Megacystis-microcolon-intestinal hypoperistalsis syndrome: don't forget the bladder.

PURPOSE: Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a well described clinical condition, but reports are focused on microcolon and intestinal hypoperistalsis, while data on bladder management are scant. Aim of the study is to present urological concerns in MMIHS. METHODS: Retrospective evaluation of clinical data on urological management of MMIHS patients treated in the last 10 years. RESULTS: Six patients were enrolled (3 male, 3 female). Three girls had prenatal diagnosis of megacystis (1 vesicoamniotic shunt was placed). All patients had genetic diagnosis: 5 had ACTG2 gene mutations and 1 MYH11 mutation. All patients were addressed to our attention for urinary symptoms, such as urinary retention, urinary tract infections, acute renal injury. Two patients presented frequent stoma prolapses. All children underwent a complete urological evaluation, and then started a bladder management protocol (clean intermittent catheterization, via urethra or cystostomy-tube placement), with improvement of urinary infections, upper urinary tract dilation and stoma prolapses, if present. All patients had good renal function at last follow-up. CONCLUSION: We believe that MMIHS patients must be addressed soon and before onset of symptoms for a multidisciplinary evaluation, including an early assessment by a pediatric urologist expert in functional disorder, to preserve renal function at its best.

Chronic intestinal pseudo-obstruction due to adult-onset acquired isolated hypoganglionosis with muscular atrophy in the small intestine: a case report and review of literature.

BACKGROUND: Chronic intestinal pseudo-obstruction (CIPO) is a rare intestinal disorder characterized by impaired propulsion of the digestive tract and associated with symptoms of intestinal obstruction, despite the absence of obstructive lesions. CIPO includes several diseases. However, definitive diagnosis of its etiology is difficult only with symptoms or imaging findings. CASE PRESENTATION: A 56-year-old man was referred to our hospital due to a 3-year history of continuous abdominal distention. Imaging, including computed tomography of the abdomen, and endoscopy revealed marked dilatation of the entire small intestine without any obstruction point. Therefore, he was diagnosed with CIPO. Since medical therapy didn't improve his symptoms, enterostomy and percutaneous endoscopic gastro-jejunostomy were performed. These procedures improved abdominal symptoms. However, he required home central venous nutrition due to dehydration. The pathological findings of full-thickness biopsies of the small intestine taken during surgery revealed decreased number and degeneration of ganglion cells in the normal plexus. These findings led to a final diagnosis of CIPO due to acquired isolated hypoganglionosis (AIHG). CONCLUSIONS: Here, we report the case of a patient with CIPO secondary to adult-onset AIHG of the small intestine. Since AIHG cannot be solely diagnosed using clinical findings, biopsy is important for its diagnosis.

Eosinophilic Myenteric Ganglionitis Presenting as Sigmoid Volvulus: A Brief Report.

Eosinophilic myenteric ganglionitis (EMG) is a rare pathologic finding within the Auerbach myenteric plexus characterized by eosinophilic infiltration on light microscopy. The plexus's ultimate obliteration results in chronic intestinal pseudo-obstruction (CIPO). EMG is almost exclusively seen in the pediatric population. The diagnosis of EMG is made through full-thickness rectal biopsy and EMG is not detectable through routine screening measures such as imaging or colonoscopy. The current treatment modality for this disorder is not standardized, and has often been treated with systemic steroids given its eosinophilic involvement. This case presents a 73-year-old male with chronic constipation presenting with new obstipation in the setting of recent orthopedic intervention requiring outpatient opioids. Admission radiographs were consistent with sigmoid volvulus. Following endoscopic detorsion, exploratory laparotomy revealed diffuse colonic dilation and distal ischemia requiring a Hartmann's procedure. Surgical pathology revealed EMG, increasing the complexity of subsequent surgical decision-making after his urgent operation.

Study of the predictive factors affecting outcomes of patients undergoing transperitoneal laparoscopic donor nephrectomy.

PURPOSE: We aim to study the preoperative and intraoperative factors and compare against specific outcomes in patients undergoing transperitoneal laparoscopic donor nephrectomy and see if we could find what were the predictive factors for these outcomes. METHODS: This is a prospective cohort study done in a single high-volume transplant center. 153 kidney donors were evaluated over a period of 1 year. The preoperative factors such as age, gender, smoking status, obesity, visceral obesity, perinephric fat thickness, number of vessels, anatomic abnormalities, comorbidities, and side of kidney and intraoperative factors such as lay of colon on the kidney, height of splenic or hepatic flexure of colon, loaded or unloaded colon, and sticky mesenteric fat were compared against specific outcomes such as duration of surgery, duration of hospital stay, postoperative paralytic ileus, and postoperative wound complications. RESULTS: Multivariate logistic regression models were used to study the variables of interest against the various outcomes. There were three positive risk factors for increased hospital stay, which were perinephric fat thickness and height of splenic or hepatic flexure of colon and smoking history. There was one positive risk factor for postoperative paralytic ileus which is lay of colon with relation to kidney and there was one positive risk factor for postoperative wound complication which was visceral fat area. CONCLUSION: The predictive factors for adverse postoperative outcomes after transperitoneal laparoscopic donor nephrectomy were perinephric fat thickness, height of splenic or hepatic flexure, smoking status, lay or redundancy of colon with relation to kidney and visceral fat area.

Clinical Utility of Ileal Motility in Children With Defecation Disorders and Children With Chronic Intestinal Pseudo-Obstruction.

BACKGROUND: Little is known about ileal motility patterns and their utility in children. Here, we present our experience with children undergoing ileal manometry (IM). METHODS: A retrospective review of children with ileostomy comparing IM between 2 groups: A [chronic intestinal pseudo-obstruction (CIPO)] and B (feasibility of ileostomy closure in children with defecation disorders). We also compared the IM findings with those from antroduodenal manometry (ADM), and evaluated the joint effect of age, sex, and study indication group on IM results. RESULTS: A total of 27 children (median age 5.8 years old, range 0.5-16.74 years, 16 were female) were included (12 in group A and 15 in group B). There was no association between IM interpretation and sex; however younger age was associated with abnormal IM ( P = 0.021). We found a significantly higher proportion of patients with presence of phase III of the migrating motor complex (MMC) during fasting and normal postprandial response in group B than in group A ( P < 0.001). Logistic regression analysis revealed that only Group B was associated with normal IM ( P < 0.001). We found a moderate agreement for the presence of phase III MMC and postprandial response between IM and ADM (kappa = 0.698, P = 0.008 and kappa = 0.683, P = 0.009, respectively). CONCLUSION: IM is abnormal in patients with CIPO and normal in patients with defecation disorders, suggesting that IM may be not needed for ostomy closure in those with defecation disorders. IM has a moderate agreement with ADM and could be used as a surrogate for small bowel motility.

Anastomotic Leak Rate and Prolonged Postoperative Paralytic Ileus in Patients Undergoing Laparoscopic Surgery for Colo-Rectal Cancer After Placement of No-Coil Endoanal Tube.

BACKGROUND: Colorectal cancer (CRC) is the second most common gastrointestinal tumor in men and the third in women. Left-hemicolectomy (LC) and low anterior resection (LAR) are considered the gold standard curative treatment. In this retrospective study, we evaluated the presence or absence of post-operative complications, in all patients who underwent Video-laparoscopic (VLS) LAR/LC with No Coil trans-anal tube positioning, and compared the data with the current literature on the topic. METHODS: Thirty-nine patients diagnosed with CRC of the descending colon, splenic flexure, sigma, and rectum were recruited. LC was performed for sigmoid and descending colon cancers, while LAR was applied for tumors of the upper two-thirds of the rectum. The No Coil trans-anal tube (SapiMed Spa, Alessandria, Italy) was placed in all patients of the study at the end of surgical treatment. RESULTS: Eighteen patients received a LAR-VLS (46%) and 21 patients received a LC-VLS (54%). The average length of hospital stay after surgery was 7 days. PPOI occurred in only one in 39 patients (2.6%) who had undergone LAR-VLS. As for complications, in no patient of the study did AL (0%) occur. CONCLUSION: In patients undergoing LAR-VLS and LC-VLS, we performed colorectal anastomosis and in the same surgical operation we introduced the No-Coil device. Although this is a preliminary study and subject to further investigation, we believe that the No Coil tube positioning may reduce the time of presence of first flatus and feces and the risk of AL.

Collateral Damage From Fetal Interventions: Increasing the Complexity of Complex Patients.

Fetal interventions are often key to fetal survival and growth; however, they can often have complications causing significant morbidity and mortality. This case highlights not only a complication of fetal surgery, but also a very unusual diagnosis. We present the case of a male fetus who was diagnosed with urethral atresia and subsequently underwent 2 vesicoamniotic shunt placements. At birth, he was diagnosed with Megacystis Microcolon Intestinal Hypoperistalsis Syndrome and was noted to have rectovesical and vesicocutaneous fistulae likely iatrogenically created from shunt placement. While fetal interventions are often required, a multidisciplinary team approach is often necessary as complications occur.

Systemic lupus erythematosus simultaneously presenting with visceral muscle dysmotility syndrome and mechanical intestinal obstruction clinically relieved by surgery: a case report and literature review.

BACKGROUND: Intestinal pseudo-obstruction (IPO) accompanied by hepatobiliary dilatation and ureterohydronephrosis is extremely rare in systemic lupus erythematosus (SLE). This triad is also called visceral muscle dysmotility syndrome (VMDS). Only 9 cases have been reported in the literature. Here, we report a rare case of VMDS with mechanical intestinal obstruction that was clinically relieved by surgery. CASE PRESENTATION: This report refers to a 31-year-old woman with SLE and gastrointestinal symptoms presented as abdominal pain, nausea and stoppage of the passage of flatus or stool without obvious reasons. The patient suffered from severe abdominal distension because of massive flatulence. Contrast-enhanced computed tomography (CT) of the abdomen performed in our hospital showed localized stenosis of the bowel, ureterohydronephrosis, and biliary tract dilatation. Endoscopy showed a stenotic segment located in the sigmoid colon. The colon biopsy samples suggested that the stenosis was caused by inflammatory tissues. Biochemical investigations showed hypoalbuminemia, electrolyte disturbance and decreased C3. Antinuclear antibody was positive. After careful assessment, transverse colostomy was performed for this patient. Gastrointestinal symptoms were clinically relieved after the surgery. CONCLUSION: To the best of our knowledge, no VMDS patients have presented with mechanical ileus before. This case is the first documented occurrence of SLE with VMDS and mechanical intestinal obstruction symptoms relieved by surgery. Due to the low incidence of this condition, no standard treatment regimen has been established. However, surgical treatment offers significant benefit in specific situations.

CHRONIC PSEUDO-OBSTRUCTION OF THE SIGMOID COLON: A CASE REPORT.

Chronic intestinal pseudo-obstruction (CIPO) is a rare syndrome characterized by signs of intestinal obstruction lasting for 6 months or more, in the absence of a definitive cause of obstruction. We report a case of CIPO in a 49-year-old female patient with a 6-month history of ongoing irregular bowel movements, manifested as constipation and diarrhea accompanied by abdominal pain and bloated feeling. Contrast-enhanced abdominal computed tomography and magnetic resonance enterography revealed focal thickening of a segment of the lienal flexure and intermittent areas of wider and narrower caliber along the sigmoid colon. No signs of a definitive cause of obstruction were found, but evidence for dolichosigma was revealed, which was later confirmed with colonoscopy. Due to persisting symptoms, the patient agreed to elective resection of the sigmoid colon. Following the procedure, symptoms regressed with a significant improvement in the quality of life. The patient has been regularly monitored in an outpatient setting and reports absence of the symptoms since the procedure. Pathophysiology of the resected section revealed more prominent lymphatic tissue, follicular arrangement, and reactively altered germinal centers, which can suggest CIPO.

Preservation of native sigmoid colon for secondary continent cystostomy after multivisceral transplantation for chronic intestinal pseudo-obstruction.

Chronic intestinal pseudo-obstruction (CIPO) is characterized by severe digestive +/- urinary dysmotility. If the conservative management fails, multivisceral transplantation (MVT) may be needed. However, urinary dysmotility remains after MVT and requires to continue urinary catheterizations and/or drainage. We report on a boy with severe CIPO complicated by (1) chronic intestinal obstruction requiring total parenteral nutrition, decompression gastrostomy, and ileostomy; (2) recurrent line infections; (3) hepatic fibrosis; and (4) distension of the bladder and upper urinary tract, and recurrent urinary infections, leading to non-continent cystostomy for urinary drainage. He underwent MVT at the age of 5 years. The transplant included the liver, stomach, duodenum and pancreas, small bowel, and right colon. The distal native sigmoid colon was preserved. Fifteen months later, he underwent a pull through of the transplanted right colon (Duhamel's procedure), together with a tube continent cystostomy (Monti's procedure) using the native sigmoid. Postoperative course was uneventful, and the remaining ileostomy was closed 3 months later. Five years post-transplant, he is alive and well. He is fed by mouth with complementary gastrostomy feeding at night. He has 3-6 stools per day, with occasional soiling. The cystostomy is used for intermittent urinary catheterization 4 times/day and continuous drainage at night. He is dry, with rare afebrile urinary infections, normal renal function, and un-dilated upper urinary tract. Conclusion: in severe CIPO with urinary involvement, preservation of the distal native sigmoid colon during MVT allows secondary creation of a continent tube cystostomy, which is useful to manage persistent urinary disease.

Chronic intestinal psuedo-obstruction and MIDD, a rare cause of acute abdomen: implications in emergency surgery.

Chronic intestinal pseudo-obstruction (CIPO) is a condition typified by the failure of the small bowel to propel contents in the absence of physical obstruction. CIPO is diagnosed after eliminating other causes, presenting a diagnostic challenge in emergency surgery. We report a case of a 32-year-old man with a rare mitochondrial disorder, Maternally inherited diabetes and deafness (MIDD), who presented to our hospital acutely unwell with peritonitis. Laparotomy revealed distended small bowel with no transition point, and turbid fluid with no macroscopic source. Postoperatively he had severe electrolyte and vitamin deficiencies. The diagnosis of CIPO leading to paralytic ileus and bacterial translocation was established and managed with aggressive electrolyte and vitamin replacement. He was discharged day 12 post operatively after a prolonged ileus with follow-up from a quaternary metabolic unit. We discuss here the challenges and gold standard in the emergency management of CIPO.

Recurrent spontaneous small bowel perforations with a rare pathology: non-familial visceral myopathy.

Visceral myopathy is a rare, often misdiagnosed disease characterised by abnormalities in the gastrointestinal smooth muscle layer. Its clinical presentation includes pseudo-obstruction, intestinal dysmotility and spontaneous perforation. We report an atypical case of a 51-year-old man with a history of recurrent small bowel perforations who presented with an acute abdomen. A laparotomy was performed with identification of a distal jejunal perforation. A small bowel resection with end-to-end anastomosis was done. Minimal adhesions were seen intraoperatively despite the patient's multiple prior surgeries. Pathology showed histiocytic inflammation and patchy loss of the muscle layer reflective of visceral myopathy. Genetic testing revealed a variant of uncertain significance in the myosin light chain kinase gene. It is difficult to make a conclusive diagnosis given the patient's clinical presentation closely mimicking other gastrointestinal disorders. However, it is crucial to consider visceral myopathy in patients with recurrent spontaneous intestinal perforations as a differential diagnosis.

Percutaneous endoscopic gastrojejunostomy in pediatric intestinal pseudo-obstruction.

Pediatric intestinal pseudoobstruction (PIPO) is the "tip of the iceberg" of the most severe gut motility disorders. In patients with PIPO, the impairment of gastrointestinal propulsive patterns is such as to result in progressive obstructive symptoms without evidence of mechanical causes. PIPO is an important cause of intestinal failure and affects growth and pubertal development. Bowel loop and abdominal distension represent one of the main features of intestinal pseudo-obstruction syndromes, hence intestinal decompression is a mainstay in the management of PIPO. So far, pharmacologic, endoscopic, and surgical treatments failed to achieve long-term relief of bowel distension and related symptoms, including pain. Recent data, however, indicated that percutaneous endoscopic gastrojejunostomy (PEG-J) might be a minimally invasive approach for intestinal decompression, thereby improving abdominal symptoms and nutritional status in adult patients with chronic intestinal pseudo-obstruction. Based on these promising results, we treated for the first time a 12-y-old patient affected by PIPO refractory to any therapeutic options to obtain intestinal decompression by PEG-J. We showed that PEG-J yielded sustained small bowel decompression in the reported PIPO patient with considerable improvement of both abdominal symptoms and nutritional status. The positive outcome of the present case provides a basis to test the actual efficacy PEG-J versus other therapeutic approaches to intestinal decompression in patients with PIPO.

Twenty Years of Gut Transplantation for Chronic Intestinal Pseudo-obstruction: Technical Innovation, Long-term Outcome, Quality of Life, and Disease Recurrence.

OBJECTIVE: To define long-term outcome, predictors of survival, and risk of disease recurrence after gut transplantation (GT) in patients with chronic intestinal pseudo-obstruction (CIPO). BACKGROUND: GT has been increasingly used to rescue patients with CIPO with end-stage disease and home parenteral nutrition (HPN)-associated complications. However, long-term outcome including quality of life and risk of disease recurrence has yet to be fully defined. METHODS: Fifty-five patients with CIPO, 23 (42%) children and 32 (58%) adults, underwent GT and were prospectively studied. All patients suffered gut failure, received HPN, and experienced life-threatening complications. The 55 patients received 62 allografts; 43 (67%) liver-free and 19 (33%) liver-contained with 7 (13%) retransplants. Hindgut reconstruction was adopted in 1993 and preservation of native spleen was introduced in 1999. Immunosuppression was tacrolimus-based with antilymphocyte recipient pretreatment in 41 (75%). RESULTS: Patient survival was 89% at 1 year and 69% at 5 years with respective graft survival of 87% and 56%. Retransplantation was successful in 86%. Adults experienced better patient (P = 0.23) and graft (P = 0.08) survival with lower incidence of post-transplant lymphoproliferative disorder (P = 0.09) and graft versus host disease (P = 0.002). Antilymphocyte pretreatment improved overall patient (P = 0.005) and graft (P = 0.069) survival. The initially restored nutritional autonomy was sustainable in 23 (70%) of 33 long-term survivors with improved quality of life. The remaining 10 recipients required reinstitution of HPN due to allograft enterectomy (n = 3) or gut dysfunction (n = 7). Disease recurrence was highly suspected in 4 (7%) recipients. CONCLUSIONS: GT is life-saving for patients with end-stage CIPO and HPN-associated complications. Long-term survival is achievable with better quality of life and low risk of disease recurrence.

Clinical and Histopathological Spectrum of Adult Gastrointestinal Inflammatory Neuropathy.

Gastrointestinal inflammatory neuropathy, namely, eosinophilic myenteric ganglioneuronitis (EMG) and lymphocytic ganglioneuronitis (LG), is a form of chronic intestinal pseudo-obstruction and results from the infiltration of the myenteric plexus by eosinophils and lymphocytes, respectively. The literature related to the clinicopathological features of adult inflammatory neuropathy is scarce. We aim to elucidate the clinical and histological details of 7 cases of inflammatory neuropathy (EMG, n = 4, and LG, n = 3) and compare the features of EMG and LG retrospectively. There was no difference between these two entities in terms of clinical, hematological, or biochemical parameters. Histologically, almost all cases (n = 6/7) showed accompanying elements of ganglion cell vacuolization, mesenchymopathy, and partial/complete desmosis in addition to the disease-defining pathology. Besides, all cases of EMG showed infiltration of the inner circular muscle of muscularis propria by eosinophils. Two cases of LG showed additional muscular pathology pertaining to the muscularis propria. Inflammatory infiltration of the myenteric plexus is pathognomonic for the diagnosis of gastrointestinal inflammatory neuropathy although additional features in the form of ganglion cell vacuolization, reduction in the number of ganglia, desmosis, mesenchymopathy, and inflammation of the muscularis propria (eosinophils in EMG) can be seen. The pathologists need proper awareness along with judicious use of special and immunostains for clinching the diagnosis.

Ultrasound-guided double central venous access for azygos vein via the ninth and tenth intercostal veins.

Some patients with intestinal failure, who are dependent on total parenteral nutrition for long periods, suffer from a lack of suitable conventional venous access points, including axillary, external jugular, internal jugular, subclavian, saphenous, and the brachio-cephalic and femoral veins, due to their occlusion. Furthermore, extensive central venous stenosis and/or thrombosis of the superior and inferior vena cava may preclude further catheterization, so uncommon routes must be used, which can be challenging. In such patients, the azygos vein via the intercostal vein is a viable candidate. Thoracotomy-assisted, thoracoscopy-assisted, and cut-down procedures are currently suggested such access. We found that ultrasound-guided percutaneous puncture method was a safe and minimally invasive approach and successfully placed two central venous lines in preparation for small bowel transplantation via two different intercostal veins (ninth and tenth). Although the lung was actually located just below the target veins, an ultrasound provided augmented and clear vision, which contributed to the safe performance of the procedure without the need for invasive surgical intervention, such as thoracotomy, thoracoscopy, or rib resection using the cut-down technique. Furthermore, constant positive-pressure ventilation during vein puncture under general anesthesia also helps avoid venous collapse. Despite carrying a slight risk of light injury to the lung, artery, and nerve along with the vein compared to other procedures, we believe that ultrasound-guided puncture under general anesthesia is feasible as a minimally invasive method.

Revascularization-first strategy in acute aortic dissection with mesenteric malperfusion.

BACKGROUND AND AIM: Mesenteric malperfusion is a complication with a higher risk of in-hospital mortality because diagnosing mesenteric ischemia before necrotic change is difficult, and when it occurs, the patient's condition has worsened. Although it contradicts the previous consensus on central repair-first strategy, the revascularization-first strategy was found to be significantly associated with lower mortality rates. This study aimed to present our revascularization-first strategy and the postoperative results for acute aortic dissection involving mesenteric malperfusion. METHODS: Among 58 patients with acute type A aortic dissection at our hospital between January 2017 and December 2019, mesenteric malperfusion was noted in six. Four hemodynamically stable patients underwent mesenteric revascularization with endovascular intervention in a hybrid operation room before central repair, and two hemodynamically unstable patients underwent central repair before mesenteric revascularization. RESULTS: No in-hospital mortality was recorded. All four patients with mesenteric revascularization-first strategy recovered with no symptoms related to mesenteric ischemia. Two patients with central repair-first strategy developed paralytic ileus for 1 week; one of them needed exploratory laparotomy, but no patients needed colon resection. CONCLUSION: No in-hospital mortality was recorded. All four patients with mesenteric revascularization-first strategy recovered with no symptoms related to mesenteric ischemia. Two patients with central repair-first strategy developed paralytic ileus for 1 week; one of them needed exploratory laparotomy, but no patients needed colon resection.

Endoscopic decompression of acute intestinal distension is associated with reduced mortality in critically ill patients.

BACKGROUND: Endoscopic placement of intestinal decompression tubes is a feasible technique for treatment of acute intestinal dilation. Given the heterogeneity of the underlying diseases leading to intestinal obstruction data on the significance of endoscopic procedures for treatment of these conditions are sparse. METHODS: In the study period from 2008 to 2019 all patients receiving a decompression tube were identified by retrospective chart review and analyzed. RESULTS: A total of 59 decompression tubes were placed in 50 patients. Technical success was achieved in 98% (58/59 tubes). As major complication one small bowel perforation occurred (1/59; 1.7%). Causes for impaired intestinal transit comprised tumor stenoses 22% (11/50), infections 18% (9/50), post-operative paralysis 14% (7/50), neurological diseases 8% (4/50), trauma 2% (1/50) and others 36% (18/50). Most patients (74%; 37/50) were critically ill and treated on intensive care unit. Treatment response after tube insertion was documented in 76% of patients (38/50) whereas 24% (12/50) did not fulfill response criteria. Patients with treatment response showed a significantly better outcome compared to non-responders. Responders had a median survival of 113 days (95% CI 41-186) compared to 15 days (95% CI 6-24) in non-responders (p = 0.002). Analysis of laboratory parameters after stratification in responders and non-responders to endoscopic therapy showed that non-responders had significantly higher levels of CRP and lower platelet count at baseline (CRP 262 mg/L (IQR 101-307) vs. 94 mg/L (IQR 26-153): p = 0.027; platelets 69 thsd/µL (IQR 33-161) vs. 199 thsd/µL (IQR 138-289): p = 0.009). CONCLUSIONS: Endoscopic decompression is a safe procedure for acute management of impaired intestinal transit even in critically ill patients. Response to therapy is associated with improved outcome and markers of inflammation and organ function such as CRP, platelet count and serum lactate have to be taken into account for therapy monitoring and evaluation of prognosis.

Use of pediatric donor en bloc kidneys along with bladder segment in pediatric liver-kidney and multivisceral-kidney transplantation.

The combination of pediatric multivisceral and kidney transplantation leads to additional recipient risks due to the number of anastomoses and to the small sizes of donor structures. The inclusion of donor kidneys, ureters, and a bladder patch en bloc with multivisceral organs decreases the number and complexity of anastomoses and has not yet been reported. Four patients were transplanted in this fashion; three underwent multivisceral-kidney and one underwent liver-kidney transplantation. The first patient was a 3-year-old male with polycystic kidney disease and congenital hepatic fibrosis. The second was a 7-year-old female with complications from necrotizing enterocolitis. The third was a 12-month-old male with megacystis microcolon intestinal hypoperistalsis syndrome and secondary hydronephrosis, and the fourth was a 3-year-old male with multiple intestinal resections secondary to incarcerated hernia. The third patient developed a right ureteral stenosis with an intact bladder patch. The fourth child expired from maintained abdominal sepsis. The first 3 patients maintained normal graft function. There were no cases of thrombosis, arterial stenosis, or urinary leakages. These reported cases demonstrate that small pediatric en bloc transplantation of the multivisceral organs and dual kidneys with a bladder patch anastomosis is a feasible and less complex alternative to the standard procedure.