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quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "In patients who have had a vaginal replacement, the most common long-term complication is stenosis of the neovagina. This can often be diagnosed with an exam under anesthesia and managed with dilations but may require a revision. If bowel interposition was used for vaginal replacement, patients may experience prolapse of the interposition, excessive mucus production, bleeding, mal-odor, and dyspareunia. Malignancy of the bowel graft has been reported, as has inflammatory bowel disease, in the segment. These are the main reasons why bowel neovaginas nowadays are avoided. These complications are eliminated in patients bridged with buccal mucosa."

Quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "For patients with uterine abnormalities, obstruction of menses is possible, and surgical correction may become necessary. Pregnancies in patients with vaginal anomalies are often considered high risk, and delivery options need to be discussed with maternal fetal medicine. The pelvic floor muscles are often not normally developed or have been iatrogenically altered, making vaginal delivery after pregnancy more traumatic. This is especially true if the perineal body was divided during reconstruction. Most patients with a history of anorectal and/or vaginal malformations are advised to use c-section as the mode of delivery."

quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "Low ARMs, like recto-vestibular and perineal fistula, tend to be associated with congenital rectal dilation above the fistula. The dilated rectum is prone to constipation, and early bowel management is recommended. Bowel management in this population utilizes laxatives and fiber to keep the rectum empty and avoid distention of the dilated rectum. The pelvic floor and sphincter muscle may be more robust in these distal malformations; however, incontinence is still possible, especially in the setting of an associated spinal or sacral anomaly. Pelvic floor physiotherapy can assist with developing the ability to fully evacuate stool and hold onto the stool, both needed to improve continence. Rectal or ante-grade enemas can be helpful in achieving social continence when laxatives are ineffective and can be used as a bridge until the child achieves the capacity for voluntary bowel movements."

Cardiac tamponade after subclavian vein porta cath insertion, case for discussion sent by a colleague male patient 2 yrs old presented with ACUTE LYMPHOBLASTIC LEUKEMIA, presented for porta cath insertion, with low platelets count. After correction of thrombocytopenia, under GA, percutaneous needle inserted to cannulate the right subclavian vein, infra clavicular, succeeded from first trial without issues, then guide wire inserted and position checked with fluoroscopy in the right atrium, followed by catheter insertion smoothly, catheter connected to the porta cath chamber with good inflow and outflow no resistance. Suddenly during skin closure patient became bradycardic then arrested, CPR started and patient returned after few cycles. We repeated portable chest xray in OR while patient still intubated and no clear evidence of hemo or pnemo thorax. Yet patient still hypotensive and about to arrest, so needle decompression tried to right chest nothing came out followed by right chest tube and no blood or air came out. Bed side echo done and cardiac tamponade found. cardiac surgery colleague contacted to join then we inserted subxyphoid needle and aspirated blood 150 cc. Decision made with cardiac surgery to proceed with median sternotomy, pericardium opened blood came out marked amount, cardiac massaging started, heart started beating, on assessment of heart no cardiac injury found and normal svc and ivc, Yet injury found at the innominate veins bifarcation which is repaired, and sternotomy closed. Patient survived but with neurological sequel. the injury could be due to the guide wire or due to the catheter, the question, is there any thing could be done to avoid this rare complication of central venous insertion?, and weather there is malpractice in the management of this patient ?

quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "Determining the presence of an upper vagina and uterus is important for future planning, and this anatomy can be clarified with diagnostic laparoscopy. One scenario is visualization of an upper vagina. In such a case, it is recommended to note the anatomy, and at the time of puberty, the vagina can be mobilized via laparoscopy or laparotomy to reach the perineum for an anastomosis to the introitus. Most commonly, there are no Müllerian structures, just remnants of Fallopian tubes. In such a case, dilation of the introitus later in life is possible. Vaginal replacements with bowel are avoided as they do not produce good long-term outcomes and there is no immediate indication or urgency to perform this operation at this age. In such a case, a buccal neovagina is an excellent option later in life, and in the future a tissue engineered vagina may be an option."

quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji - "Up to 10% of female infants with ARM have associated gynecologic anomalies, including an absent vagina in less than 1% of cases. Vaginoscopy is helpful to characterize the fistula and check for a vagina. An absent vagina may not be immediately apparent in the setting of a recto-vestibular fistula, especially if the fistula is mistaken for a vagina and a rectovaginal fistula is assumed to be present. In fact, absence of the vagina is only recognized about 50% of the time prior to anorectoplasty. When an absent vagina is suspected preoperatively, complete work-up can be pursued using US and/or MRI to delineate the pelvic organs although these are often unhelpful as the structures hoping to be seen are so small."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "The most frequent complication in the first month is outflow failure, which is usually caused by omentum or remnant portions of the omentum obstructing the catheter. If there are inflow problems as well, the trouble may be due to malposition. Laparoscopic exploration, repositioning, and cleaning out of the catheter can be successful, but replacement is necessary in most cases. Leakage is also common immediately after placement. It can be treated by decreasing the amount of dialysate and increasing the number of exchanges. Nearly all of these leaks will seal with conservative management unless the inner cuff has been dislodged."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "Hydrometrocolpos has been classified into five types (Fig. 73.1) on the basis of the type and level of obstruction: 1. Low hymenal obstruction. 2. Mid-plane transverse membrane or septum. (a) Without communication. (b) With a small orifice as communication. 3. High obstruction with distal vaginal atresia. (a) Without perineal swelling. (b) With perineal swelling. 4. Vaginal atresia with persistence of the urogenital sinus. 5. Vaginal atresia with cloacal anomaly."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "Hydrometrocolpos presents at the two extremes of childhood: initially during the neonatal period, when there is a high level of maternal hormones, and then at early puberty, when the patient herself begins to have production of estrogenic hormones. The distal vaginal obstruction is mostly due to imperforate hymen (in two thirds of cases), followed by a transverse vaginal septum and less commonly, vaginal atresia (with or without persistence of a urogenital sinus or cloaca)." "Associated anomalies are common and quite often severe. These include anorectal malformations and unilateral or bilateral agenesis of the kidneys, ureters, and trigone. Ultrasonography, micturating cystourethrography (MCU), and MR urography can help to evaluate the associated genitourinary anomalies."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "Several problems have been encountered during the establishment of the ACE. The most common is pain during the washout, which is reported in up to 60% of patients. This usually settles spontaneously during the first 3 months but can be helped by reducing the concentration of the phosphate, reducing the rate of the infusion, or using an antispasmodic prior to the enema (such as Colofac, Solvary, UK). Despite regular washouts, patients may still become constipated, which also produces pain and should always be excluded. One of the other problems encountered is the time taken for the enema to pass and achieve a result; this has been a significant contributory factor to failure. The placement of the conduit in the distal descending colon has improved this situation considerably. Patients in whom the standard caecal ACE did not work have achieved excellent results with the left colonic ACE."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "None of our patients have needed secondary augmentation because of increased contractile activity. Even after the ileal segment is isolated from the gastrointestinal tract, it retains its absorption and secretory properties. As a result of the electrolyte exchange, hyperchloremic metabolic acidosis may develop. The extent of the electrolyte exchange depends on the amount of intestinal surface area in contact with urine and the time that the urine remains in contact with the intestine. Patients with normal renal function prior to augmentation have adequate compensatory mechanisms and do not have significant problems with acute metabolic changes. Patients who develop persistent, severe metabolic changes must be evaluated for insufficient bladder emptying. Another concern in these patients is a possible increased incidence of urinary tract infection (UTI), as bacterial flora from the augmentation may colonize the urinary tract. In recent years, we have used in our patients daily irrigation of the augmented bladder with 30 mL of 120 mg gentamicin per 250 mL of normal saline, and have found a low incidence of UTI with no evidence of gentamicin absorption or renal function deterioration."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "The site of the stoma on the skin is selected entirely for the patient’s convenience. The umbilicus provides a good passage and the best cosmetic result, but it is not an option in exstrophy patients. With patients in wheelchairs, the spine tends to become twisted with time, causing progressive abdominal compression, and the abdomen becomes hidden from the patient’s field of vision. For these patients, a stoma site close to the xiphisternum is the best option."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "Augmentation cystoplasty is now commonly performed at most pediatric urological centres. Bladder augmentation has three major goals: to provide a compliant bladder reservoir, to limit bladder contractility, and to increase bladder capacity. Augmentation cystoplasty should allow the urinary tract to remain intact while preserving renal function and providing urinary continence. Various substrates are utilized to augment the bladder; the most commonly used is a segment of ileum, but stomach and large bowel also have been used. Ileum has been demonstrated to be the least contractile segment and therefore has become the tissue most often used for bladder augmentation. Sufficient augmentation should lead to effective bladder capacity."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "Successful initial closure of the bladder and posterior urethra is the most important factor for achieving urinary continence and sufficient bladder capacity. The fragile mucosa and the detrusor function are best preserved by closing the bladder in the newborn period, but the size and the functional capacity of the detrusor muscle are important considerations for the outcome. Therefore, in the rare presence of a small, fibrotic bladder patch without elasticity or contractility, the operation should be deferred until adequate growth of the bladder template occurs. The risk of bladder neck failure is higher for the group with smaller bladder capacities (<100 mL). If sufficient size is not reached 4–6 months after birth, alternative options like creation of a colon conduit or ureterosigmoidostomy must be employed. Later in life, the former urinary diversion can be converted into a continent catheterizable pouch bladder or augmented bladder if the template is still intact."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "Before the operation, the bladder capacity is measured annually by gravity cystograms with the child in anaesthesia. A bladder capacity of 100 mL or more is necessary to undergo bladder neck reconstruction. All children undergo an intense voiding training program, along with urodynamic evaluation, prior to the reconstruction."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "The ureteral stents are left in place for 10–14 days, and the suprapubic tube is removed 4 weeks postoperatively, after calibrating the bladder outlet to warrant free drainage. Note that the urethra is not stented at the end of the operation to avoid pressure necrosis, infection, and secretion accumulation. The pelvis is approximated in the midline by gently applying pressure over the greater trochanters bilaterally. Horizontal mattress sutures of #2 nylon are placed in the pubis. It is important to tie the knot away from the neourethra to avoid material migration into the posterior urethra. A second stitch of #2 nylon is used at the most caudal insertion of the rectus fascia onto the pubic bone for added security, if it can be easily done and does not compromise the first stitch."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "The operations are performed with general anaesthesia, with the patient in a supine position even for the osteotomies. A tunneled epidural catheter is placed when possible to reduce the intraoperative amount of anaesthetic agents and for postoperative pain control. It is typically left in place for several weeks after the closure. Care must be taken to create a latex-free environment in the operation room, as many children with bladder exstrophy are prone to latex allergies. Perioperative broad-spectrum antibiotics are administered and continued throughout the first postoperative week."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "Combined exstrophy and epispadias repair: In a few selected cases, newborn exstrophy closure can be combined with epispadias repair. This approach requires good phallic length, a deep urethral groove, and an adequate amount of penile skin, however, and it should be attempted only by experienced exstrophy surgeons, as the complications can be severe. Combined exstrophy and epispadias repair is best applied in the patient undergoing delayed primary or reoperative exstrophy closure. The preoperative use of intramuscular testosterone in reoperative exstrophy patients will allow for improved vascularity and more penile skin for the reconstruction."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "pelvic osteotomies are performed if the patient is older than 72 h, for a symphyseal diastasis of more than 4 cm or if a tension-free closure cannot be achieved. In those cases, osteotomies are crucial to ensure tension-free approximation of the bladder, posterior urethra, and abdominal wall, preventing dehiscence or bladder prolapse. Furthermore, it places the urethra deep within the pelvic ring, enhances bladder outlet resistance, and ensures alignment of the large pelvic floor muscles to support the bladder neck."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "Achieving urinary continence with a sufficient bladder capacity is strongly dependent on initial successful closure of the bladder and the posterior urethra as well as the size of the bladder template. Therefore, the first step of the reconstruction is conversion of bladder exstrophy into a penile epispadias with incontinence with a balanced posterior outlet resistance that preserves renal function but stimulates bladder growth."

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth - "The technique includes early closure of the bladder, posterior urethra, and abdominal wall, usually with pelvic osteotomy in the newborn period, subsequently followed by an early epispadias repair at 6 months to 1 year of age after testosterone stimulation by intramuscular injection. Around age 4–5 years, when adequate bladder capacity is reached and the child is ready to participate in a very structured preoperative and postoperative voiding program, a competent bladder neck is reconstructed, with bilateral ureteral reimplantation."