Posterior Sagittal Approach Provides Optimal Exposure for Urethral Reconstruction in Children With a History of Anorectal Malformations.

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.

Cloacal Malformation with Associated Urethral Atresia.

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.

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.

Prenatal ultrasound-and MRI-based imaging predictors of respiratory symptoms at birth for congenital lung malformations.

BACKGROUND: Congenital lung malformations (CLM) are rare developmental anomalies of the fetal lung with a minority of patients exhibiting symptoms around the time of birth. Although ultrasound remains the gold standard, fetal MRI has recently been incorporated as an adjunct imaging modality in the workup and prenatal counseling of patients with CLM as it is thought to more accurately delineate lesion boundaries and diagnose lesion type. We evaluate what prenatal variables correlate with postnatal respiratory symptoms. METHODS: We performed a retrospective review of patients with prenatal diagnosis of CLM treated at our institution between 2006-2020. Fetal ultrasound and magnetic resonance imaging (MRI) parameters including maximal congenital pulmonary airway malformation volume ratio (CVR), absolute cyst volume, and observed to expected normal fetal lung volume (O/E NFLV) were correlated with outcomes including postnatal respiratory symptoms, need for supplementary oxygen or mechanical ventilation, delay in tolerating full feeds, resection in the neonatal period. RESULTS: Our study included 111 patients, all of whom underwent fetal ultrasound with 64 patients additionally undergoing fetal MRI. Postnatal respiratory symptoms were noted in 22.5% of patients, 19.8% required supplemental oxygen, 2.7% mechanical ventilation and two patients requiring urgent resection. Ultrasound parameters including absolute cyst volume and maximal CVR correlated with need for mechanical ventilation (p=0.034 and p=0.024, respectively) and for urgent resection (p=0.018 and p=0.023, respectively) and had a marginal association with postnatal respiratory symptoms (p=0.050 and p=0.052). Absolute cyst volume became associated with postnatal respiratory symptoms (p=0.017) after multivariable analysis controlling for maternal steroid administration and gestational age. O/E NFLV did not correlate with perinatal outcomes. CONCLUSION: We have found that ultrasound-based measurements correlate with postnatal respiratory symptoms, while MRI derived O/E NFLV does not. Further studies are needed to elucidate the role of MRI in the prenatal workup of congenital lung malformations. TYPE OF STUDY: Study of Diagnostic Test. LEVEL OF EVIDENCE: Level I.

Sparing the Perineal Body: A Modification of the Posterior Sagittal Anorectoplasty for Anorectal Malformations with Rectovestibular Fistulae.

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.

Unsupervised and quantitative intestinal ischemia detection using conditional adversarial network in multimodal optical imaging.

Purpose: Intraoperative evaluation of bowel perfusion is currently dependent upon subjective assessment. Thus, quantitative and objective methods of bowel viability in intestinal anastomosis are scarce. To address this clinical need, a conditional adversarial network is used to analyze the data from laser speckle contrast imaging (LSCI) paired with a visible-light camera to identify abnormal tissue perfusion regions. Approach: Our vision platform was based on a dual-modality bench-top imaging system with red-green-blue (RGB) and dye-free LSCI channels. Swine model studies were conducted to collect data on bowel mesenteric vascular structures with normal/abnormal microvascular perfusion to construct the control or experimental group. Subsequently, a deep-learning model based on a conditional generative adversarial network (cGAN) was utilized to perform dual-modality image alignment and learn the distribution of normal datasets for training. Thereafter, abnormal datasets were fed into the predictive model for testing. Ischemic bowel regions could be detected by monitoring the erroneous reconstruction from the latent space. The main advantage is that it is unsupervised and does not require subjective manual annotations. Compared with the conventional qualitative LSCI technique, it provides well-defined segmentation results for different levels of ischemia. Results: We demonstrated that our model could accurately segment the ischemic intestine images, with a Dice coefficient and accuracy of 90.77% and 93.06%, respectively, in 2560 RGB/LSCI image pairs. The ground truth was labeled by multiple and independent estimations, combining the surgeons' annotations with fastest gradient descent in suspicious areas of vascular images. The total processing time was 0.05 s for an image size of 256 × 256 . Conclusions: The proposed cGAN can provide pixel-wise and dye-free quantitative analysis of intestinal perfusion, which is an ideal supplement to the traditional LSCI technique. It has potential to help surgeons increase the accuracy of intraoperative diagnosis and improve clinical outcomes of mesenteric ischemia and other gastrointestinal surgeries.

A Deeper Curse: A Hirschsprung Patient's Evaluation Unmasks a Rare Association with Congenital Central Hypoventilation Syndrome and Neuroblastoma.

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.

Where Is the Vagina? A Rectal Stricture after a Presumed Cloacal Repair Turns Out to be the Mobilized Vagina and a Missed High Rectovaginal Fistula.

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.

Fetal and Newborn Management of Cloacal Malformations.

Cloaca is a rare, complex malformation encompassing the genitourinary and anorectal tract of the female in which these tracts fail to separate in utero, resulting in a single perineal orifice. Prenatal sonography detects a few cases with findings such as renal and urinary tract malformations, intraluminal calcifications, dilated bowel, ambiguous genitalia, a cystic pelvic mass, or identification of other associated anomalies prompting further imaging. Multi-disciplinary collaboration between neonatology, pediatric surgery, urology, and gynecology is paramount to achieving safe outcomes. Perinatal evaluation and management may include treatment of cardiopulmonary and renal anomalies, administration of prophylactic antibiotics, ensuring egress of urine and evaluation of hydronephrosis, drainage of a hydrocolpos, and creation of a colostomy for stool diversion. Additional imaging of the spinal cord and sacrum are obtained to plan possible neurosurgical intervention as well as prognostication of future bladder and bowel control. Endoscopic evaluation and cloacagram, followed by primary reconstruction, are performed by a multidisciplinary team outside of the neonatal period. Long-term multidisciplinary follow-up is essential given the increased rates of renal disease, neuropathic bladder, tethered cord syndrome, and stooling issues. Patients and families will also require support through the functional and psychosocial changes in puberty, adolescence, and young adulthood.

Characterization of Extracorporeal Membrane Oxygenation Support for Single Ventricle Patients.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) can provide crucial support for single ventricle (SV) patients at various stages of palliation. However, characterization of the utilization and outcomes of ECMO in these unique patients remains incompletely studied. METHODS: We performed a single-center retrospective review of SV patients between 2010 and 2017 who underwent ECMO cannulation with primary end point of survival to discharge and secondary end point of survival to decannulation or orthotopic heart transplantation (OHT). Multivariate analysis was performed for factors predictive of survival to discharge and survival to decannulation. RESULTS: Forty SV patients with a median age of one month (range: 3 days to 15 years) received ECMO support. The incidence of ECMO was 14% for stage I, 3% for stage II, and 4% for stage III. Twenty-seven (68%) patients survived to decannulation, and 21 (53%) patients survived to discharge, with seven survivors to discharge undergoing OHT. Complications included infection (40%), bleeding (40%), thrombosis (33%), and radiographic stroke (45%). Factors associated with survival to decannulation included pre-ECMO lactate (hazard ratio [HR]: 0.61, 95% confidence interval [CI]: 0.41-0.90, P = .013) and post-ECMO bicarbonate (HR: 1.24, 95% CI: 1.0-1.5, P = .018). Factors associated with survival to discharge included central cannulation (HR: 40.0, 95% CI: 3.1-500.0, P = .005) and lack of thrombotic complications (HR: 28.7, 95% CI: 2.1-382.9, P = .011). CONCLUSIONS: Extracorporeal membrane oxygenation can be useful to rescue SV patients with approximately half surviving to discharge, although complications are frequent. Early recognition of the role of heart transplant is imperative. Further study is required to identify areas for improvement in this population.

Extracorporeal Cardiopulmonary Resuscitation (ECPR) in Infants and Children: A Single-Center Retrospective Study.

BACKGROUND: Extracorporeal cardiopulmonary resuscitation (ECPR) is increasingly used to rescue patients with cardiac arrest refractory to conventional therapy, necessitating evaluation of factors that may affect outcomes. METHODS: A single-center retrospective review of pediatric patients (<21 years old) who underwent ECPR from January 2010 to November 2017. Comparisons between nonsurvivors and survivors, to decannulation and discharge, were made. Factors associated with survival and rate of complications were examined. RESULTS: Seventy patients were supported with ECPR. Forty-nine (70%) patients survived to decannulation and 38 (54%) patients to discharge. There was no statistical difference between baseline characteristics of survivors and nonsurvivors, including age at cannulation, weight (kg), time to cannulation (minutes), and total time on extracorporeal membrane oxygenation (hours). Survivors to discharge had significantly higher pH prior to cannulation compared to nonsurvivors (7.11 ± 0.24 vs 6.97 ± 0.21, P = .01). Of all, 23.2% of patients received renal replacement therapy (RRT), 39.4% had significant bleeding, 22.5% had thrombotic complications, and 68.8% had neurologic injury on imaging studies. A greater number of nonsurvivors received RRT compared to survivors to discharge (35.5% vs 10.8%, P = .02). There were no differences in bleeding or thrombotic complications or radiographically established neurologic injury. CONCLUSIONS: Although ECPR effectively increases overall survival, a better characterization of long-term outcomes is needed.