Non traumatic right Bochdalek hernia with loss of Domain in adults. Our experience and review of literature.

INTRODUCTION: Right-sided Bochdalek hernia (BH) is a rare developmental defect in the posterolateral diaphragm, allowing herniation of abdominal contents into the thorax. To date only 44 reported cases of right-sided BH have been surgically managed in adults in literature. We report one additional case of right-sided BH with loss of Domain. "Loss of domain" (LOD) is a term used commonly in the hernia literature to describe the distribution of abdominal content between the hernia and residual abdominopelvic cavity. After repairing hernias with significant LOD, serious physiological complications can arise. METHODS: PubMed and Cochrane bibliographical databases were searched (last search: February 2022) for studies concerning BH. CASE PRESENTATION: We report the case of a 50-year-old woman whose right-sided diaphragmatic hernia strangulated loops of small bowel and who was thus treated via urgent laparoscopy. After reduction of the intrathoracic contents we were unable to primarily close the midline fascia.We performed a staged abdominal wall reconstruction as the chronicity of the hernia led to loss of intra-abdominal domain. DISCUSSION: Bochdalek hernia (BH) is the most common type of congenital diaphragmatic hernia and is usually leftsided. It typically presents in neonates and diagnosis in adults is a rarity. Various surgical repair options include open surgery, laparoscopic repair, thoracoscopic approach and robotic transthoracic approaches. CONCLUSION: BH should be managed timely regardless of its symptoms to avoid future complications. The closure of the defect can be done by different methods. When, after diaphragmatic hernia repair, it is suspected that the herniated viscera have lost their domain, it is preferable to use a Temporary Abdominal Closure to prevent compartment syndrome. KEY WORDS: Bochdalek hernia with loss of Domain, Bochdalek hernia in adults, Non traumatic Bochdalek hernia, Right-sided diaphragmatic hernia.

Adenocarcinoma of the sigmoid colon causing sigmoido-rectal intussusception: A rare entity in adults.

INTRODUCTION: Intussusception occurs when a more proximal portion of the bowel (intussusceptum) invaginates into the more distal bowel (intussuscipiens). The pathomechanism is thought to involve altered bowel peristalsis at the intraluminal lesion, which is then a lead point for the intussusceptum. Intestinal intussusception is rare in adults, accounting for approximately 1 % of all bowel obstructions. We report a unique case in which a partially obstructing sigmoid cancer caused full thickness rectal prolapse requiring surgical intervention. PRESENTATION OF CASE: A 75-year-old male presented in the emergency department due to anal haemorrhage for 5 days. On clinical examination his abdomen was distended with signs of peritoneal irritation in the right quadrants. The CT scan showed sigmoid-rectal intussusception with an sigmoid colonic tumour. The patient underwent emergency anterior resection of the rectum without reduction of the intussusception. Histological examination revealed a sigmoid adenocarcinoma. DISCUSSION: Intussusception is the most common urgent situation among the pediatric population but its incidence in adults is very rare. The diagnosis is difficult to establish with history and physical exam findings alone. Since in adults, unlike children, in most cases a malignant pathology acts as a lead point, the treatment of this pathology still reserves doubts. Recognizing and understanding pertinent signs, symptoms, and imaging findings is essential to the early diagnosis and appropriate management of adult intussusception. CONCLUSION: The appropriate management of adult intussusception is not always clear cut. There is controversy about the reduction before resection in cases of sigmoidorectal intussusception.

Esophageal Retained Lithium Battery in Children Younger than 6 Years: A Prompt Structured Multidisciplinary Approach Is Essential to Reduce Long-Term Consequences.

OBJECTIVES: Disk battery esophageal retention in children younger than 6 years represents an increasing endoscopic emergency, followed by a relevant risk of life-threatening late complications. Surgical removal after a failed endoscopic approach is rarely reported in the literature. We describe our experience in this scenario. METHODS: Two female asymptomatic patients aged 26 and 29 months presented within 4 hours after a witnessed ingestion of a 2-cm, 3-V lithium battery (CR2032) retained in the cervical esophagus. Both patients underwent a prolonged unsuccessful emergent endoscopic removal with a flexible instrument performed by an adult gastroenterologist. Both batteries fused with the esophageal wall were extracted through a longitudinal left cervical esophagotomy combined with minimal resection of necrotic tissues and repaired over a 12F feeding tube. RESULTS: Patients were extubated after 12 and 72 hours, respectively. Contrast study was performed after 20 and 13 days, respectively, before resuming oral feeding. At endoscopy, the first patient developed a 3-cm-long severe esophageal stenosis (35th day), followed by an asymptomatic tracheoesophageal fistula (60th day), which was conservatively treated. After spontaneous resolution of the tracheoesophageal fistula, esophageal stenosis progressed, partially responsive to esophageal stenting. Short esophagectomy is under evaluation. The second patient developed an asymptomatic limited stenosis, not requiring dilatation. CONCLUSIONS: The emergent management of lithium battery ingestion needs a structured timely multidisciplinary approach in the emergency department, an experienced pediatric endoscopist, and a simultaneous engagement of pediatric surgical expertise, even in patients who do not show bleeding, to reduce esophageal exposure time to high-voltage current released by batteries, which represents the main factor conditioning tissue damage and prognosis.

Pitfalls and technical errors in the first approach to neonates with anorectal malformations in a non-specialist context: can we do any better? A review from three Eastern African Centres.

INTRODUCTION: In sub-Saharan Africa, Anorectal malformations (ARM) are the most frequent cause of neonatal obstruction. Referral to a Pediatric Surgeon is frequently delayed. The first treatment is often delivered at not specialist level and mismanagement may result. AIM: To study ARM patients referred beyond neonatal period and managed at a non-specialist level. MATERIALS AND METHODS: One hundred and thirty patients were included (M/F ratio 63/67) among 144 admitted to three Eastern African Hospitals with Pediatric Surgical facilities. Demographics, type of anomaly, delay on referral, previous management, most commonly observed errors are reported. RESULTS: The Mean age at referral was 23 months (range five weeks - 23 years). Colostomy was the most frequent surgery (92 cases). Stomas often did not follow the recommended criteria. Ten per cent were not on the sigmoid, and 35% were not divided. "Loop" or "double-barrel" colostomies did not exclude the distal loop. Inverted (10,5%), prolapsed stomas (7,5%), short distal loop (16%) were observed. Twenty-four cases (26%) needed redo. Primary perineal exploration in eight patients resulted in incontinence. CONCLUSIONS: Investments on training practitioners, acting at District/Rural level, and closer links with tertiary centres are recommended to avoid ARM mismanagement and delayed referral to a Specialist.

Challenges, constraints and failures that are related to the posterior sagittal anorectoplasty approach to anorectal malformations in a low-resource context: An experience from a sudanese tertiary referral centre.

BACKGROUND: Anorectal malformations (ARMs) in the sub-Saharan Africa are a common cause of neonatal referral for intestinal obstruction, and the posterior sagittal anorectoplasty (PSARP) approach is rapidly spreading. The small number of paediatric surgeons and the low-resource context limit children's access to care and constrain the quality of results. A retrospective, observational study has been done on a consecutive series of ARM cases admitted to a Sudanese tertiary paediatric surgical centre within the framework of a partnership between Italian and Sudanese academic institutions addressed to review and upgrade the standard of care of major congenital anomalies. MATERIALS AND METHODS: The authors collected 94 ARM cases in a 3 years' period. Conditions on referral, operative procedures, post-operative course and follow-up were recorded and examined. Their correlations with complications and outcome were analysed. RESULTS: The male/female ratio was 47/47. Eighty patients presented with an untreated ARM; 66 had a divided stoma and 14 had already a PSARP procedure, followed by a poor outcome or sequelae. In 25% of the cases, colostomy required re-doing. In 57 cases, a staged PSARP (primary or re-do) was done. Surgical-site infections occurred in nine patients. Some patients were lost to follow-up after preliminary colostomy. Post-operative dilatation programme suffered from the lack of systematic follow-up, and colostomy closure was possible in 46% of the cases due to problems in travelling and accessing hospital care. Anal stenosis was frequently observed among unfollowed patients. CONCLUSION: Despite PSARP's widespread adoption in Africa, the risk of complications and failures is high. Primary management is often inappropriate, and a high rate of colostomy-related complications is observed. Poverty and lack of transportation reduce attendance to follow-up, hampering the final results. Investments in healthcare facilities and retention of trained health providers are needed to improve the standard of care.

Isolated fallopian tube torsion in pediatric age: An Italian multicenter retrospective study.

PURPOSE: The purpose of this study was to quantify the experience of five Italian centers on the diagnosis and management of isolated fallopian tube torsion (IFTT) in children. METHODS: We retrospectively reviewed the data of 20 patients aged 1-16 years of age with surgically diagnosed IFTT between 1991 and 2017 from five Italian centers of pediatric surgery. We analyzed common presenting signs, symptoms, and radiographic findings, as well as surgical interventions to describe management offering further insight into the diagnosis and treatment of this rare entity. RESULTS: Twenty cases of IFTT were collected. Median age was 13.1 years. Menarche was present in 14 cases. A clinical history of abdominal pain was present in 13 patients, whereas in 7 patients the clinical picture was an acute abdomen. Ultrasonography was the first diagnostic examination in 16 cases. Surgical approach was by laparoscopy in 16 cases and laparotomy in 4 cases. In 11 patients IFTT was associated with another pathologic condition. In 7 of the remaining 9 IFTT without pathologic association, the girls played sports. Salpingectomy was performed in 13 patients and de-torsion in 7. CONCLUSIONS: IFTT is a rare condition that seems to occur in younger adolescents. Vague clinical presentation contributes to low preoperative suspicion. IFTT should be considered in girls with abdominal pain who practice sports with sudden body movements. Preoperative suspicion may be increased based on radiographic findings of an enlarged tubular/cystic structure with adjacent normal ovary. Conservative management is controversial but could be preferred in order to provide the best option for future fertility of these girls. TYPE OF STUDY: Treatment Study (Retrospective Study) - Level IV.

Intestinal malrotation in infants with omphalocele: A systematic review and meta-analysis.

PURPOSE: The management of intestinal malrotation in infants with omphalocele varies among surgeons. Herein, we aimed to determine whether infants with omphalocele should be investigated for malrotation. METHODS: Using a defined search strategy, three investigators identified all studies reporting patients with omphalocele and malrotation. Outcome measures included: 1. incidence of malrotation; 2. correlation with the abdominal size defect in patients with omphalocele; 3. risk of volvulus in those not investigated for malrotation; 4. incidence of adhesive bowel obstruction in those who underwent Ladd's procedure. The meta-analysis was conducted according to PRISMA guidelines and using RevMan 5.3. RESULTS: Of 111 articles analyzed, 12 (3888 children) reported malrotation in 136 patients (3.5%). Malrotation was equally found in patients with major (15.2%) and minor (13.6%; p = 0.52) omphalocele. A volvulus was more common in children who had Ladd's procedure (8%) than in those who did not (1%; p = 0.03). Adhesive bowel obstruction rate was similar in both groups (5% vs. 3%; p = 0.21). CONCLUSION: The incidence of malrotation in infants with omphalocele is low but probably underreported, and is not influenced by the size of the defect. At present, there is no evidence in the literature to support investigations to detect malrotation in infants with omphalocele. TYPE OF STUDY: Therapeutic. LEVEL OF EVIDENCE: III.

Unilateral Papillary Cystadenoma of the Epididymis as a First Presentation of Von Hippel-Lindau Disease.

Association between papillary cystadenoma of the epididymis (PCE) and Von Hippel-Lindau Disease (VHLD) is well known and stronger for bilateral tumors. Unilateral PCE occurs either as a sporadic tumor without evidence of VHLD or in the context of a known diagnosis of VHLD, indeed it has never been reported as the first manifestation of VHLD. In contrast, we report the case of a boy with an apparently isolated, unilateral PCE that resulted to be the first manifestation of an unknown VHLD. Thus, we recommend screening for VHLD in patients with a new diagnosis of unilateral PCE, especially if the patients are young.

Atropine Treatment for Hypertrophic Pyloric Stenosis: A Systematic Review and Meta-Analysis.

INTRODUCTION: Several authors have reported the use of atropine as an alternative treatment to pyloromyotomy in infants with hypertrophic pyloric stenosis (HPS). Our aims were to review the efficacy of atropine in treating HPS and to compare atropine therapy versus pyloromyotomy. MATERIALS AND METHODS: Using a defined search strategy (PubMed, MEDLINE, OVID, Embase, Cochrane databases), two investigators independently identified studies reporting the use of atropine for HPS. Case reports and opinion articles were excluded. Outcome measures included success rate, side effects, and length of hospital stay. Maneuvers were compared using Fisher's exact test, and meta-analysis was conducted using RevMan 5.3. Data are expressed as mean ± standard deviation. RESULTS: Systematic review: of 2,524 abstracts screened, 51 full-text articles were analyzed. There were no prospective or randomized studies. Twelve articles (508 infants) reported HPS resolution using atropine in 402 (79.1%) patients. Atropine side effects were documented in 38/251 (15.1%) infants and included tachycardia, increased transaminases, and flushed skin. Meta-analysis: five studies compared atropine treatment (293 infants) with pyloromyotomy (537 infants). Pyloromyotomy had higher success rate (100%) than atropine (80.8%; p < 0.01) and shorter hospital stay (5.6 ± 2.3 vs. 10.3 ± 3.8 days, respectively; p < 0.0001). CONCLUSION: Comparative but nonrandomized studies indicate that atropine is less effective than pyloromyotomy to treat infants with HPS. Currently, there is no evidence-based literature to support atropine treatment in these infants. To our knowledge, atropine should be reserved for patients unfit for general anesthesia or surgery.

Long-Term Follow-Up of Testicular Microlithiasis in Children and Adolescents: Multicenter Prospective Cohort Study of the Italian Society of Pediatric Urology.

Introduction Testicular microlithiasis (TM), characterized by the presence of intratubular calcifications in a single or both the gonads, is an uncommon entity with unknown etiology and outcome in pediatric and adolescent age. In this study, the results of a multicenter long-term survey are presented. Materials and Methods From 11 units of pediatric urology/surgery, patients with TM were identified and yearly, followed up in a 7-year period, adopting a specific database. The recorded items were: age at diagnosis, presenting symptoms/associated abnormalities, ultrasonographic finding, surgery and histology at biopsy, if performed. Results Out of 85 patients, 81 were evaluated yearly (4 patients lost to follow-up). TM was bilateral in 66.6% of the patients. Associate genital abnormalities were present in 90%, more frequently undescended/retractile testis (23.4%) and varicocele (22.2%). TM remained unchanged at 4.7 years follow-up in 77 patients (93.8%) and was reduced in 4 patients after 1 to 5 years of inguinoscrotal surgery. Orchiectomy was performed in three patients (3.7%), one for severe testicular hypoplasia and two for seminoma (2.5%), respectively, concurrent and metachronous to diagnosis of TM. Tumorectomy with parenchymal sparing surgery was performed in a teratoma associated with TM. Conclusion TM is a controversial entity, often associated with several inguinogenital features, which rarely can recover. Testicular malignancy, although present in TM, has not proven definitively associated to microliths. Proper counseling, yearly ultrasound, and self-examination are long-term recommended.

Incidence, prevention, and treatment of parenteral nutrition-associated cholestasis and intestinal failure-associated liver disease in infants and children: a systematic review.

BACKGROUND: Cholestasis is a significant life-threatening complication in children on parenteral nutrition (PN). Strategies to prevent/treat PN-associated cholestasis (PNAC) and intestinal failure-associated liver disease (IFALD) have reached moderate success with little supporting evidence. Aims of this systematic review were (1) to determine the incidence of PNAC/IFALD in children receiving PN for ≥ 14 days and (2) to review the efficacy of measures to prevent/treat PNAC/IFALD. METHODS: Of 4696 abstracts screened, 406 relevant articles were reviewed, and studies on children with PN ≥ 14 days and cholestasis (conjugated bilirubin ≥ 2 mg/dL) were included. Analyzed parameters were (1) PNAC/IFALD incidence by decade and by PN length and (2) PNAC/IFALD prevention and treatment (prospective studies). RESULTS: Twenty-three articles (3280 patients) showed an incidence of 28.2% and 49.8% of PNAC and IFALD, respectively, with no evident alteration over the last decades. The incidence of PNAC was directly proportional to the length of PN (from 15.7% for PN ≤ 1 month up to 60.9% for PN ≥ 2 months; P < .0001). Ten studies on PNAC met inclusion criteria. High or intermediate-dose of oral erythromycin and aminoacid-free PN with enteral whey protein gained significant benefits in preterm neonates (P < .05, P = .003, and P < .001, respectively). None of the studies reviewed met inclusion criteria for treatment. CONCLUSIONS: The incidence of PNAC/IFALD in children has no obvious decrease over time. PNAC is directly correlated to the length of PN. Erythromycin and aminoacid-free PN with enteral whey protein have shown to prevent PNAC in preterm neonates. There is a lack of high-quality prospective studies, especially on IFALD.

Long-term followup of primary nonrefluxing megaureter.

PURPOSE: We evaluated outcomes of nonoperative management of primary nonrefluxing megaureter at long-term followup to identify clinical predictors of spontaneous resolution. MATERIALS AND METHODS: A total of 75 patients (88 primary megaureters) were diagnosed between 1990 and 2005 and followed for more than 6 months. Of the patients 63 (74 primary megaureters) were included in the main study population. Indications for surgery were obstructive hydroureteronephrosis, functional impairment and persistent symptoms. RESULTS: Of the 74 primary megaureters 20 (27%) required surgery up to 7 years after diagnosis. Surgery was not indicated in 82% of primary megaureters with grade I or II hydronephrosis vs 62.9% of those with grade III or higher hydronephrosis (difference not significant), nor in 76.5% of types I and II primary megaureters vs 33.3% of type III primary megaureters (p = 0.040), 78.7% of renal units with differential function 40% or greater vs 0% with differential function less than 40% (p = 0.027), 80% of primary megaureters with a nonobstructive washout pattern vs 44.4% with an intermediate/obstructive pattern (p = 0.032), 67.9% of patients with perinatal presentation vs 25% with postneonatal presentation (p = 0.008) or 63.2% of patients presenting with symptoms vs 76.4% of those who were asymptomatic (difference not significant). On multivariate analysis age at presentation and washout pattern were significant predictors of spontaneous resolution. CONCLUSIONS: Most cases of primary megaureter resolve spontaneously or improve without loss of function or development of symptoms. Careful observation allows surgery to be delayed beyond the neonatal period in most patients. Long-term followup is recommended because symptoms can develop years later. Washout pattern and age at presentation are statistically significant predictors of spontaneous resolution.

Intercostal muscle flap to protect the bronchial stump in pediatric lobectomy for lung abscess.

Lung suppurative diseases in children are usually responsive to medical treatment or percutaneous drainage. Rarely, pulmonary resection is required for lung abscess in childhood, particularly in presence of co-morbidities. In these cases, a lobectomy is usually performed through an open thoracotomy, with a reported incidence of bronco-pleural fistula up to 9.1% of pediatric series. This consequence is mainly due to the inflammatory condition; however the lack of knowledge of pediatric and thoracic surgeons with this rare condition in childhood can also play a role. In adults with lung cancer, the buttressing of bronchial stump with the additional support of an intercostal muscle (ICM) flap has proved to prevent this complication, as well as to reduce post-operative pain. We report the first pediatric experience of ICM flap used in 2 immunocompetent children requiring lobectomy for suppurative lung conditions. Our preliminary experience confirms the feasibility of protecting the bronchial stump after lobectomy in children, especially in conditions at risk for bronco-pleural fistula development.

Giant abdomino-pelvic adipose tumors of childhood.

Adipose tumors are rare in pediatric age and are commonly represented by benign forms: lipoma and lipoblastoma/lipoblastomatosis. Generally localized in subcutaneous tissue of the trunk and extremities, they can exceptionally occur as giant intra-peritoneal or pelvic masses. These tumors could reach considerable size prior to diagnosis since they are asymptomatic. The authors report a case series of three giant abdomino-pelvic adipose tumors.

Transurethral puncture for ureterocele-which factors dictate outcomes?

PURPOSE: We evaluated which clinical factors influence the outcome of primary transurethral puncture for ureterocele. MATERIALS AND METHODS: A total of 45 patients (47 ureteroceles) underwent primary transurethral incision between 1994 and 2008 at 2 institutions. Age at and mode of presentation, upper tract status, ureterocele site, preoperative vesicoureteral reflux and the corresponding upper pole or kidney function were analyzed to identify which factors influenced the need for secondary surgery. RESULTS: Transurethral puncture was the only treatment in 24 of 45 patients (53%) while 21 (47%) required further surgery. After transurethral puncture secondary surgery was required in 56% of patients who presented prenatally vs 27% of those who presented postnatally (p = 0.165), in 18% with a single system vs 58% with a duplex system (p = 0.036), in 30% with intravesical vs 63% with ectopic ureterocele (p = 0.039) and in 61% vs 37% with ureterocele units with vs without preoperative vesicoureteral reflux (p = 0.148). Fisher's 2-tailed exact test revealed an inconsistent distribution of negative prognostic factors, including duplex systems, ectopic ureterocele and vesicoureteral reflux at presentation, in prenatally vs postnatally and in asymptomatically vs symptomatically presenting subgroups. CONCLUSIONS: Upper tract status and ureterocele site influence the outcome of primary transurethral puncture as a definitive procedure. After puncture secondary surgery is least likely in patients with a single system and intravesical ureterocele.

Bilateral scrotal abscesses caused by Klebsiella pneumoniae in a newborn.

The management of acute scrotal swelling can be challenging in neonatal age, with scrotal infections being great mimickers of testicular torsion. Only a few unilateral cases of scrotal abscess have been previously reported, mostly caused by Staphylococcus and Salmonella. We describe the case of a newborn who developed bilateral scrotal abscesses caused by Klebsiella pneumoniae and discuss the rarity of the case, regarding both the bilaterality and the pathogen, never reported before.

Pancreatic tumors in children and adolescents: the Italian TREP project experience.

INTRODUCTION: Malignant pancreatic tumors are exceedingly rare in pediatric age and their clinical features and treatment usually go unappreciated by most pediatric oncologists and surgeons. METHODS: From January 2000 to July 2009, 21 patients <18 years old with pancreatic tumors were prospectively registered in the Italian cooperative TREP project dedicated to very rare pediatric tumors. RESULTS: Tumor types were 4 pancreatoblastomas, 2 pancreatic carcinomas, 3 neoplasms of the endocrine pancreas, and 12 solid pseudopapillary tumors. Three of the four patients with pancreatoblastoma had advanced disease at diagnosis and were given chemotherapy; at the time of this report, three patients were alive in first remission, while one died due to treatment toxicity. Both the cases of pancreatic carcinoma had the acinar cell subtype and successfully underwent pancreaticoduodenectomy with complete tumor resection, remaining without evidence of disease at the time of this analysis. The histological diagnoses of the three endocrine tumors were a malignant islet cell tumor, a gastrinoma, and a well-differentiated tumor. All 12 patients with solid pseudopapillary tumors underwent complete tumor resection and were given no adjuvant treatment; 11 were alive in first remission, while one experienced a local and distant relapse 5 years after diagnosis. CONCLUSIONS: Surgery remains the keystone of treatment for pancreatic tumors in pediatric age as in adults. The TREP project shows that prospective cooperative studies are feasible even for such very rare tumors as these and may serve as a model for developing international cooperative schemes.