-
Hospital Pediatrics Aug 2023Disparities in pediatric health outcomes are widespread. It is unclear whether rurality negatively impacts outcomes of infants with surgical congenital diseases. This...
OBJECTIVES
Disparities in pediatric health outcomes are widespread. It is unclear whether rurality negatively impacts outcomes of infants with surgical congenital diseases. This study compared outcomes of rural versus urban infants requiring complex surgical care at children's hospitals in the United States.
METHODS
Rural and urban infants (aged <1 year) receiving surgical care at children's hospitals from 2016 to 2019 for esophageal atresia, gastroschisis, Hirschsprung's disease, anorectal malformation, and congenital diaphragmatic hernia were compared over a 1-year postoperative period using the Pediatric Health Information System. Generalized linear mixed effects models compared outcomes of rural and urban infants.
RESULTS
Among 5732 infants, 20.2% lived in rural areas. Rural infants were more frequently white, lived farther from the hospital, and lived in areas with lower median household income compared with urban infants (all P < .001). Rural infants with anorectal malformation and gastroschisis had lower adjusted hospital days over 1 year; rural infants with esophageal atresia had higher adjusted odds of 30-day hospital readmission. Adjusted mortality, hospital days, and readmissions were otherwise similar between the 2 groups. Outcomes remained similar when comparing urban infants to rural infant subgroups with the longest hospital travel distance (≥60 miles) and lowest median household income (<$35 000).
CONCLUSIONS
Despite longer travel distances and lower financial resources, rural infants with congenital anomalies have similar postoperative outcomes to urban infants when treated at children's hospitals. Future work is needed to examine outcomes for infants treated outside children's hospitals and to determine whether efforts are necessary to increase access to children's hospitals.
Topics: Humans; Child; Infant; United States; Esophageal Atresia; Gastroschisis; Rural Population; Anorectal Malformations; Retrospective Studies; Treatment Outcome; Hospitals
PubMed: 37470121
DOI: 10.1542/hpeds.2023-007227 -
Annals of African Medicine 2024Colostomy is one of the common surgical procedures performed in pediatric surgical practice. The aim of this study was to retrospectively review our experience with...
BACKGROUND
Colostomy is one of the common surgical procedures performed in pediatric surgical practice. The aim of this study was to retrospectively review our experience with colostomy and closure (reversal) in children.
PATIENTS AND METHODS
A retrospective review of the data of all children aged 15 years and below who had colostomy and colostomy closure in the past 5 years.
RESULTS
Of the 67 children who had colostomy 42 (62.7%) boys and 25 (37.3%) girls, with an age range between 13 months and 8 years. Fifty-six (83.6%) of the children were <2 years. Anorectal malformation 53 (79.1%) was the common indication. Divided colostomy was performed in 62 (92.5%) patients and loop colostomy was performed in 5 (7.5%) patients. All the patients had intraperitoneal colostomy closure. A complication rate of 26.4% was seen. Duration of hospital stay ranged between 4 and 10 days. No mortality was recorded.
CONCLUSION
Colostomy reversal is a safe procedure but morbidity may ensure and can easily manage.
Topics: Male; Child; Female; Humans; Infant; Colostomy; Retrospective Studies; Intestine, Large; Anorectal Malformations; Morbidity; Postoperative Complications
PubMed: 38358167
DOI: 10.4103/aam.aam_95_23 -
Journal of Pediatric Surgery Dec 2023Long-term gynecologic data are lacking to inform the care of patients with cloacal malformations. We seek to examine perceived sexual and reproductive health challenges...
STUDY OBJECTIVE
Long-term gynecologic data are lacking to inform the care of patients with cloacal malformations. We seek to examine perceived sexual and reproductive health challenges of patients born with cloacal anomalies and characterize the experiences of patients as adults.
DESIGN AND SETTING
Virtual semi-structured focus groups and single-participant interviews were conducted using an online video platform. Retrospective chart review was performed to abstract available demographics and surgical history.
PARTICIPANTS
Adult patients were contacted from a database of 143 patients born with cloaca who had been seen at or referred to a tertiary care pediatric colorectal center. Participants were recruited until data collection reached thematic saturation. Twenty patients aged 18-53 years participated in 5 focus groups and 3 single-participant interviews. The hospital IRB determined the research activities to be exempt from IRB review and oversight.
INTERVENTIONS AND MAIN OUTCOME MEASURES
Interviews and focus groups were transcribed and analyzed using the constant comparative method to identify themes regarding sexual and reproductive health and compared with medical and surgical history abstracted from chart review.
RESULTS
Participants reported many perceived barriers to intercourse and intimacy including bowel and bladder continence management. All participants reported discussion of pregnancy and fertility with healthcare providers as important. Other common themes included concerns about independence and transitioning from pediatric to adult providers.
CONCLUSION
Anorectal malformations are associated with sexual and reproductive health concerns. Patients seek guidance on family building, intimacy, and transition to adult care. Future quantitative study of these topics is needed to develop best practices for counseling and clinical management of these patients.
LEVEL OF EVIDENCE
Level VI.
TYPE OF STUDY
Prognosis Study.
Topics: Pregnancy; Animals; Humans; Adult; Female; Child; Retrospective Studies; Cloaca; Reproductive Health; Anorectal Malformations; Sexual Behavior; Qualitative Research
PubMed: 37690871
DOI: 10.1016/j.jpedsurg.2023.08.011 -
Archives of Disease in Childhood. Fetal... Dec 2023Anorectal malformations (ARMs) are a spectrum of congenital anomalies where there is abnormal development of the anus and rectum. With an incidence of 1:5000 live births... (Review)
Review
Missed or delayed diagnosis of anorectal malformations: a review of the literature, current training and practice in the UK in relation to detection via the NIPE programme.
Anorectal malformations (ARMs) are a spectrum of congenital anomalies where there is abnormal development of the anus and rectum. With an incidence of 1:5000 live births and affecting both males and females, these anomalies vary in their appearance and presentation, lack features enabling antenatal detection and should be detected at birth by the examining midwife or within 72 hours through the newborn and infant physical examination (NIPE) screening programme. However, it is recognised that the diagnosis of ARMs can be missed or delayed leading to morbidity and mortality. In the UK, despite the existence of the NIPE screening programme and NICE guidelines, published literature shows that nearly a quarter of ARMs are not diagnosed at birth. This review takes a critical look at the frequency of missed/delayed diagnosis of ARMs at birth, the implications of delayed diagnosis, and the possible reasons for this related to education and training of healthcare professionals involved in newborn examination, focusing on the UK national screening programme for NIPE. We propose a strategy for enhancing detection of ARMs in a timely manner through the existing framework of the NIPE screening programme.
Topics: Infant; Infant, Newborn; Male; Humans; Female; Pregnancy; Anorectal Malformations; Delayed Diagnosis; Rectum; Physical Examination; United Kingdom
PubMed: 36732046
DOI: 10.1136/archdischild-2022-324667 -
Journal of Pediatric Surgery May 2024Children with colorectal diseases such as anorectal malformations (ARM), Hirschsprung disease (HD), and functional constipation (FC) undergo bowel management programs...
BACKGROUND
Children with colorectal diseases such as anorectal malformations (ARM), Hirschsprung disease (HD), and functional constipation (FC) undergo bowel management programs (BMPs) to achieve cleanliness. While patient outcomes, such as cleanliness and quality of life, are well understood, patient experience, such as relationships, ability to participate in sports, and independence and self-confidence is less well understood. We aimed to assess the relationship between BMP and patient experience.
METHODS
A cross-sectional survey was administered to 295 patients ≥3 years old with ARM, HD, and FC completing BMP. The survey contains 22 questions regarding patient-reported experience measures (PREMs) and 11 regarding patient-reported outcomes measures (PROMs). Each was graded on a Likert scale, with higher scores meaning better experience. Scores were compared by demographics and clinical characteristics and logistic regression was performed controlling for clinically significant variables. A p-value of ≤0.05 was significant.
RESULTS
There were 205 eligible respondents (69.5%) with a median age of 8.9 years [IQR: 6.1-12.4]. ARM was most common (51.2%) and most achieved cleanliness on BMP (69.3%). There were no differences in experience scores by age, diagnosis, or bowel regimen. Patients that were clean had significantly higher PREM scores (67.7 [IQR: 64.0-83.0] vs. 64.8 [IQR: 55.0-70.1], p = 0.0002) and PROM scores (36.8 [IQR: 33.0-41.0] vs. 34.0 [31.0-38.5], p = 0.005). On regression analysis, cleanliness remained a strongly significant predictor of positive experience scores (β 7.37, SE 1.86, p < 0.0001).
CONCLUSIONS
Achieving cleanliness was associated with positive patient experience of bowel management programs. This finding suggests that achieving cleanliness, regardless of regimen, may allow patients the best functional and experiential outcomes.
PubMed: 38816305
DOI: 10.1016/j.jpedsurg.2024.05.002 -
Journal of Pediatric Surgery Oct 2023To provide a high-fidelity, animal tissue-based model for the advanced surgical simulation of a Posterior Sagittal Anorectoplasty (PSARP) for rectovestibular fistula in...
PURPOSE
To provide a high-fidelity, animal tissue-based model for the advanced surgical simulation of a Posterior Sagittal Anorectoplasty (PSARP) for rectovestibular fistula in anorectal malformation (ARM).
MATERIALS AND METHODS
A chicken cadaver was used to assess the feasibility of simulating a PSARP for rectovestibular fistula in ARM. No modification was required to implement the surgical simulation.
RESULTS
A detailed description of the high-fidelity surgical simulation model is provided. The PSARP can be simulated while providing realistic anatomy (e.g. common wall between rectovestibular fistula and vagina), adequate rectal size, location and placement of the rectovestibular fistula, and proximity to the vagina. Haptic conditions of the tissue resemble human tissue and operative conditions as well.
DISCUSSION
Concerning the decreased exposure of index cases of pediatric surgical trainees and pediatric surgeons in practice, simulation-based training can provide means to acquire or maintain the necessary skills to perform complex surgical procedures [1-5] Surgical simulation models for ARM are limited. Few low-cost trainers are available with predominant artificial and mostly unrealistic tissue [6-8] Animal models have the advantage of realistic multilayer tissue haptic feedback [6].
CONCLUSION
We provide a low-cost, high-fidelity model for correcting a rectovestibular fistula in a child with ARM, a complex operative procedure with low incidence but high-stake outcomes. The described tissue model utilizing the chicken cloaca anatomy provides a high-fidelity model for operative correction of rectovestibular ARM. For simulation purposes in the treatment of ARM, this model appears to be promising in terms of providing realistic pathology and haptic feedback in pediatric dimensions.
LEVEL OF EVIDENCE
V.
Topics: Female; Child; Humans; Infant; Retrospective Studies; Rectum; Rectal Fistula; Plastic Surgery Procedures; Anorectal Malformations; Anal Canal; Rectovaginal Fistula
PubMed: 36935227
DOI: 10.1016/j.jpedsurg.2023.02.055 -
African Journal of Paediatric Surgery :... Jan 2024Penile agenesis is complete absence of the penis in an otherwise normal phenotypic and genotypic male at birth that results from failure of development of the genital... (Review)
Review
Penile agenesis is complete absence of the penis in an otherwise normal phenotypic and genotypic male at birth that results from failure of development of the genital tubercle. It is an extremely rare anomaly that may be associated with anomalies in other organ systems, the extent and severity of which may affect the prognosis. The management is challenging and may have far reaching implications for the individual and family. While gender reassignment with bilateral orchidectomy and feminising genitoplasty has been carried out for most patients, significant psychosexual and social issues related to the male identity may occur due to foetal or postnatal sex steroid imprinting. We report a neonate with penile agenesis with bilateral renal agenesis and anorectal malformation.
Topics: Infant, Newborn; Humans; Male; Anorectal Malformations; Kidney Diseases; Genotype; Genitalia; Congenital Abnormalities; Kidney
PubMed: 38259027
DOI: 10.4103/ajps.ajps_81_22 -
Pediatric Surgery International Jul 2023To assess the number, characteristics, and functional short-, and midterm outcomes of patients with rectal atresia (RA) and stenosis (RS) in the ARM-Net registry.
PURPOSE
To assess the number, characteristics, and functional short-, and midterm outcomes of patients with rectal atresia (RA) and stenosis (RS) in the ARM-Net registry.
METHODS
Patients with RA/RS were retrieved from the ARM-Net registry. Patient characteristics, associated anomalies, surgical approach, and functional bowel outcomes at 1 and 5-year follow-up were assessed.
RESULTS
The ARM-Net registry included 2619 patients, of whom 36 (1.3%) had RA/RS. Median age at follow-up was 7.0 years (IQR 2.3-9.0). Twenty-three patients (63.9%, RA n = 13, RS n = 10) had additional anomalies. PSARP was the most performed reconstructive surgery for both RA (n = 9) and RS (n = 6) patients. At 1-year follow-up, 11/24 patients with known data (45.8%, RA n = 5, RS n = 6) were constipated, of whom 9 required stool softeners and/or laxatives. At 5-year follow-up, 8/9 patients with known data (88.9%, RA n = 4, RS n = 4) were constipated, all requiring laxatives and/or enema.
CONCLUSION
RA and RS are rare types of ARM, representing 1.3% of patients in the ARM-Net registry. Additional anomalies were present in majority of patients. Different surgical approaches were performed as reconstructive treatment, with constipation occurring in 46% and 89% of the patients at 1 and 5-year follow-up. However, accurate evaluation of long-term functional outcomes remains challenging.
Topics: Humans; Child, Preschool; Child; Rectum; Laxatives; Constriction, Pathologic; Rectal Diseases; Anorectal Malformations; Constipation; Anal Canal; Retrospective Studies
PubMed: 37507508
DOI: 10.1007/s00383-023-05518-7 -
Children (Basel, Switzerland) Mar 2024We aimed to compare among patients with high-type anorectal malformations (ARM): (i) short- and long-term outcomes of laparoscopic-assisted anorectoplasty (LAARP)... (Review)
Review
Short and Long-Term Outcomes of PSARP versus LAARP and Single versus Staged Repair for Infants with High-Type Anorectal Malformations: A Systematic Review and Meta-Analysis.
BACKGROUND
We aimed to compare among patients with high-type anorectal malformations (ARM): (i) short- and long-term outcomes of laparoscopic-assisted anorectoplasty (LAARP) compared to classic posterior sagittal anorectoplasty (PSARP) and (ii) the results of single-stage versus staged PSARP.
METHODS
Using a defined search strategy, two independent investigators systematically reviewed the English literature. PRISMA guidelines were followed, and meta-analysis was performed using RevMan5.3.
RESULTS
Of 567 abstracts screened, 7 papers have been included (254 pts; 121 PSARP, 133 LAARP) in the first systematic review and meta-analysis. The length of hospitalization was shortened in LAARP versus PSARP (10.9 versus 14.4 days; < 0.0001). PSARP and LAARP were comparable in terms of early postoperative complications (28.9% versus 24.7%; = ns) and rectal prolapse (21.6% versus 17.5%; = ns). At long-term follow-up, the presence of voluntary bowel movements (74.0% versus 83.5%; = ns) and the incidence of soiling (45.5% versus 47.6%; = ns) were similar in both PSARP and LAARP. Six papers (297 pts) were included in the second systematic review, with three comparative studies included in the meta-analysis (247 pts; 117 one-stage, 130 staged procedures). No significant difference in terms of presence of voluntary bowel movements after single-stage versus staged procedures (72.6% versus 67.3%; = ns) has been detected.
CONCLUSIONS
LAARP seems to be a safe and effective procedure, showing short- and long-term outcomes similar to PSARP. One-stage PSARP could be a safe alternative to the classic three-stage procedure, even for those infants with high-type ARM. Further and larger comparative studies would be needed to corroborate these partial existing data.
PubMed: 38539411
DOI: 10.3390/children11030376 -
Pediatric Surgery International Jul 2023During "bowel management week," abdominal radiographs are used to monitor the amount and location of stool. A radiologist familiar with the treatment plan can provide an...
INTRODUCTION
During "bowel management week," abdominal radiographs are used to monitor the amount and location of stool. A radiologist familiar with the treatment plan can provide an improved interpretation. The goal of this paper is to standardize the radiological reports during a bowel management week.
METHODS
We saw 744 patients during bowel management week from May 2016 until March 2023. Diagnosis included: anorectal malformation (397), idiopathic constipation (180), Hirschsprung disease (89), and spina bifida (78). Laxatives were the treatment for 51% of patients, and 49% received enemas. Characteristic radiographs were selected for each treatment group for a proposed reading standardization.
RESULTS
When the stool is visualized, it is crucial to report its location. Having a contrast enema helps with the correct interpretation of the colonic anatomy. It is also essential to always compare the amount of stool with the radiograph from the previous day to determine if there is an increase or decrease in stool. Examples of radiographs are shown to guide the use of the preferred proposed terminology.
CONCLUSION
Providing information regarding which treatment modality the patient is receiving and stating that a patient is on a bowel management week treatment is crucial for the radiologist to provide adequate interpretation. The radiologist must be familiar with the treatment goals and purpose of the daily radiograph.
Topics: Humans; Constipation; Laxatives; Intestines; Enema; Hirschsprung Disease; Fecal Incontinence
PubMed: 37468717
DOI: 10.1007/s00383-023-05513-y