioeconomic challenges. This emphasizes the urgent need for targeted strategies and resources to enhance outcomes for ARM patients across different regions. LEVEL OF EVIDENCE: Level III.

3.
J Pediatr Surg ; : 161677, 2024 Aug 10.
Artigo em Inglês | MEDLINE | ID: mdl-39217005

RESUMO

BACKGROUND: Children with total colonic Hirschsprung disease (TCHD) are a unique group of patients with pre- and postoperative management challenges. This review provides a rational, expert-based approach to diagnosing and managing TCHD. METHODS: The guidelines were developed by the Hirschsprung Disease Interest Group members established by the American Pediatric Surgical Association (APSA) Board of Governors. Group discussions, literature review, and expert consensus were used to summarize the current knowledge regarding diagnosis, staged approach, the timing of pull-through, and pre-and postoperative management in children with TCHD. RESULTS: This paper presents recommendations for managing TCHD before and after reconstruction, including diagnostic criteria, surgical approaches, bowel management, diet, antibiotic prophylaxis, colonic irrigations, and post-surgical considerations. CONCLUSIONS: A clear understanding of the unique challenges posed by TCHD and consensus on its treatment are lacking in the literature. This review standardizes this patient group's pre- and postoperative management. LEVEL OF EVIDENCE: V.

4.
European J Pediatr Surg Rep ; 12(1): e45-e49, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-39015759

RESUMO

Concomitant presentation of jejunal atresia and Hirschsprung's disease is rare and places children at high risk for developing short bowel syndrome and parenteral nutrition dependence, which can affect the feasibility/timing of pull-through. A patient was born with jejunal atresia with a delayed diagnosis of Hirschsprung's disease. After several procedures and bowel resections, the patient was ultimately left with an end jejunostomy and long Hartman's pouch with short bowel syndrome, dependent on parenteral nutrition. The patient initially presented to our institution at age 2 with failure to thrive secondary to an obstructed/dilated jejunostomy and mild enterocolitis of their defunctionalized segment. The patient subsequently underwent completion of subtotal colectomy and revision of jejunostomy utilizing a serial transverse enteroplasty to manage the dilated bowel and gain length. The patient was able to wean off parenteral nutrition and achieve nutritional autonomy by age 5. Following this, the patient was able to undergo an ileoanal pull-through. After the pull-through, the patient was able to pass stool independently and suffered no major complications to date. Serial transverse enteroplasty can be successfully utilized in patients with a history of Hirschsprung's disease and jejunal atresia to achieve nutritional autonomy and ultimately reestablish gastrointestinal continuity with pull-through.

5.
J Pediatr Surg ; 59(10): 161600, 2024 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-38981833

RESUMO

BACKGROUND: Children with Hirschsprung disease (HSCR) proximal to the splenic flexure or those needing a redo pull-through (PT) are at risk for tension and ischemia of the PT which could result in leak, stricture, or loss of ganglionated bowel. Colonic derotation is a technique used to minimize tension and avoid duodenal obstruction. The aim of this study was to describe this technique and outcomes in a series of patients requiring this intervention. METHODS: All patients underwent initial diversion and colonic mapping. The derotation procedure involves mobilization of the remaining colon, counterclockwise rotation via the stoma closure site, placement of the pull through (the right colon) lying on the right of the pelvis, and ligation of the middle colic artery with preservation of the marginal branch running from the ileocolic artery. This maneuver prevents compression of the duodenum by the mesenteric vessels and allows for an isoperistaltic, tension-free anastomosis. Intraoperative indocyanine green fluorescence angiography (ICG-FA) was utilized in many of the cases to map the blood supply of the pull-through colon. We reviewed outcomes for all children with HSCR who underwent colonic derotation from 2014 to 2023. Descriptive statistics were performed. RESULTS: There were 37 children included. Most were male (67.5%) with the original transition zone proximal to the rectosigmoid (81.1%). The median age at PT was 9.3 months [6.1-39.7]. Median operative time was 6.6 h [4.9-7.4] and 19 cases (51.4%) used ICG-FA. Most children had no 30-day postoperative complications (67.6%); in those who did develop complications, readmissions for electrolyte imbalance was most common (50.0%). There were zero cases of anastomotic leak at PT anastomosis. At long-term follow up, median 4.4 years [2.3-7.0], three children (8.1%) developed an anastomotic stricture, all were amenable to anal dilation, and five experienced episodes of enterocolitis (14.7%). Most children had between 1 and 4 stools per day (58.8%). CONCLUSION: Colonic derotation is a useful strategy to ensure well-perfused colonic length, protect the marginal artery blood supply, avoid duodenal compression, and ensure a tension-free anastomosis with minimal complications. TYPE OF STUDY: Original research, retrospective cohort. LEVEL OF EVIDENCE: III.


Assuntos
Doença de Hirschsprung , Laparoscopia , Humanos , Doença de Hirschsprung/cirurgia , Masculino , Feminino , Lactente , Laparoscopia/métodos , Pré-Escolar , Estudos Retrospectivos , Complicações Pós-Operatórias/prevenção & controle , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/epidemiologia , Colo/cirurgia , Colo/irrigação sanguínea , Resultado do Tratamento , Anastomose Cirúrgica/métodos
6.
European J Pediatr Surg Rep ; 12(1): e23-e25, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38298568

RESUMO

Optimal surgical management of patients with intractable constipation and diffuse colonic motility is not well defined. We present a patient with such a history, who ultimately achieved successful surgical management of constipation through a stepwise approach. An 8-year-old female presents with longstanding constipation and diffuse colonic dysmotility demonstrated with colonic manometry. She initially underwent sigmoid resection and cecostomy which failed and required diverting ileostomy. We initially proceeded with an extended resection, colonic derotation (Deloyers procedure), and neo-appendicostomy (neo-Malone) which resulted in successful spontaneous stooling for 1 year. Her constipation recurred and she subsequently underwent completion colectomy with ileorectal anastomosis given that she previously demonstrated ability to stool independently. Six months from surgery the patient continues to stool daily with assistance of fiber and loperamide. This case highlights a stepwise surgical approach to managing constipation due to diffuse colonic dysmotility and demonstrates that diffuse dysmotility may benefit from an upfront subtotal resection; however, it is crucial to assess a patient's ability to empty their rectum prior.

7.
Urology ; 183: 192-198, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37805049

RESUMO

OBJECTIVE: To present a unique series of children with previously repaired anorectal malformations (ARM) with subsequent urethral pathology repaired via a posterior sagittal exposure and highlight the associated technical advantages. METHODS: Using a retrospective review of all procedures performed in our pediatric colorectal and pelvic reconstruction program from January 2020 through December 2022, we compiled a case series of patients with a history of ARM and prior posterior sagittal anorectoplasty (PSARP) who had urethral pathology and concurrent indication for redo-PSARP. Clinical features, operative details, and postoperative outcomes were collected. RESULTS: Six male patients presented at a median age of 4.3 years, all born with an ARM of recto-urinary fistula type, of which 3 were recto-prostatic, 1 recto-bladder-neck, and 2 unknown type. In addition to redo-PSARP, 2 underwent remnant of the original fistula excision and 4 had urethral stricture repair. One required post-operative Heineke-Mikulicz anoplasty. Patients underwent cystoscopy 4-6 weeks post-reconstruction, and none showed urethral stricture requiring treatment. Post-procedurally, 5 patients were able to void urethrally and 1 required additional bladder augmentation/Mitrofanoff. CONCLUSION: Redo-PSARP completely mobilizes the rectum, thereby providing excellent exposure to the posterior urethra for repair. This approach also allows the option of a rectal flap for augmented urethroplasty as well as harvest of an ischiorectal fat pad for interposition.


Assuntos
Malformações Anorretais , Fístula Retal , Estreitamento Uretral , Humanos , Masculino , Criança , Pré-Escolar , Malformações Anorretais/complicações , Malformações Anorretais/cirurgia , Uretra/cirurgia , Estreitamento Uretral/patologia , Canal Anal/anormalidades , Resultado do Tratamento , Reto/cirurgia , Reto/anormalidades , Estudos Retrospectivos , Fístula Retal/cirurgia
8.
Eur J Pediatr Surg ; 2024 Mar 11.
Artigo em Inglês | MEDLINE | ID: mdl-37940124

RESUMO

PURPOSE: The purpose of the study was to determine if antegrade continence enema (ACE) alone is an effective treatment for patients with severe functional constipation and segmental colonic dysmotility. METHODS: A retrospective study of patients with functional constipation and segmental colonic dysmotility who underwent ACE as their initial means of management. Data was collected from six participating sites in the Pediatric Colorectal and Pelvic Learning Consortium. Patients who had a colonic resection at the same time as an ACE or previously were excluded from analysis. Only patients who were 21 years old or younger and had at least 1-year follow-up after ACE were included. All patients had segmental colonic dysmotility documented by colonic manometry. Patient characteristics including preoperative colonic and anorectal manometry were summarized, and associations with colonic resection following ACE were evaluated using Fisher's exact test and Wilcoxon rank-sum test. p-Values of less than 0.05 were considered significant. Statistical analyses and summaries were performed using SAS version 9.4 (SAS Institute Inc., Cary, North Carolina, United States). RESULTS: A total of 104 patients from 6 institutions were included in the study with an even gender distribution (males n = 50, 48.1%) and a median age of 9.6 years (interquartile range 7.4, 12.8). At 1-year follow-up, 96 patients (92%) were successfully managed with ACE alone and 8 patients (7%) underwent subsequent colonic resection for persistent symptoms. Behavioral disorder, type of bowel management, and the need for botulinum toxin administered to the anal sphincters was not associated with the need for subsequent colonic resection. On anorectal manometry, lack of pelvic floor dyssynergia was significantly associated with the need for subsequent colonic resection; 3/8, 37.5% without pelvic dyssynergia versus 1/8, 12.5% (p = 0.023) with pelvic dyssynergia underwent subsequent colonic resection. CONCLUSION: In patients with severe functional constipation and documented segmental colonic dysmotility, ACE alone is an effective treatment modality at 1-year follow-up. Patients without pelvic floor dyssynergia on anorectal manometry are more likely to receive colonic resection after ACE. The vast majority of such patients can avoid a colonic resection.

10.
Children (Basel) ; 10(6)2023 Jun 19.
Artigo em Inglês | MEDLINE | ID: mdl-37371309

RESUMO

BACKGROUND: Functional constipation (FC) affects up to 32% of the pediatric population, and some of these patients are referred to pediatric surgery units to manage their constipation and/or fecal incontinence. The aim of the current paper is to report the recent updates on the evaluation and management of children with FC as a part of a manuscript series on bowel management in patients with anorectal malformations, Hirschsprung disease, spinal anomalies, and FC. METHODS: A literature search was performed using Medline/PubMed, Google Scholar, Cochrane, and EMBASE databases and focusing on the manuscripts published within the last 5-10 years. RESULTS: The first step of management of children with FC is to exclude Hirschsprung disease with a contrast study, examination under anesthesia, anorectal manometry (AMAN). If AMAN shows absent rectoanal inhibitory reflex, a rectal biopsy is performed. Internal sphincter achalasia or high resting pressures indicate botulinum toxin injection. Medical management options include laxatives, rectal enemas, transanal irrigations, and antegrade flushes. Those who fail conservative treatment require further assessment of colonic motility and can be candidates for colonic resection. The type of resection (subtotal colonic resection vs. Deloyer's procedure) can be guided with a balloon expulsion test. CONCLUSION: Most of the patients with FC referred for surgical evaluation can be managed conservatively. Further studies are required to determine an optimal strategy of surgical resection in children unresponsive to medical treatment.

11.
European J Pediatr Surg Rep ; 11(1): e1-e4, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36760663

RESUMO

Introduction Cloacal malformations comprise a heterogeneous group of anomalies that are considered the most complex anorectal malformations (ARMs) in females. Precise evaluation to identify the unique anatomy prior to reconstruction with collaboration between colorectal surgeons, urologists, and gynecologists is vital. Here, we present a rare anatomical variation in a patient with a cloacal malformation which affected operative and postoperative management. Case description A 6-year-old female with cloaca who underwent colostomy, vaginostomy, and vesicostomy as a newborn presented for reconstruction. Her VACTERL workup was negative except for an atretic right kidney. Her ARM index included the cloaca, a normal spine, and sacrum with a lateral sacral ratio of 0.7, predicting good potential for bowel continence. Cystoscopy through the vesicostomy showed a small bladder with normal ureteral orifices, and a closed bladder neck, with no identifiable urethra. A cloacagram showed an atretic common channel, a single small vagina, and a rectum below the pubococcygeal line. The patient underwent a posterior sagittal anorectovaginourethroplasty, vaginal patch using rectum, rectoplasty, and perineal body reconstruction. The urethra was not amenable to reconstruction, so the vesicostomy was preserved and a future Mitrofanoff was planned. Conclusion Urethral atresia is a rare and challenging finding in cloaca patients, and a vesicostomy is needed to drain urine in the newborn period. Preoperative examination under anesthesia, cystoscopy, vaginoscopy, and cloacagram are crucial to identify the precise anatomy and to plan accordingly.

12.
Eur J Pediatr Surg ; 2023 Mar 07.
Artigo em Inglês | MEDLINE | ID: mdl-36693415

RESUMO

OBJECTIVE: Wound dehiscence after posterior sagittal anorectoplasty (PSARP) or anorectal vaginal urethroplasty (PSARVUP) for anorectal malformation (ARM) is a morbid complication. We present a novel anoplasty technique employing para-U-stitches along the anterior and posterior portions of the anoplasty, which helps buttress the midline U-stitch and evert the rectal mucosa. We hypothesized that, in addition to standardized pre- and postoperative protocols, this technique would lower rates of wound dehiscence. MATERIALS AND METHODS: A retrospective review of patievnts who underwent primary PSARP or PSARVUP with the para-U-stitch technique from 2015 to 2021 was performed. Wound dehiscence was defined as wound disruption requiring operative intervention within 30 days of the index operation. Superficial wound separations were excluded. Descriptive statistics were calculated. The final cohort included 232 patients. RESULTS: Rectoperineal fistula (28.9%) was the most common ARM subtype. PSARP was performed in 75% and PSARVUP in 25%. The majority were reconstructed with a stoma in place (63.4%). Wound dehiscence requiring operative intervention occurred in four patients, for an overall dehiscence rate of 1.7%. The dehiscence rate was lower in PSARPs compared with PSARVUPs (0.6 vs. 5.2%) and lower for reconstruction without a stoma compared with a stoma (1.2 vs. 2.0%). There were additional six patients (2.6%) with superficial wound infections managed conservatively. CONCLUSION: We present the para-U-stitch anoplasty technique, which is an adjunct to the standard anoplasty during PSARP and PSARVUP. In conjunction with standardized pre- and postoperative protocols, this technique can help decrease rates of wound dehiscence in this patient population.

13.
Eur J Pediatr Surg ; 33(6): 463-468, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36356590

RESUMO

BACKGROUND: The posterior sagittal anorectoplasty (PSARP) used to repair an anorectal malformation (ARM) with a rectovestibular fistula involves incising the perineal body skin and the sphincter muscles and a posterior sagittal incision to the coccyx. Perineal body dehiscence is the most common and morbid complication post-PSARP which can have a negative impact on future bowel control. With consideration of all the other approaches described to repair this anomaly, we developed a perineal body sparing modification of the standard PSARP technique. METHODS: Four patients with ARM with a rectovestibular fistula were repaired with a perineal body sparing modified PSARP at a single institution between 2020 and 2021. The incision used was limited, involving only the length of the anal sphincter, with no incision anterior or posterior to the planned anoplasty. Dissection of the distal rectum and fistula was performed without cutting the perineal body. Once the distal rectum was mobilized off the posterior vaginal wall and out of the vestibule, the perineal body muscles, where the fistula had been, were reinforced and an anoplasty was then performed. RESULTS: Operative time was the same as for a standard PSARP. There were no intraoperative or postoperative complications. No postoperative dilations were performed. All patients healed well with an excellent cosmetic result. All are too young to assess for bowel control. CONCLUSION: We present a new technique, a modification of the traditional PSARP for rectovestibular fistula, which spares the perineal body. This approach could eliminate the potential complication of perineal body dehiscence.


Assuntos
Malformações Anorretais , Fístula Retal , Feminino , Humanos , Lactente , Reto/cirurgia , Malformações Anorretais/cirurgia , Estudos Retrospectivos , Fístula Retal/cirurgia , Canal Anal/cirurgia , Resultado do Tratamento
14.
J Pediatr Surg ; 58(2): 246-250, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36411110

RESUMO

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.


Assuntos
Incontinência Fecal , Doença de Hirschsprung , Criança , Humanos , Enema/métodos , Colo/cirurgia , Constipação Intestinal/diagnóstico , Constipação Intestinal/etiologia , Constipação Intestinal/cirurgia , Colo Sigmoide/cirurgia , Colostomia/métodos , Doença de Hirschsprung/complicações , Doença de Hirschsprung/cirurgia , Estudos Retrospectivos , Incontinência Fecal/cirurgia
15.
J Pediatr Surg ; 58(2): 189-197, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36418202

RESUMO

A basic premise in the care of complex patients is that experience, increased volume of cases, and an integrated, multi-disciplinary approach yields improved outcomes. Is this true using the example of the care of children with colorectal and pelvic reconstructive needs? This review gives a brief historical context on how care for this patient group evolved, delineates the key elements to create a collaborative care model, and describes multiple advances that have been developed, based on the model, which have improved patient care and quality of life. LEVEL OF EVIDENCE: Review.


Assuntos
Neoplasias Colorretais , Procedimentos de Cirurgia Plástica , Humanos , Criança , Qualidade de Vida , Pelve
16.
Eur J Pediatr Surg ; 33(3): 241-248, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35853466

RESUMO

INTRODUCTION: Anal sphincter botulinum toxin injections (BTIs) are used in the treatment of children with severe defecation disorders, including Hirschsprung disease (HD) and functional constipation (FC). Our objective was to evaluate the outcomes of BTI in these children. MATERIALS AND METHODS: We performed a prospective cohort study of children undergoing BTI from July 2018 to December 2018. We recorded perceived effect of the BTI, including effectiveness ranging from 0 (not at all effective) to 4 (extremely effective). In addition, we recorded symptoms and the Cleveland Clinic Constipation Score (CCCS). Data were collected at baseline and at 2 weeks, 2 months, and 4 months post-injection. RESULTS: Forty-two children (HD = 25, FC = 17) were included in the study (median age 4.3 years, IQR 2.4-7.2, 52% male). Twenty-two (88%) children with HD and eight (47%) children with FC had previously undergone a BTI. BTIs were perceived effective in 16 (76%) and 12 (71%) children with HD and eight (47%) and seven (47%) children with FC at 2-week and 2-month follow-up, respectively. Effectiveness was not rated differently between groups except at the 2-month follow-up, when patients with HD rated the BTI more effective compared to those with FC (median 2 [HD] vs. median 1 [FC], p = 0.022). Over the course of the study, 17/39 (44%) children reported self-limiting adverse effects such as fecal incontinence and pain at the injection site. CONCLUSION: Anal sphincter BTIs can be effective in the treatment of constipation in both HD and FC patients.


Assuntos
Toxinas Botulínicas , Doença de Hirschsprung , Criança , Humanos , Masculino , Pré-Escolar , Feminino , Canal Anal , Doença de Hirschsprung/complicações , Doença de Hirschsprung/tratamento farmacológico , Estudos Prospectivos , Resultado do Tratamento , Constipação Intestinal/tratamento farmacológico , Constipação Intestinal/etiologia
17.
J Pediatr Surg ; 58(3): 484-489, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36470689

RESUMO

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.


Assuntos
Síndrome de Down , Incontinência Fecal , Doença de Hirschsprung , Humanos , Canal Anal/cirurgia , Doença de Hirschsprung/cirurgia , Incontinência Fecal/etiologia , Incontinência Fecal/cirurgia , Manometria , Doença Iatrogênica , Resultado do Tratamento
18.
European J Pediatr Surg Rep ; 10(1): e156-e159, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36466361

RESUMO

We present a rare case of a 2-year-old male patient referred for primary evaluation of constipation and ultimately treatment of Hirschsprung disease (HSCR) whose preoperative workup incidentally revealed a posterior paraspinal mass. Following the biopsy of the mass, the patient exhibited hypoventilation and hypoxia requiring a delayed extubation, raising suspicion for congenital central hypoventilation syndrome (CCHS). We focus on the known history of associations between HSCR and CCHS, in addition to recently found genetic mutations in paired-like homeobox 2B that link HSCR, CCHS, and neuroblastoma.

19.
European J Pediatr Surg Rep ; 10(1): e145-e147, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36225532

RESUMO

We present a case of a rare complication in a 10-month-old female referred to our institution for an anal stricture after primary cloacal repair as an infant. Multimodal imaging, careful physical exam, and endoscopic evaluation revealed her vagina had been pulled through to the location of her anal sphincter muscle complex. We describe the correction of this problem, including identification of her rectum.

20.
European J Pediatr Surg Rep ; 10(1): e118-e121, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-35992311

RESUMO

We present a case of a newborn male with imperforate anus who was found to have colonic triplication with a high rectovesical fistula. The case is presented with a focus on surgical strategies for the management of this rare malformation.

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