Blast injuries involving the perineum are rare, particularly in the pediatric population, and may result in complex combined anorectal and genitourinary trauma. These injuries present major reconstructive challenges because of contamination risk, infection, and possible long-term functional impairment. Management has traditionally favored staged procedures with fecal diversion, especially in severe anorectal and rectovaginal injuries. We report here the case of a 10-year-old girl who sustained a severe blast injury resulting in a large perineal defect involving the anal canal, perineal body, and posterior vaginal wall. The patient underwent a successful single-stage reconstruction surgery consisting of posterior vaginal wall repair, perineal body reconstruction, and anterior anorectal wall repair without diverting colostomy. She tolerated the procedure well and remained hemodynamically stable throughout the perioperative course. At 8-week follow-up, the reconstructed tissues had completely healed, with preserved fecal continence and no pain, although residual scarring from the initial injury was noted. This case adds to the limited pediatric literature on severe perineal trauma and suggests that, in select hemodynamically stable patients with extraperitoneal injury and viable tissues, primary layered reconstruction without fecal diversion may be feasible.
Vesicovaginal fistula (VVF) is rare in children and is most commonly associated with retained vaginal foreign bodies. Surgical repair is technically demanding and carries a significant risk of recurrence, which has limited the application of minimally invasive approaches. We report the first pediatric case of robot-assisted laparoscopic transvesical repair of a VVF. A 14-year-old girl presented with longstanding urinary incontinence, malodorous vaginal discharge, and recurrent urinary tract infections, since early childhood. Initial imaging revealed a 30-mm bladder stone. Cystoscopy identified a large infratrigonal VVF associated with the stone, which had formed around a retained plastic ring. The foreign body and stone were removed through a suprapubic cystotomy. As spontaneous fistula closure did not occur after 1 year, definitive repair was undertaken. A robot-assisted laparoscopic transvesical approach was performed following cystoscopic identification of the ureteral orifices and placement of bilateral double-J stents. The fistula was closed in two watertight layers, with separate closure of the vaginal and bladder defects using absorbable sutures ( Video 1 ). No intraoperative or postoperative complications occurred. The patient was discharged on postoperative day 2. At 1-year follow-up, she remained asymptomatic, with complete fistula closure, normal urinary continence, and normal uroflowmetry findings. Robot-assisted transvesical repair provides excellent visualization and precise suturing within the confined pelvic space, representing a promising minimally invasive option for the management of complex pediatric VVF.
Congenital esophageal stenosis is a rare and heterogenous malformation often associated with esophageal atresia (EA). Failure of conservative endoscopic treatment leads to surgery. Transthoracic approach is rarely advocated due to the peridiaphragmatic localization of the stricture. Robotic-assisted thoracoscopic surgery (RATS) may offer enhanced precision in confined spaces; its application in patients under 10 kg is still considered a challenge. We report the first description of RATS resection of cartilaginous congenital stenosis and esophagoesophagostomy in a 9.5 kg patient. A female patient with a history of EA type III was corrected via videothoracoscopy, two cardiac surgeries via sternotomy, and laparoscopic treatment of duodenal atresia. During weaning, she did not tolerate thickened food, and endoscopy revealed a patent anastomosis but a distal esophageal stricture. A course of pneumatic dilation was attempted. Persistent clinical and radiological findings indicated the need for surgery. At 15 months, the patient underwent three-trocar thoracoscopy with the da Vinci Xi system. Esophagoscopy helped the identification of the stricture. A longitudinal incision exposed a 2-cm segment of thickened esophageal wall requiring complete excision. A tension-free end-to-end anastomosis was performed using 4/0 PDS. Tracheobronchial remnants were confirmed at histopathologIcal finding. Esophageal contrastography demonstrated a good diameter anastomosis. One month after the operation, she was able to swallow solid food. Surgery for congenital stenosis is indicated when endoscopic management fails. RATS for esophageal diseases in patients under 10 kg is feasible, and peridiahragmatic esophagus particularly suits to this technique. Previous accesses to the thorax seems not to be a limitation.
Bronchogenic cysts are congenital foregut malformations most often found in the mediastinum or lung parenchyma. Abdominal locations are exceedingly rare and most commonly found in the left retroperitoneum. They may mimic other intra-abdominal masses and pose a preoperative diagnostic challenge. A 17-year-old female presented with 4 days of diffuse, mild abdominal pain. Ultrasound and MRI identified a 5.5 × 3.8 × 3.1 cm unilocular, hypoechoic cyst adjacent to the terminal ileum at the ileocecal valve. Preoperative differential diagnoses included an ileal duplication cyst. Single-incision laparoscopy was converted to a small open ileocecal resection. Histopathology revealed a bronchogenic cyst, lined with ciliated respiratory epithelium with focal non-keratinized squamous epithelium. The patient recovered uneventfully and remained asymptomatic at 3-week follow-up. Although rare and often detected incidentally, bronchogenic cysts should be considered in the differential diagnosis of abdominal cystic lesions. Complete surgical excision is recommended to establish the diagnosis, relieve symptoms, and prevent potential complications. To our knowledge, this is the first reported case of a bronchogenic cyst located at the terminal ileum.
Introduction Epigastric heteropagus twinning is an extremely rare form of asymmetric conjoined twinning, characterized by a parasitic twin attached to the upper abdomen of a structurally normal autosite. Case Presentation We report a full-term female neonate born via elective cesarean section with an epigastric heteropagus parasitic twin. The parasitic twin had a well-formed head and neck with a rudimentary trunk, and absence of limb structures. The autosite was clinically stable, with normal cardiovascular and respiratory status, and no congenital anomalies were detected on echocardiography. Contrast-enhanced computed tomography revealed a well-formed calvarium and cervical spine in the parasitic twin, absent thoracic and abdominal organs, and two major feeding vessels from the autosite. There was no visceral sharing. Surgical separation was performed on day 10 of life by a multidisciplinary team. Both feeding vessels were carefully ligated, the parasitic twin was completely excised, and the anterior abdominal wall was reconstructed. Postoperative recovery was uneventful; oral feeding resumed on day 3, and the infant was discharged on day 7. At 9-month follow-up, growth and developmental milestones were normal, with no evidence of hernia or other complications. Conclusion Early separation of epigastric heteropagus twins is safe and effective when there is no visceral sharing, the vascular anatomy is clearly defined, and multidisciplinary planning is implemented.
Primary sclerosing cholangitis (PSC) is a rare cause of pediatric cholestasis and may exceptionally present as a focal mass-forming lesion. We report the case of a 9-year-old boy presenting with progressive obstructive jaundice in whom magnetic resonance cholangiopancreatography revealed a solid lesion at the hepatic duct confluence, highly suspicious for malignancy. Given persistent biliary obstruction and the inability to reliably exclude cholangiocarcinoma using noninvasive or endoscopic diagnostic methods, surgical exploration was undertaken. Complete excision of the lesion followed by Roux-en-Y hepaticojejunostomy was performed. Histopathological examination demonstrated concentric periductal fibrosis consistent with PSC, associated with established biliary cirrhosis, without evidence of malignancy. The postoperative course was uneventful, with rapid clinical and biochemical improvement. This case highlights a rare pseudotumoral presentation of pediatric PSC and underscores the role of surgery as a definitive diagnostic and therapeutic option when malignancy cannot be excluded.
Congenital intestinal web of the jejunum is an exceptionally rare condition. While most intestinal atresias present during the neonatal period, jejunal webs with central apertures can lead to delayed presentations, often causing diagnostic challenges. We report two cases of jejunal web in children diagnosed beyond the neonatal period. Both patients presented with failure to thrive and bilious vomiting. One patient, a 2-year-3-month-old girl, was diagnosed with a single jejunal web and underwent successful web excision. The other, a 2-year-8-month-old girl, was found to have double jejunal webs-an extremely rare occurrence. She underwent resection and anastomosis but required reoperation due to anastomotic disruption. These cases highlight the diagnostic challenges associated with delayed presentation of jejunal webs and reinforce the importance of considering this rare anomaly in cases of chronic partial intestinal obstruction.
Background We present a case of a premature female neonate with a triad of persistent urogenital sinus with urinary ascites, bilateral hydrocolpos in a duplex uterus, and abdominal wall hypoplasia resembling Prune Belly-like syndrome, combined with severe bilateral cystic dysplastic kidneys and complex urinary obstruction. Case Report A female infant was born at 34(3/7) weeks' gestation via cesarean section due to prenatal detection of hydrops fetalis and massive urinary ascites. Prenatal ultrasound had shown oligohydramnios, ascites, megacystis, and hydrocolpos. Postnatally, urinary ascites, a persistent urogenital sinus, severe upper urinary tract dilation, bilateral dysplastic kidneys, and an obstructive vaginal septum were confirmed. Management included staged urinary drainage, vaginal septum incision, intensive respiratory and renal support, and multidisciplinary care. Conclusion This unique combination of anomalies presents significant diagnostic and therapeutic challenges. Early recognition and individualized multidisciplinary management are essential to improve postnatal outcomes and guide long-term planning in such cases.
Background:Fibrous hamartoma of infancy (FHI) is a rare benign soft tissue tumor of early childhood, often misdiagnosed due to its clinical and/or radiological resemblance to vascular malformations or pediatric soft tissue neoplasms. Case Presentation:A 7-month-old male presented with a rapidly enlarging, firm, non-pulsatile subcutaneous mass involving the anterior aspect of almost the entire right arm. MRI suggested a low-flow vascular malformation; however, due to clinical concern for alternative pathology and the lesion's benign appearance, large size, superficial location, and resectability, complete excision was performed. Histopathology revealed the characteristic triphasic pattern confirming fibrous hamartoma of infancy. The patient recovered well with no recurrence at 3-month follow-up. Conclusion:This case highlights the diagnostic pitfalls of FHI, which may closely mimic vascular anomalies on imaging, and underscores the importance of surgical excision for both definitive diagnosis and curative treatment. To our knowledge, this represents the first reported case of FHI from Palestine.
Waugh's syndrome, the rare coexistence of intussusception and intestinal malrotation, has rarely been reported in literature, with fewer than 100 cases described globally. Its diagnosis is challenging due to non-specific symptoms of both conditions and the frequent success of non-operative reduction of intussusception, which often results in underdiagnosis of malrotation. We present a case of a 6-month-old boy who developed recurrent vomiting, feeding intolerance, and failure to thrive. Ultrasound imaging revealed ileocolic intussusception and a mobile cecum. Laparoscopy confirmed malrotation, and the patient underwent manual reduction of intussusception, followed by Ladd procedure. The child had an uneventful postoperative recovery without complications. Early diagnosis of Waugh's syndrome requires a high index of suspicion, particularly in recurrent obstructive symptoms where malrotation may be present. Enhanced imaging techniques can facilitate prompt diagnosis and guide appropriate surgical intervention, preventing complications. Clinicians should maintain a high degree of suspicion for Waugh's syndrome to ensure timely and effective intervention.
Indocyanine Green Fluorescence (ICG-F)-guided surgery has, in recent years, optimized the precision and safety of surgical procedures. Although its applications are increasingly widespread, in most cases, the dye is administered intravenously. We present a case of inhaled indocyanine green use for the identification and resection of pulmonary metastatic nodules. A 12-year-old female patient with a history of sternal Ewing sarcoma with bone and pulmonary metastases was treated with chemotherapy, proton therapy, partial sternal and costal cartilage resection, and reconstruction with mesh and absorbable plates. She later developed pulmonary relapse, with two subpleural metastases identified in segments 6 and 9 of the right lung. After initiating chemotherapy, thoracoscopic surgery was planned to resect the nodules. Before surgery, nebulization of indocyanine green was performed via an endotracheal tube at 0.2 mg/kg using an inhalation chamber for 5 minutes at 6 liters per minute. During surgery, fluorescence was observed in the insufflated lung parenchyma, allowing for clear differentiation of metastatic nodules from peripheral fibrotic or inflammatory tissue and enabling a safe wedge resection of both lesions. The postoperative period was uneventful, and the patient is currently completing postoperative chemotherapy cycles.
Congenital hernia in the umbilical cord is a rare form of ventral abdominal wall defect, which, if not diagnosed, can lead to iatrogenic intestinal injuries due to improper umbilical clamping. We report a case of a newborn referred to our center for intestinal obstruction caused by a decapitated Meckel's diverticulum located within the umbilical cord. Only a few similar cases have been documented in the literature, including four fatalities. Our aim is to present this rare case to the surgical community to raise awareness about it as a potential differential diagnosis in neonatal obstruction cases and emphasize the importance of early treatment to reduce the risk of high morbidity and even mortality.
This case report describes the recurrence of an immature thyroid infiltrating teratoma in a female infant. Initially treated surgically for a cervical mass, the teratoma recurred, requiring further intervention. The case highlights the importance of multidisciplinary care and long-term follow-up in managing complex pediatric neoplasms.
A posterior cloacal variant is a congenital malformation where a urogenital sinus terminates anterior to a normally placed anus. These are rare malformations with highly variable anatomy. We report on three cases of a novel phenotype of posterior cloaca encountered at our institutions between October 2021 and November 2023. Three newborn girls were referred with ambiguous external genitalia and an anorectal malformation. In all cases, a midline sac, which is likely fused labioscrotal folds, replacing the clitoris was noted anterior to the perineal orifices. Two of the three patients demised as a result of renal failure. The third patient underwent reconstruction and is well. This posterior cloacal phenotype appears to be frequently associated with severe renal insufficiency. In survivors of the neonatal period, a good cosmetic outcome is achievable. Functional outcomes remain to be assessed.
Background:Kaposiform hemangioendothelioma (KHE) is a rare, aggressive vascular tumor frequently complicated by Kasabach-Merritt phenomenon (KMP), a life-threatening consumptive coagulopathy. Neonatal KHE-KMP management requires urgent intervention but is complicated by immunosuppression risks, especially in Bacillus Calmette-Guérin (BCG)-vaccinated infants. Methods:A full-term male neonate with prenatal right upper limb thickening presented postnatally with a violaceous, firm mass. Laboratory findings confirmed KMP. Due to recent BCG vaccination, sirolimus was initially withheld. First-line therapies failed, prompting sirolimus initiation on day 3, supplemented by fibrinogen transfusions. Results:Platelets normalized by day 13 (283 × 10 9 /L) with marked tumor regression. Transient fever/diarrhea resolved with supportive care. At discharge (day 27), platelets stabilized (183 × 10 9 /L). Three-month follow-up showed sustained platelet recovery (268-532 × 10 9 /L), near-complete tumor resolution, and age-appropriate development. Prophylactic trimethoprim-sulfamethoxazole prevented infections. Conclusion:Sirolimus is a critical salvage therapy for refractory neonatal KHE-KMP, even in BCG-vaccinated infants. Timely initiation reverses life-threatening coagulopathy and achieves favorable outcomes, necessitating multidisciplinary monitoring to balance immunosuppression risks.
A newborn of 32 + 6 weeks' gestational age with prenatal diagnosis of gastroschisis was born through elective caesarean section. Ultrasonography at 16 + 4 gestational weeks (GW) showed a gastroschisis with free bowel loops floating in amniotic fluid. From 27 + 4 GW onward, serial ultrasounds showed the disappearance of extra-abdominal intestine and progressive intra-abdominal intestinal loops dilation, raising suspicion for vanishing gastroschisis. Birth weight was 2,136 grams and the external appearance of the abdomen was normal. An exploratory laparotomy was performed, finding a dilated proximal jejunal loop with a type III intestinal atresia, microcolon, and no other remainder bowel in between. The total length of the small intestine was 21 cm. Serial transverse enteroplasties for intestinal lengthening (reaching 38 cm), along with lateroterminal jejunocolic anastomosis were performed. The patient was discharged after 5 months of hospitalization with home parenteral nutrition. At 2 years and 8 months of age, the child is thriving and off parenteral support. Vanishing gastroschisis is a rare and severe form of complex gastroschisis whose prenatal diagnosis is crucial for parental counseling, timely delivery, and early surgical intervention. Multidisciplinary approach is essential to manage intestinal failure and improve long-term outcomes in these patients.
We report the use of endoscopic cholecystoduodenostomy in a 6-year-old child to manage postanastomotic stricture of the common bile duct (CBD) secondary to an intraoperative injury sustained during the resection of an abdominal neuroblastoma (NB). The patient was diagnosed with stage M NB, characterized by dissemination to the bone marrow and vertebrae, and MYCN amplification. Following multiple cycles of chemotherapy and subsequent hematopoietic stem cell transplantation, the patient was scheduled for surgical resection. Preoperative imaging identified several image-defined risk factors, including infiltration of the porta hepatis and of the duodenopancreatic complex. During the dissection of the tumor, an incidental injury to the CBD occurred, which was subsequently repaired via end-to-end anastomosis. Seven months postoperatively, the patient presented with obstructive jaundice due to an anastomotic stricture, which was successfully managed through the placement of an endoscopic ultrasound-guided lumen-apposing metal stent (LAMS) between the dilated gallbladder and the duodenum. In our experience, endoscopic cholecystoduodenostomy constitutes a novel approach for addressing postoperative iatrogenic CBD strictures in pediatric patients. Further research is warranted to elucidate its benefits and risks as well as to evaluate its long-term efficacy and potential for broader application.
Background:Neuroblastoma, the most common extracranial solid tumor in children, requires meticulous surgical interventions due to its complex anatomical location and proximity to vital structures. Emerging technologies, such as virtual reality (VR) and indocyanine green (ICG) fluorescence-guided imaging, offer promising solutions to enhance surgical precision and outcomes. Despite their potential, their use in pediatric oncology remains underexplored. This case report highlights the integration of VR and ICG fluorescence imaging in the surgical treatment of neuroblastoma, emphasizing their benefits, limitations, and the need for further advancements. Case Description:A 12-month-old female with a prenatal diagnosis of cloacal malformation, Müllerian anomalies, and a horseshoe kidney was under care at our center for the management of her complex urogenital anomalies. During preoperative imaging to plan her reconstructive surgery, an abdominal MRI revealed a solid retroperitoneal mass, later confirmed as a right adrenal neuroblastoma. After six cycles of chemotherapy, metaiodobenzylguanidine (mIBG) scans indicated persistent uptake, suggesting the possible presence of tumor viability. Consequently, a definitive surgical resection was scheduled. The procedure incorporated VR for navigation and ICG fluorescence for real-time vascular mapping, facilitating precise dissection and preservation of critical structures. The patient's postoperative recovery was uneventful, and she was discharged in stable condition. Follow-up evaluations (i.e., MRI, mIBG) showed no evidence of residual macroscopic disease. Conclusion:VR and ICG fluorescence imaging hold promise for enhancing surgical precision and safety in pediatric neuroblastoma. While current limitations include the lack of real-time image overlay and inadequate visualization of tumor margins, future advancements in navigation systems and targeted probes may overcome these barriers and significantly improve oncologic outcomes.
Urethral stone impaction (USI) is an extremely rare cause of acute urinary retention (AUR) in pediatric urology. Few case reports are available, and no consensus guidelines currently exist for managing this condition. We describe our management of such a case and provide a review of the relevant literature. A 4-year-old boy with no prior urological history presented to our emergency department with abdominal pain lasting 8 days. An ultrasound performed 5 days earlier identified a 7-mm stone at the ureterovesical junction. Symptomatic treatment with paracetamol and non-steroidal anti-inflammatory drugs was initiated. However, dysuria, painful penile swelling, and AUR developed 7 days after the onset of pain. A CT scan revealed bilateral hydronephrosis, urinary retention, and a 9-mm stone (980 Hounsfield Units) that had migrated to the proximal anterior urethra. Under general anesthesia, a suprapubic puncture removed 400 mL of urine for analysis. A 7.5-Fr cystoscope was used to identify an impacted stone at the base of the penile urethra. In situ lithotripsy was performed using a holmium laser to fragment the stone in the urethra. The fragments were then pushed into the bladder for complete disintegration. Intravesical fragments were subsequently removed with a Dormia basket. No urethral wounds were observed, and a 10-Fr catheter was placed. Recovery was uneventful, with catheter removal and spontaneous voiding on postoperative day 1. At the 3-month follow-up, the patient exhibited normal voiding and uroflowmetry. AUR secondary to USI is rare and lacks standardized management protocols in pediatric urology. Management of USI should be tailored to the size and location of the calculus, as well as the presence of any associated urethral pathology, with a preference for minimally invasive endoscopic surgery whenever possible. If necessary, urethral in situ laser lithotripsy appears to be a safe and effective treatment option to consider.
Superior mesenteric artery (SMA) syndrome is a rare cause of proximal bowel obstruction in pediatric surgery. We present the management of a rare, idiopathic, and acute form of SMA syndrome in a teenage girl, successfully treated with a robotic approach. A 14.5-year-old girl with no prior medical history and a normal body mass index (BMI) for her age (18.4 kg/m 2 ) was admitted to our department with acute proximal bowel obstruction syndrome. Initial imaging, including an abdominal X-ray, computed tomography scan, and upper gastrointestinal tract radiography, confirmed the diagnosis of SMA syndrome. Conservative management was initiated with nasogastric tube placement, postural adjustments, and optimal nutritional support. However, symptoms persisted, and surgery was performed 5 months after the initiation of conservative treatment. A robotic-assisted duodenojejunostomy, preserving the fourth portion of the duodenum, was performed without intraoperative complications. Postoperatively, the patient experienced immediate symptom relief and was discharged on postoperative day 4. The postoperative course and long-term follow-up (7 years) were uneventful. This case report describes an acute, idiopathic form of SMA syndrome successfully treated with robotic-assisted duodenojejunostomy in a teenage girl with a normal BMI. To our knowledge, this is only the second reported case of robotic surgery for SMA syndrome in the pediatric literature.
