Pediatric Rectosigmoid Atypical Juvenile Polyps Presenting With Rectal Prolapse and Acute Bleeding: A Case Report and a Comprehensive Literature Review.

Rectosigmoid solitary juvenile polyps are benign lesions, relatively frequent in childhood. The clinical debut of a pediatric polyp with bleeding is relatively frequent, but there are very few reports of rectal prolapse of polyps. We present the case of a 7-year-old female patient with no previous history who presented with rectal prolapse of a polyp with acute bleeding. An urgent endoscopic examination was performed and 2 rectosigmoid polypoid lesions were found and resected. The anatomopathological study showed that these were 2 hamartomatous polyps with mild dysplasia. The patient is asymptomatic and is being followed up. The literature concerning rectal prolapse of polyps in the pediatric population is scarce. In a pediatric patient with a rectal prolapse, this entity should be considered in the differential diagnosis.

Congenital Meatal Urethral Stenosis in a Female Patient: A Case Report.

We present the case of a 6-year-old girl who presented with alterations in the voiding stream. On physical examination, a very small urethral meatus was identified at the expense of a membrane. The renovesical ultrasound showed no alterations. An uroflowmetric study was performed, showing a bladder outlet obstruction pattern. The urethral meatus was calibrated and a ventral meatotomy was performed. The histological study of the resected membrane showed a transitional urethral mucosa with chronic focal inflammation and discrete hyperplasia. The patient evolved favorably, with resolution of the symptoms and no notable complications. This is, to the best of our knowledge, the first reported case with a histological study of a congenital meatal urethral stenosis. In the presence of lower urinary tract obstruction, this entity should be considered in the differential diagnosis. Surgical treatment is curative.

Pediatric Vulvar Superficial Angiomyxoma: A Case Report With Clinical, Radiological, and Anatomopathological Characterization and a Comprehensive Review of the Literature.

Superficial angiomyxoma is characterized as a benign, slow-growing vascular cutaneous myxoma. A 6-year-old Arab girl with no medical history presented with a vulvar tumor located on the left labia majora. The lesion was present since birth, but it had significantly increased over the last 6 months. She did not have any associated symptoms. Physical examination revealed an exophytic tumor of the left labia majora, which measured 5 cm in its major axis. Doppler ultrasound study showed a mass with abundant arterial and venous vascularization, and magnetic resonance imaging showed a highly vascular contrast-enhanced mass with well-delimited margins, which depended on the labia majora. A macroscopically complete resection was performed, achieving a tension-free primary closure. Histologically, the lesion was characterized as a well-demarcated superficial tumor with thin-walled vessels and myxoid stroma, S100 (-), CD34 (+), vimentin (+), and actin (+). The final histopathological diagnosis was superficial angiomyxoma. The literature review of this entity in the pediatric population shows a predominance of this lesion in the vulvar location. Local recurrence has been described. Loss of PRKAR1A expression may be involved in the pathogenesis of superficial angiomyxoma.

Congenital Prepubic Sinus as a Variant of Incomplete Urethral Dorsal Duplication: A Case Report with New Insights into its Immunohistochemical Characterization and a Comprehensive Literature Review.

Congenital prepubic sinus (PS) is an extremely infrequent malformation consisting of a prepubic fistulous tract that classically does not communicate with the genitourinary system. Previous studies centered on its immunohistochemical characterization have shown inconsistent results, and the etiology has not been clarified. We present the case of a 2-year-old male who presented since birth with a fistulous orifice on the dorsum of the penis. He had no associated symptoms. Under general anesthesia, the fistulous tract was explored, and methylene blue was instilled through it. After cystoscopically verifying the absence of communication with the urethra, a complete resection of the lesion was performed. The immunohistochemical study showed positivity for low and high molecular weight keratins and a transitional pattern for keratin 7 and GATA3, with positivity at cul de sac level and negativity at proximal level. These findings suggest that this lesion is an incomplete dorsal duplication variant.

Development and clinical application of a new sternal zenithal traction system in video-assisted percutaneous thoracoplasty.

INTRODUCTION: Video-assisted percutaneous thoracoplasty involves a complex surgical access with risk of damaging vital structures during the procedure. Historically, different traction and sternal elevation systems have been applied during the intervention to minimize the risk associated with the passage of the instruments between the sternum and the pericardium. MATERIAL AND METHODS: A new sternal traction system is presented by means of an illustrated description. Clinical and sociodemographic data were extracted from the 36 patients operated in our center for Pectus Excavatum between July 2017 and August 2021. The Haller index was not applied as a criterion to determine the use or not of the sternal traction system. Patients were classified according to whether the sternal traction system (TE) or not (ST) had been employed. Statistical analysis of the data collected was performed with STATA, version 15.0 (StataCorp). RESULTS: Both groups were comparable. Haller's Index was 4.19 ± 0.7 for the sternal traction (TE) group and 3.79 ± 0.3 for the no sternal traction (ST) group. In 25 of the patients the described traction system was used, and in 11 no traction system was used. All patients were operated on by the same surgeon. The mean operative time in minutes was 97.73 ± 46.2 for the ST group and 88.13 ± 18.1 for the TE group (p = 0.87). The mean total days of admission was 7.67 ± 0.82 (TE) and 7.73 ± 1.35 (ST). Mean days of intravenous PCA was 6.08 ± 0.72 (TE) and 5.89 ± 1.45 (ST). The mean number of epidural PCA days was 3.79 ± 0.5 (TE) and 3.36 ± 0.5 (ST) (p = 0.01). All patients presented a favorable postoperative evolution. None of the patients presented discomfort at the level of the sternal wounds during hospitalization. Cosmetic evolution was favorable in all patients. CONCLUSIONS: The sternal traction system presented provides safety when performing retrosternal dissection and placement of the thoracoplasty bar, leading to a decrease in surgical time in cases with a Haller Index higher than 3.5. There have been no complications associated with its use, nor has there been an increase in the number of days of hospitalization or analgesic requirements. There are no aesthetic sequelae for the patients.

[Solitary dorsal paravertebral tumor: radiological and histopathological characterization of a pediatric case of nodular fasciitis].

Nodular fasciitis is a benign soft tissue lesion with rapid fibroblastic or myofibroblastic proliferation, rarely observed in pediatric patients. Here, we present the case of a seven-year-old boy with no relevant medical records, in whom an asymptomatic dorsal tumor was incidentally identified. Magnetic resonance imaging showed a left dorsal paravertebral lesion with hypointensity on T1, hyperintensity on T2, peripheral contrast enhancement, and the so-called fascial tail sign. Complete surgical resection of the lesion was achieved. The histopathological study showed a proliferation of spindle or stellate cells with nuclei without atypia in a myxoid or collagenized stroma. The immunohistochemical profile showed positivity for smooth muscle actin, muscle-specific actin antibody HHF35, and calponin. The lesion was diagnosed as nodular fasciitis, an entity with broad and complex differential diagnosis. Presence of specific radiological signs and adequate immunohistochemical characterization of the lesion help perform an accurate diagnosis.

[Urachal remnant and acute abdomen: when it's not what it seems].

Among the causes of acute surgical abdomen, infection of a urachal remnant may go unnoticed despite routine complementary studies. We present three cases in boys aged 11, 6, and 4 years who were brought to the emergency department for right iliac fossa pain, fever, and urinary symptoms. Examination and complementary tests results were compatible with acute complicated appendicitis and were sent to surgery. While in the operating room, appendicitis was excluded and inflammatory changes suggestive of infection of the urachal remnant were documented. Complications of urachal remnants should be considered in the differential diagnosis of acute surgical abdomen, since advanced infection of the urachus may be a cause of confusion. A more accurate presumptive diagnosis can change the therapeutic/surgical approach and follow-up.

Crossed Testicular Ectopia: Report of Two Cases in Children of Consanguineous Parents.

Crossed testicular ectopia (CTE) is an extremely rare anomaly of urogenital development. The etiopathogenic mechanism is unknown. Medical records of two biological siblings with a confirmed diagnosis of CTE being managed at our center were collected. The first patient was born with a non-palpable left testis. An exploratory laparoscopy was performed and a CTE was found. The second patient had an incarcerated left inguinal hernia and a mesopenile hypospadias. During surgery a CTE was observed. The parents were consanguineous. This is the first reported case of CTE in siblings.

Transumbilical laparoscopic-assisted appendectomy in children: is it worth it?

INTRODUCTION: Transumbilical laparoscopic-assisted appendectomy (TULAA) is the technique of choice for all types of appendicitis in our Department. It combines the advantages of laparoscopy (global vision and minimally invasion) and open surgery (lower cost). The objective was to assess the results of our TULAA series and compare them to the results of standard laparoscopic appendectomies (SLA) performed during the same period. METHODS: Retrospective review of total appendectomies performed since TULAA introduction (September 2003 to December 2015) with statistic analysis of the results. RESULTS: A total of 1309 patients underwent TULAA approach, but 126 (9.6%) needed reconversion to open appendectomy, 1 (0.08%) to SLA, and 9 (0.7%) introduction of a second port. Mean age and weight of patients was 121.5 ± 36 months and 37.6 ± 14 kg, respectively. Mean operative time was 40.9 ± 15.5 min, ranging from 11 to 110. All types of appendicitis were present, with 394 being complicated (29.9%). Postoperative complications were seen in 168 patients (14.3%), 37 being readmitted (3.2%), and only five needing reintervention (Two intestinal occlusions and three abscess debridement). When comparing TULAA and SLA, there were no significant differences in the length of hospitalization, time to tolerate soft diet, analgesic requirements, and complications depending on the type of appendicitis, but TULAA was significantly faster and cheaper (average 900€). CONCLUSIONS: In our hands, TULAA has shown to be effective, easy to learn, and fast to perform. Low surgical cost is probably its principal advantage, which might be encouraging in times of crisis.

[Placement of pH-monitoring probes using height-related formulas. Is it an applicable method to adults?]. / Colocación de sondas de pHmetría mediante fórmula relacionada con la talla. ¿Es un método aplicable a los adultos?

INTRODUCTION: Oesophageal pH-monitoring allows the quantification of gastric reflux episodes in patients with gastroesophageal reflux disease (GERD). The accuracy of the test depends on correct positioning of the pH sensor 5 cm above the lower oesophageal sphincter (LES). The most precise manner to locate the LES is through prior determination by oesophageal manometry. However, because this technique is uncomfortable, mathematical formulas tend to be used in children. OBJECTIVES: To evaluate the applicability of paediatric formulas to estimate oesophageal length in adults and their effect on diagnostic accuracy. MATERIAL AND METHODS: A prospective study was carried out in adult patients, in whom the distance between the nasal orifice and the LES was determined by manometry and was compared with the estimated height-related distance calculated by four paediatric formulas (numbered 1 to 4). We also evaluated the relationship between the position of the probe and the percentage of reflux detected in our series of impedance measurements. RESULTS: Formula 1 (9.31 + height in cm × 0.197) was the most accurate (comparison of means -0.38 with 95%CI -0.70/-0.06, P = .019). With this formula, none of the patients had estimation errors of ± 6 cm. With formulas 2, 3 and 4, the percentage of error was 4.4%, 1.5% and 32.0%, respectively. CONCLUSION: Oesophageal length estimation in adults by using formula 1 is acceptable and can be used in adult patients who refuse to undergo prior manometry.

Bladder neck closure in children: long-term results and consequences.

AIM: Only few studies have gathered information on the long-term outcomes of children undergoing bladder neck closure (BNC). In the present study, we analyze the long-term results and consequences in this population. PATIENTS AND METHODS: The medical records of 20 patients (12 males and 8 females), who underwent BNC after several failed procedures to improve urinary incontinence were revised. Exstrophy complex was the underlying diagnose in all the cases: bladder exstrophy in 15 and cloacal exstrophy in 5. The median age of the patients at the time of surgery was 11.5 years (range 4-19 years). Previous surgeries were bladder neck reconstruction in 14 patients, bladder neck injection in 4 patients, and previous BNC in 1 patient. Overall 17 patients had concomitant bladder augmentation. The catheterizable stoma was made with appendix in 14, bowel in 3, and ureter in 3. The nine male patients aged>18 years at the time of this study received a questionnaire with specific questions on erections, orgasm, and details of ejaculation and were also asked to provide a semen sample. RESULTS: The median follow-up was 10 years (range 2-17 years) and median patient age was 21 years (range 13-32 years). Urinary continence was achieved initially in 16 patients (80%); 4 had a bladder neck fistula, 3 of them underwent surgical revision and achieved dryness. Long-term complications were: bladder stones (eight patients); stomal problems (four stenosis and three leaks); bladder perforation (two patients), and orchitis (one patient). All patients above the age of 18 years reported erections and orgasms although two had medium erectile dysfunction. The ejaculate volume was reported as normal in five, scarce in three, and absent in one, with slow ejection in seven. Only four supplied semen samples and only two showed normal values. CONCLUSIONS: BNC is an effective approach to incontinence when other procedures have failed. In the long term, the most frequent complications are those related with catheterizable stoma and stones. The high incidence report of a low fertility index and erectile dysfunction meant further study in a larger cohort.

Failed Nissen fundoplication in children: causes and management.

INTRODUCTION: Nissen fundoplication (NF) is the gold standard procedure for the treatment of gastroesophageal reflux (GER) in children. However, it fails in a significant proportion of patients. The purpose of this study was to identify preoperative predictors of failure and to examine the results of reoperations. PATIENTS AND METHODS: The charts of patients who underwent NF at our institution between 1992 and 2011 were retrospectively reviewed. Surgery was indicated in patients with symptomatic GER in whom medical treatment failed, particularly, in cases of esophageal atresia (EA), congenital diaphragmatic hernia (CDH), and neurologic impairment (NI). Chi-square comparisons and logistic regression were used to test comorbidities, previous abdominal surgery, surgical technique, gastrostomy, pyloromyotomy or pyloroplasty, age, weight, and surgical complications as possible predictors of NF failure. RESULTS: A total of 360 children (217 male and 143 female) underwent NF. Comorbidities were NI (n = 100, 27.8%), EA (n = 50, 13.9%), CDH (n = 22, 6.1%), and abdominal wall defects (AWD) (n = 6, 1.7%). A total of 35 patients (9.7%) had esophageal stenosis. Age at surgery was 3.06 years (0.04-20.7 years) and weight was 12 kg (2-77 kg). NF was open in 196 patients (54.4%) and laparoscopic in 164 patients (45.6%) (with 9 conversions). Follow-up was 6.7 years (0.01-18.7 years). A total of 42 patients (11, 7%) had postoperative complications (10 wound infection, 9 dumping syndrome, 8 gastrostomy related complications, 7 intestinal obstruction, 5 evisceration, 2 chylothorax, and 1 pneumothorax). Reflux recurred in 42 patients (11.7%) and 35 patients (9.7%) underwent redo NF 1.01 years (0.02-8.4 years) after the initial surgery. A total of nine patients (2.5%) required further interventions (five another redo NF, three esophageal replacements, and one esophago-gastric disconnection). A total of 29 patients (8.1%) died during the follow-up (25 because of their baseline disease, 3 in the postoperative period, and 1 because of pulmonary aspiration 3 years after surgery). EA (31.6% failure) and CDH (46.7% failure) were the only comorbidities predictive of NF failure (p < 0.05). CONCLUSIONS: Failure of NF is particularly frequent in patients previously operated upon for EA or CDH and can be predicted preoperatively. However, the benefits of the operation may outweigh this risk. Redo NF is indicated if symptoms of GER recur, but the proportion of failure is even higher. In subsequent failures, other options like esophageal replacement or esophagogastric dissociation should also be considered.

Pyloroduodenal duplication cysts: treatment of 11 cases.

INTRODUCTION: Abdominal enteric duplications are found in 1 out of 4,500 autopsies, and only 4 to 5% of them are located in the duodenum, where they may be connected with the biliary or pancreatic ducts. The aim of this study was to describe the clinical features, management, and outcome of a large series of duodenal duplication cysts. MATERIALS AND METHODS: The charts of all patients treated at our institution between 1985 and 2011 were reviewed retrospectively with particular attention to imaging, surgical technique, pathology, and outcome. RESULTS: During that period, 11 cases (81.8% females) were treated. Out of the 11 patients, 8 were symptomatic (vomiting in 3, recurrent acute pancreatitis in 2, and abdominal pain in 3) and 3 were tentatively diagnosed prenatally as choledochal cysts. Median age at surgery was 2.3 years (0 to 13.7) and preoperative diagnosis was correct in five cases. Five cysts were developed intraluminally and three communicated with the biliary duct (one), pancreatic duct (one), or both (one). Surgical treatment consisted of complete resection (four cases, including one pancreaticoduodenectomy), partial removal including all mucosa (four cases), and internal marsupialization (three cases). In all cases, the ductal communications were divided and opened into the duodenal lumen. In six cases, ectopic gastric mucosa was found. All patients recovered uneventfully. CONCLUSION: Duodenal duplication cysts are rare and may have bizarre anatomical patterns due to biliopancreatic involvement. Optimal treatment is complete surgical removal, and, if this is not possible, partial removal including the mucosa or marsupialization are also good alternatives. In cases with biliary and pancreatic tract connections, these have to be taken down carefully and drained into the duodenum.