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1.
JNMA J Nepal Med Assoc ; 62(276): 542-544, 2024 Jul 31.
Article in English | MEDLINE | ID: mdl-39369402

ABSTRACT

ABSTRACT: Pentalogy of Fallot is a rare form of congenital cyanotic heart disease with a prevalence of 3/10,000 live births characterized by an association of Tetralogy of Fallot with Atrial Septal Defect. Pentalogy of Fallot with anorectal malformation is also a rare combination. Here we describe one of the rare case reports of a full-term, 38 weeks, female baby diagnosed with pentalogy of Fallot with imperforate anus and rectovaginal fistula at a tertiary care hospital. Pentalogy of Fallot combined with an imperforate anus and rectovaginal fistula is an exceptionally rare and complex congenital condition. The co- existence of these anomalies emphasizes the need for thorough prenatal and postnatal evaluation for early detection and management.


Subject(s)
Anus, Imperforate , Humans , Female , Anus, Imperforate/complications , Anus, Imperforate/diagnosis , Infant, Newborn , Rectovaginal Fistula/diagnosis , Rectovaginal Fistula/surgery , Pentalogy of Cantrell/diagnosis , Pentalogy of Cantrell/complications , Anorectal Malformations/complications , Anorectal Malformations/diagnosis , Tetralogy of Fallot/complications , Tetralogy of Fallot/diagnosis
2.
Pediatr Surg Int ; 40(1): 269, 2024 Oct 10.
Article in English | MEDLINE | ID: mdl-39387935

ABSTRACT

PURPOSE: The aim was to investigate sexual function and fertility in female adults operated on for anorectal malformations (ARM). METHODS: This was a cross-sectional questionnaire-based study including female adult patients treated for ARM at our institution between 1994 and 2003. Sexual function in females was assessed using the Profile of Sexual Function (PFSF). Additional questions regarding fertility were answered by the participants. Patient characteristics were retrospectively retrieved from the medical records and descriptive statistics were used for analysis. Sexual function outcomes were compared to a control group from a previously published group of females. Composite outcome analysis was performed using previously published data to determine the potential impact of bowel function and health-related quality of life on sexual function. The ethics review authorities approved the study. RESULTS: A total of 14 of 30 (46.7%) females responded to the questionnaires and had a mean age of 21.1 years (range 18-26). No association was found between PFSF and age or bowel function (Bowel Function Score), however, a strong correlation was found between PFSF and health-related quality of life (HRQoL) with a Spearman correlation of ρ 0.82 (p = 0.0011). The general satisfaction question was strongly associated with their total PFSF score (ρ = 0.71, p = 0.0092). Except for the "desire" item, the females in this cohort did not have significantly worse sexual function than the control population(p = 0.015). Ten of fourteen (71.4%) females had had their sexual debut at a mean age of 16.3 years and two of these women (20%) have been pregnant. All females had had menarche at a mean age of 12.7 years. CONCLUSION: Sexual function in adult females was comparable to healthy controls except for the "desire" item where the cohort reported poorer outcomes. The cohort's sexual function had a direct association with their reported HRQoL where individuals with worse HRQoL also reported poorer sexual function. LEVEL OF EVIDENCE: III.


Subject(s)
Anorectal Malformations , Quality of Life , Humans , Female , Cross-Sectional Studies , Adult , Anorectal Malformations/surgery , Anorectal Malformations/complications , Young Adult , Adolescent , Surveys and Questionnaires , Retrospective Studies , Fertility , Sexual Behavior/physiology , Sexual Dysfunction, Physiological/etiology
3.
J Pediatr Surg ; : 161974, 2024 Sep 27.
Article in English | MEDLINE | ID: mdl-39358082

ABSTRACT

INTRODUCTION: The optimal type of colostomy for patients with anorectal malformations (ARM) remains unclear. We conducted a systematic review and meta-analysis to compare the clinical outcomes of loop colostomies (LC) versus divided colostomies (DC) in patients with ARM. METHODS: After review registration (PROSPERO: CRD42024513335), we searched multiple databases for comparative studies on LCs and DCs in patients with ARMs. Gray literature was sought. The complications examined included stoma prolapse, urinary tract infection (UTI), skin excoriation, stoma retraction, parastomal hernia, wound infection rate, and stoma stricture. Three reviewers independently assessed the eligibility and quality of the included studies. Meta-analysis of selected complications was performed using Revman 5.4, with p < 0.05 considered significant. RESULTS: Eleven studies were included in the analysis, incorporating a total of 2550 neonates with ARMs, of which 1147 underwent LCs and 1403 underwent DCs. The meta-analysis revealed no significant differences between the two groups in the incidence of stoma prolapse (OR: 1.55, 95 % CI: 0.63 to 3.79; p = 0.34), UTIs (OR: 1.78, 95 % CI: 0.50 to 6.36; p = 0.38), skin excoriation (OR: 1.26, 95 % CI: 0.68 to 2.34; p = 0.46), stoma retraction (OR: 0.79, 95 % CI: 0.09 to 6.64; p = 0.83), parastomal hernia (OR: 0.99, 95 % CI: 0.22 to 4.48; p = 0.99), wound infection (OR: 0.35, 95 % CI: 0.10 to 1.20; p = 0.10), and stoma stricture (OR: 0.70, 95 % CI: 0.22 to 2.18; p = 0.53). CONCLUSIONS: The findings suggest that LCs and DCs are viable options for fecal diversion, presenting similar risks and benefits. The choice between these techniques should consider individual patient characteristics and surgical expertise. TYPE OF STUDY: Meta-analysis. LEVEL OF EVIDENCE: II.

4.
Ultrasound Obstet Gynecol ; 64(4): 521-527, 2024 Oct.
Article in English | MEDLINE | ID: mdl-39219041

ABSTRACT

OBJECTIVES: To introduce a two-dimensional sonographic method to assess the fetal anus, and to evaluate the feasibility of this method to diagnose anal atresia prenatally and identify the presence or absence of anoperineal fistula (in males) and anovestibular fistula (in females). METHODS: This was an observational study of suspected cases of anal atresia referred to a single center in Israel between August 2018 and October 2023. In addition to conventional evaluation of the perineum in the axial plane, fetuses referred to our center for suspected malformation were scanned with a new method termed the 'infracoccygeal/transperineal window'. This window consisted of a midsagittal view of the fetal pelvis, including the distal rectum and the anal canal. Normal anatomy was confirmed when the anal canal was continuous with the rectum and terminated at the expected location on the perineum. In female fetuses, the normal anal canal runs parallel to the vaginal canal and diverges posteriorly, terminating at the perineal skin, distant from the vestibule. In male fetuses, the normal anal canal diverges posteriorly in relation to the corpora cavernosa, terminating at the perineal skin, distant from the scrotum. High anal atresia was identified when a blind-ending rectal pouch was demonstrated in the pelvis without a fistula to the perineum or vestibule. Low anal atresia was determined when a rectal pouch was continuous with an anteriorly deflected fistula. In females, the fistula converges with the vaginal canal, terminating at the vestibule; in males, the fistula deflects anteriorly, terminating at the base of the scrotum. Postnatally, the diagnosis and type of anal atresia were confirmed through physical examination with direct visualization of the fistula, radiographic studies, surgical examination and/or postmortem autopsy. RESULTS: Of the 16 fetuses diagnosed prenatally with anal atresia, eight were suspected to have low anal atresia and eight were suspected to have high anal atresia. The median gestational age at diagnosis was 23 (range, 14-37) weeks. All cases showed additional structural malformation. Eleven patients opted for termination of pregnancy, of which four had low anal atresia and seven had high anal atresia. Postnatal confirmation was not available in four cases due to curettage-induced mutilation or in-utero degradation following selective termination of the affected twin, leaving 12 cases for analysis, of which seven were diagnosed with low anal atresia and five with high anal atresia. In these 12 cases, all prenatal diagnoses were confirmed as correct, rendering 100% sensitivity and 100% specificity in this high-risk fetal population. CONCLUSIONS: The infracoccygeal/transperineal window is an effective method to detect and classify the level of anal atresia prenatally. This may improve prediction of postnatal fetal continence and optimize prenatal counseling. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.


Subject(s)
Anal Canal , Anus, Imperforate , Ultrasonography, Prenatal , Humans , Female , Pregnancy , Male , Anal Canal/diagnostic imaging , Anal Canal/embryology , Anal Canal/abnormalities , Anus, Imperforate/diagnostic imaging , Anus, Imperforate/embryology , Ultrasonography, Prenatal/methods , Perineum/diagnostic imaging , Perineum/embryology , Feasibility Studies , Gestational Age , Rectal Fistula/diagnostic imaging , Rectal Fistula/embryology , Israel
5.
Khirurgiia (Mosk) ; (9): 106-109, 2024.
Article in Russian | MEDLINE | ID: mdl-39268743

ABSTRACT

Rectal prolapse is a common disease in childhood and observed mainly at the age of 1-4 years old (95% of cases). If conservative treatment is ineffective, surgical correction of rectal prolapse in children without previous anorectal surgery is performed at the age of over a year. There is a single report on examination of patients aged 4-16 years after surgical correction of anorectal malformations with postoperative rectal prolapse. We present diagnosis and successful surgical treatment of rectal prolapse in an infant who underwent previous perineal proctoplasty for fistulous form of anorectal malformation.


Subject(s)
Rectal Prolapse , Rectum , Humans , Rectal Prolapse/surgery , Rectal Prolapse/etiology , Rectal Prolapse/diagnosis , Rectum/surgery , Rectum/abnormalities , Treatment Outcome , Postoperative Complications/etiology , Postoperative Complications/surgery , Postoperative Complications/diagnosis , Male , Anorectal Malformations/surgery , Anorectal Malformations/diagnosis , Female , Child, Preschool , Reoperation/methods
6.
Sci Rep ; 14(1): 22217, 2024 09 27.
Article in English | MEDLINE | ID: mdl-39333170

ABSTRACT

To evaluate the necessity of performing whole spine magnetic resonance imaging (MRI) as opposed to solely lumbosacral MRI for detecting high-level spinal lesions (in the brain, cervical, or thoracic spine) in patients with anorectal malformations (ARM). This retrospective cohort study included 69 patients treated for ARM at a medical center between January 2011 and January 2022. Before March 2021, lumbosacral MRI was used for patients with ARM. Since March 2021, anomalies in the posterior cranial fossa and whole spine were assessed using routine MRI. Both male (n = 30, 43.4%) and female patients were included in the study. Out of the 69 patients, 19 (27.5%) underwent whole spine MRI, and 50 (72.5%) underwent lumbosacral MRI. In the whole spine group, 4 patients had anomalies beyond the lumbosacral area. In the lumbosacral group, 4 patients subsequently underwent whole spine MRI due to symptoms, with 3 revealing higher-level anomalies.High-level spinal and brain anomalies, although infrequent, are present in patients with ARM. The findings suggest considering routine whole spine MRI in screening ARM patients to avoid missing high-level anomalies.


Subject(s)
Anorectal Malformations , Magnetic Resonance Imaging , Spine , Humans , Male , Female , Magnetic Resonance Imaging/methods , Anorectal Malformations/diagnostic imaging , Retrospective Studies , Spine/diagnostic imaging , Spine/abnormalities , Spine/pathology , Child , Child, Preschool , Adolescent , Infant
7.
Pediatr Surg Int ; 40(1): 253, 2024 Sep 23.
Article in English | MEDLINE | ID: mdl-39311969

ABSTRACT

BACKGROUND: Posterior sagittal anorectoplasty and laparoscopic-assisted anorectal pull-through are preferred for anorectal malformation (ARM) today, while careful pull-through procedures with sacroperineal approach yield excellent outcomes. This study focuses on a pull-through procedure emphasizing continence mechanism preservation and compares outcomes with historical studies with various procedures. METHODS: Bowel function of patients with intermediate ARM followed up for over 10 years post-surgically was assessed. Data collected included ARM type with the Krickenbeck classification, comorbidities, complications, post-surgical examinations, follow-up, and bowel function at the latest clinic visit. The literature review collected original articles including more than 10 post-anorectoplasty cases which were followed for over 10 years. RESULTS: Eleven cases were identified, with a median age at anorectoplasty and follow-up length of 6.9 months and 14.4 years. Two fistula recurrences required surgical treatment. Long-term incontinence and constipation were observed in 9% and 45% of the cohort, respectively. Good rectal angulation and a positive rectoanal inhibitory reflex were confirmed in most cases examined. A literature review identified eight studies with various outcome-measuring instruments. CONCLUSION: Outcomes of the introduced pull-through procedure were favorable, while the literature review highlights the variation in outcomes of various anorectoplasty. EVIDENCE LEVEL: Level IV.


Subject(s)
Anorectal Malformations , Humans , Anorectal Malformations/surgery , Male , Female , Treatment Outcome , Infant , Follow-Up Studies , Child, Preschool , Postoperative Complications , Anal Canal/surgery , Anal Canal/abnormalities , Plastic Surgery Procedures/methods , Rectum/surgery , Rectum/abnormalities , Child , Retrospective Studies , Fecal Incontinence/etiology , Fecal Incontinence/surgery , Adolescent , Laparoscopy/methods
8.
Article in English | MEDLINE | ID: mdl-39293505

ABSTRACT

OBJECTIVE: Patients with anorectal malformations (ARMs) may have concurrent gynecologic abnormalities. As patients grow, they typically transition from pediatric subspeciality care and seek adult OB/GYN related services. We aimed to assess adult OB/GYN physicians' knowledge, competency, and comfort meeting the sexual and reproductive health care needs of patients with ARM. METHODS: We performed a cross-sectional observational survey-based study of graduates from a single academic OB/GYN residency program from 2013-2022. Physicians were surveyed on experience, comfort, and challenges caring for patients with ARMs and given a knowledge assessment. Descriptive and comparative statistics between those who did and did not complete a pediatric and adolescent gynecology (PAG) rotation were generated. RESULTS: There were 59 respondents (53.6%). Fewer than half (39.0%) report caring for a patient with ARM, an appendicovesicostomy (12.3%) or an appendicostomy (5.4%). Most felt uncomfortable (80.4%) or felt they lacked competence caring for these patients (81.8%). The majority (64.3%) felt ARMs should be discussed in residency. Only one physician (1.7%) answered all questions in the knowledge assessment correctly; 33.9% did not answer any question correctly. On subgroup analysis, more physicians completing a PAG rotation recalled learning about ARMs (83.3 vs 51.9%, p=0.03); however, there were no differences in experience, comfort, competence, or willingness to learn. CONCLUSION: OB/GYN providers report lack of knowledge and comfort in caring for patients with ARMs. Development of a standardized OB/GYN residency curriculum and education for practicing OB/GYN physicians is necessary to allow access to knowledgeable sexual and reproductive health for this patient population.

9.
J Pediatr Surg ; : 161697, 2024 Aug 18.
Article in English | MEDLINE | ID: mdl-39256064

ABSTRACT

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.

10.
J Pediatr Surg ; 59(10): 161624, 2024 Oct.
Article in English | MEDLINE | ID: mdl-39089895

ABSTRACT

BACKGROUND: This commentary discusses the social impact of bowel management programs (BMPs) on children with colorectal diseases, including anorectal malformations (ARM), Hirschsprung disease (HD), functional constipation (FC), and spina bifida. Previous studies focused on functional outcomes, but this study bridges the gap to daily life experiences. METHODS: The study examined children's experiences in BMPs, focusing on school participation, vacation ability, and overall patient experience. Cleanliness, defined as fewer than one stool soiling episode per week, was achieved by 70% of participants. RESULTS: Positive patient experiences were linked to achieving stool cleanliness, regardless of the management method. Invasive methods like enemas did not negatively affect experiences if cleanliness was maintained. Validated patient-reported experience measures (PREMs) and patient-reported outcomes measures (PROMs) were used, though the median age of 8.9 years posed limitations. CONCLUSION: The commentary highlights the significance of stool cleanliness in improving patient experiences and supports the effectiveness of various BMP methods. Future research should include longitudinal follow-ups to assess BMP durability and gather data from older children.


Subject(s)
Anorectal Malformations , Constipation , Spinal Dysraphism , Humans , Child , Constipation/therapy , Anorectal Malformations/surgery , Spinal Dysraphism/psychology , Spinal Dysraphism/complications , Hirschsprung Disease/therapy , Patient Reported Outcome Measures , Fecal Incontinence/therapy , Fecal Incontinence/psychology , Female , Male , Patient Satisfaction/statistics & numerical data
11.
Pediatr Surg Int ; 40(1): 220, 2024 Aug 09.
Article in English | MEDLINE | ID: mdl-39172191

ABSTRACT

PURPOSE: Surgical procedures for anorectoplasty for anorectal malformations (ARMs), particularly rectourethral fistula (RUF), depend on the institution. We investigated the diagnosis and treatment of RUF in male patients with ARMs in Japan using a questionnaire survey. METHODS: An online survey inquiring about the diagnosis and treatment (diagnostic modalities, surgical approaches, fistula dissection devices, and fistula closure techniques) of each type of ARM in male patients was conducted among institutional members of the Japanese Study Group of Anorectal Anomalies. Fisher's exact test was used to compare surgical methods between posterior sagittal anorectoplasty (PSARP) and laparoscopy-assisted anorectoplasty (LAARP). RESULTS: Sixty-one institutions (100%) completed the survey. LAARP was the preferred approach for high-type ARM (75.4%). PSARP was preferred for intermediate-type ARM (59.0%). Monopolar devices were most commonly used (72.1%) for RUF dissection. Blunt dissection was more frequent in the PSARP group (PSARP vs. LAARP: 55.6 vs. 20.0%, p < 0.005). Cystoscopy/urethroscopy to confirm the extent of dissection was used more frequently in the LAARP group (70.0% vs. 25.0%, p < 0.005). Clips and staplers were used more frequently in the LAARP group (p < 0.05). CONCLUSION: Distinct fistula management strategies for PSARP and LAARP were revealed. Further studies are needed to investigate the postoperative outcomes associated with these practices.


Subject(s)
Anorectal Malformations , Rectal Fistula , Urethral Diseases , Urinary Fistula , Humans , Male , Rectal Fistula/surgery , Rectal Fistula/diagnosis , Japan , Anorectal Malformations/surgery , Urinary Fistula/surgery , Urinary Fistula/diagnosis , Surveys and Questionnaires , Urethral Diseases/surgery , Urethral Diseases/diagnosis , Anus, Imperforate/surgery , Anus, Imperforate/diagnosis , Laparoscopy/methods
12.
J Pediatr Surg ; : 161652, 2024 Aug 02.
Article in English | MEDLINE | ID: mdl-39181779

ABSTRACT

PURPOSE: To analyze and compare the outcomes in patients with anorectal malformation with rectoprostatic and rectourethral fistula between laparoscopic-assisted anorectoplasty (LAARP) versus posterior sagittal anorectoplasty (PSARP). METHOD: We performed a retrospective review on all males with anorectal malformation (ARM) with recto-prostatic (ARM-RP) or recto-bulbar urethral fistula (ARM-RB) treated in five tertiary paediatric surgical centres in the past 25 years. Defecative function was assessed using the Krickenbeck classification and Kelly's score. Functional outcomes between patients with LAARP and PSARP were compared. RESULTS: There were a total of 136 males with ARM-RP and ARM-RB for analysis, among which 73 (53.7%) had ARM-RP and 63 (46.3%) had ARM-RB. The median age of the patients was 9.4 years (range 0.8-24.7 years) and the median age at operation was 0.4 years (0 day-3.1 years). 57 (41.9%) and 79 patients (58.1%) underwent PSARP and LAARP respectively. 34 patients (25%) had VACTERL association. 111 (81.6%) and 103 patients (75.7%) had sacral and spinal cord anomalies respectively. 19 patients (13.9%) eventually required Malone's Antegrade Continence Enema (MACE). For the comparison between PSARP and LAARP, no difference in Kelly scores (4.58 ± 1.63 versus 4.67 ± 1.36) was identified (p = 0.79). Logistic regression for voluntary bowel movement showed that VACTER association (p = 0.02) and fistula location (p = 0.01) were significant prognostic factors, whereas the operation approach (PSARP or LAARP) was not (p = 0.65). CONCLUSION: VACTERL association and fistula location were significant prognostic factors for voluntary bowel movement, and there appeared to be no significant difference in functional outcome between PSARP and LAARP. LEVEL OF EVIDENCE: IV.

13.
J Pediatr Urol ; 2024 Aug 07.
Article in English | MEDLINE | ID: mdl-39147608

ABSTRACT

INTRODUCTION: Anorectal malformations (ARMs) may be associated with congenital anomalies affecting other body parts namely vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb (VACTERL) with varying incidences of 7%-60% . Genitourinary defects might occur approximately in 50% of all patients with anorectal malformations hence patients should be evaluated from birth to rule out these defects. OBJECTIVE: To identify urological anomalies associated with anorectal malformation in southwestern Uganda. STUDY DESIGN: This was a descriptive retrospective cohort study conducted at our regional referral hospital in Southwestern Uganda involving patients who have undergone surgical correction of ARMs between June 2021 and July 2023. RESULTS: The overall prevalence of renal anomalies in our study patient population was 18.05%. Of those with ARM-associated renal anomalies, Specific anomalies included; renal agenesis (6.8%), hydronephrosis, (4.5%), duplex collecting system (3.8%), crossed fused kidney (1.5%), and ectopic kidney (0.75%). (Table) DISCUSSION: We found that the prevalence of ARM-associated renal anomalies was 18.05%, and the commonest anomaly was unilateral agenesis (6.8%) similar to other studies. Previous data have shown renal anomalies are common anomalies in ARM. While the exact values vary across studies, they all concluded that the rate of associated anomalies is extremely high in ARMs and warrants a thorough preoperative investigation once the ARMs are detected. This finding therefore underscores the importance of thorough evaluation and a multidisciplinary approach of care and follow-up system for ARM management including urologists even when the children are asymptomatic now. The main limitation of our study was missing information on patients' charts, we were not able to get the diagnosis since most patients didn't have their discharge forms at the time of evaluation. CONCLUSION: ARM associated with renal anomalies may remain undiagnosed and asymptomatic. Those identified as asymptomatic need to be followed in a multidisciplinary fashion including pediatric urologists.

14.
J Indian Assoc Pediatr Surg ; 29(4): 319-328, 2024.
Article in English | MEDLINE | ID: mdl-39149441

ABSTRACT

Context: Cloacal malformations are rare and are typically reported in females. There are a few scattered reports in males. It is not clear why they are so rare in males since both sexes negotiate this stage of embryonal development. Aims: The present study aims to share our experience and review all the cases of persistent cloaca and cloacal variants in males reported in the literature. Materials and Methods: The male cloaca is defined as a single common channel of varying lengths with separate inlets for the urinary tract (urethra) anteriorly and the rectum posteriorly at its cranial end and with a solitary perineal orifice/opening for external drainage. We also carried out an electronic literature search for cloaca, persistent cloaca, common cloaca, cloacal dysgenesis, cloacal malformation, cloacal membrane agenesis, urorectal malformation sequence, rectourinary perineal fistula, sirenomelia, and caudal regression syndrome. Results: After eliminating other cloacal anomalies and persistent cloaca in females, we found a total of 22 males with persistent cloaca or cloacal variant reported in the literature. In addition, we are adding two cases we have managed since our previous report. Conclusions: An effort should be made to search for the presence of the common channel in male patients with a single perineal opening. Recognition of the anomaly, width of the common cloacal channel, location of the rectal pouch with relation to the sacrum or pubis, status of the spine and sacrum, and nature of the anal sphincter are vital pieces of information to successfully manage the anomaly. It would be worthwhile if future reports on the subject also include long-term information about urinary and fecal functions and continence.

15.
J Indian Assoc Pediatr Surg ; 29(4): 334-339, 2024.
Article in English | MEDLINE | ID: mdl-39149440

ABSTRACT

Introduction: Reconstruction of a cloacal defect in a girl is often difficult and complicated.[1] This is most often done either by the sacro-perineal route or through the posterior sagittal route.[2] The procedures may involve total mobilization of the cloaca with or without the creation of a lower vagina with the help of a loop of vascularized bowel.[3] In our approach, such defects can be corrected using the lower anorectum for the creation of a lower vagina and abdominoperineal pull-through of the proximal divided bowel. This method can be used both in short and long common channel cloaca. Materials and Methods: We have used this procedure in one patient of posterior cloaca and four patients of anterior cloaca. These operations were done in patients of 1 year and 6 months to 4 years. Only one patient is waiting for the closure of the colostomy. Results: In all the cases, the urethra, the vagina, and the anus were successfully separated. Conclusion: This operation restores normal pelvic anatomy and physiology with minimal mutilation of the pelvic floor.

16.
Bioinformation ; 20(5): 566-570, 2024.
Article in English | MEDLINE | ID: mdl-39132234

ABSTRACT

Parenting starts much before the baby is born. It always comes with mixed feelings during parenting. Therefore, it is of interest to report the quality of life and caregiver burden for Indian children with post-operative congenital anorectal malformations. We recruited total 56 caregivers for the present study. Data shows that the highest caregiver burden in economical and the lowest in psychological. Quality of life (QOL) was highest in physical domain and there was moderate negative correlation among caregiver burden, psychological health, and social relationship.

17.
Pediatr Surg Int ; 40(1): 235, 2024 Aug 19.
Article in English | MEDLINE | ID: mdl-39160337

ABSTRACT

PURPOSE: Early post-operative fever (< 48 h) is common in adults and children and seldom indicative of an infection. Guidance to limit excessive evaluation in adults is well-characterized but similar studies for the pediatric population is scarce. This study was performed to better clarify which infants should undergo investigation for post-operative fever after colorectal reconstructive surgical procedures. METHODS: We performed a retrospective chart review of all infants under one year of age who underwent elective reconstruction for anorectal malformations (ARM) and Hirschsprung Disease (HD) between June 2018 and April 2020 at a single institution. Patient and perioperative characteristics were analyzed to evaluate for possible factors associated with infection. RESULTS: Sixty-eight infants met study criteria - 38 (55.9%) had HD and 30 (44.1%) had ARM. Twenty-two infants (32.4%) had early post-operative fever. A definitive infectious cause was identified in only two infants. The presence of a colostomy pre-operatively and longer operative times were associated with increased risk of post-operative fever (62.5% vs. 22.7% and 175 min vs. 150 min respectively, p < 0.05). CONCLUSION: Early post-operative fever in infants after colorectal surgery is common and rarely associated with an infection. Further research is needed to determine which infants require further work-up and which can be safely observed.


Subject(s)
Fever , Hirschsprung Disease , Postoperative Complications , Humans , Retrospective Studies , Male , Female , Infant , Fever/etiology , Hirschsprung Disease/surgery , Hirschsprung Disease/complications , Postoperative Complications/epidemiology , Infant, Newborn , Anorectal Malformations/surgery , Risk Factors
18.
Children (Basel) ; 11(7)2024 Jun 27.
Article in English | MEDLINE | ID: mdl-39062235

ABSTRACT

Recent advancements in pediatric surgery have embraced telehealth (TH) modalities, transitioning from traditional in-person consultations to virtual care. This shift has broadened access to healthcare, potentially enhancing affordability, patient and caregiver satisfaction, and clinical outcomes. In pediatric colorectal surgery, telehealth has been effectively utilized to support Bowel Management Programs (BMPs) for children suffering from constipation and fecal incontinence. A systematic review was conducted to assess the effectiveness of virtual BMPs, analyzing studies from January 2010 to December 2023, sourced from MEDLINE (via PubMed), Embase, and the Cochrane Library, with five studies included. Remote BMPs, implemented through video or telephone consultations, reported satisfaction rates exceeding 75% among families, indicating a strong preference for virtual interactions over traditional visits. Significant findings from the studies include improvements in Vancouver and Baylor scores, reductions in the duration of multidisciplinary consultations, enhancements in pediatric quality of life and Cleveland scores, and decreased frequency of laxative treatments. The implementation of TH has facilitated patient-led care, enabling timely adjustments in treatment and efficient distribution of medical supplies. The findings suggest that virtual BMPs are a viable and effective alternative to conventional approaches, yielding high caregiver satisfaction and superior clinical outcomes while promoting patient independence.

19.
Pediatr Surg Int ; 40(1): 194, 2024 Jul 16.
Article in English | MEDLINE | ID: mdl-39014174

ABSTRACT

AIM: To study the published literature on robotic-assisted pull-through procedures for anorectal malformation. METHOD: A PubMed search was done on 10th April 2024, with the words "robotic AND Anorectal malformation". The articles were screened for relevance and the data were compiled on the safety, feasibility, technical details, and limitations of robotic-assisted procedures in children for anorectal malformation. RESULTS: The search robotic and anorectal malformation gave ten articles. Two were excluded as they were not relevant. Two articles were added from cross-reference. Ten publications on robotic-assisted procedures for anorectal malformation were studied, describing procedures in thirty-three cases. The youngest child operated was 3 months old. All except one case were done in males. Most articles were from the US and the Kingdom of Saudi Arabia (KSA). The principles involved in robotic-assisted anorectoplasty (RAARP) were the same as that of laparoscopic procedures. Complications reported included pelvic abscess, epididymo-orchitis, and stricture of pelvic tunnel or conversion to open. The magnification and endo-wrist technology of robotics facilitated the sharp dissection and ligation at origin of fistula. The mean operating time was 228.7 min (docking and console time), shortest being 86 min and mean hospital stay was 7 days. The number of ports used varied from 3 to 4 with 8.5 mm being the most commonly used umbilical port and 8 mm as working ports, although in one article, a 12 mm port was used for telescope. The prolonged operating time and cost are the two factors to be addressed in RAARP. CONCLUSION: Robotic surgery is feasible in infants with ARM and safe in expert hands. Robotics is a very effective tool with its better ergonomics, tremor filtration, 3D magnification, and dexterity. Increasing awareness and referral to high-volume centers can tide over the cost factor, and good training of the surgeons can reduce the operative time.


Subject(s)
Anorectal Malformations , Robotic Surgical Procedures , Humans , Robotic Surgical Procedures/methods , Anorectal Malformations/surgery , Male , Infant , Female , Operative Time , Rectum/surgery , Rectum/abnormalities
20.
Pediatr Surg Int ; 40(1): 193, 2024 Jul 16.
Article in English | MEDLINE | ID: mdl-39014287

ABSTRACT

PURPOSE: To study the published literature for various models used for simulation and training in the field of pediatric colorectal surgery. METHOD: A PubMed search was conducted for studies of simulation models in anorectal malformation on 24 March 2024 with the search words 'simulation pediatric colorectal surgery' followed by another search on 'simulation AND anorectal malformation' that gave 22 and 14 results, respectively (total 36). After removing 4 duplicate publications, 12 were found relevant to simulation and training in colorectal diseases. One publication relevant to the topic was added from literature, thirteen articles were studied. RESULTS: Of these, 5; 1; 4; and 3 were on inanimate models; animate model; 3D reconstructions; and training, respectively. Simulation models are available for posterior sagittal anorectoplasty. The same inanimate model was used in five articles. The animate model was based on a chicken cadaver. 3D models have been made for personalized preoperative assessment and to understand the imaging in anorectal malformation. One 3D model was made by regeneration of organoid epithelium. Training modules were made to evaluate surgical dissection, standardize surgical techniques, and improve proficiency. CONCLUSION: Simulation models are an important tool for teaching the steps of surgery and discussing the nuances of operative complications among mentors and peers. With advances in this field, the development of high-fidelity models, more training modules, and consensus on surgical techniques will benefit surgical training.


Subject(s)
Anorectal Malformations , Models, Anatomic , Simulation Training , Humans , Anorectal Malformations/surgery , Simulation Training/methods , Colorectal Surgery/education , Colorectal Surgery/methods
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