European Journal of Pediatric Surgery Reports最新文献

The fusion of gonadal structures with internal organs is very rare. The close proximity between the left gonad and spleen during embryogenesis may result in splenogonadal fusion (SGF). Moreover, the trapping of hepatocyte-destined mesenchyme cells in gonads is defined as hepatogonadal fusion (HGF). The fusion of gonads with intra-abdominal organs may be continuous and may impair testicular descent during the prenatal period. We herein report an 18-month-old boy presented with bilateral nonpalpable testis due to concomitant continuous HGF and SGF. To our knowledge, this is the first case of concomitant HGF and SGF in a boy with bilateral intra-abdominal testis. Laparoscopic excision of fibrous cords and orchidopexy can be achieved despite continuous fusions.

Congenital anorectal malformations are generally diagnosed and repaired as a neonate or infant, but repair is sometimes delayed. Considerations for operative repair change as the patient approaches full stature. We recently encountered a 17-year-old male with an unrepaired congenital rectourethral fistula and detail our experience with his repair. We elected to utilize a combined abdominal and perineal approach, with robotic assistance for division of his rectourethral fistula and pullthrough anoplasty. Cystoscopy was used simultaneously to assure full dissection of the fistula and to minimize the risk of leaving a remnant of the original fistula (also known as a posterior urethral diverticulum). The procedure was well tolerated without complications. His anoplasty was evaluated 60 days postoperatively and was well healed without stricture. At 9 months of follow-up, he has good fecal and urinary continence. Robotic assistance in this procedure allowed minimal perineal dissection while ensuring precise rectourethral fistula dissection. The length of the intramural segment of the fistula was longer than anticipated. Simultaneous cystoscopy, in conjunction with the integrated robotic fluorescence system, helped reduce the risk of leaving a remnant of the original fistula.

An effective case report delivers clear and valuable clinical or surgical information to the medical community. Case reports dealing with pediatric surgical issues raise the medical community's awareness of rare diseases, unusual presentations of common disorders, or novel surgical or nonsurgical treatment approaches. Thus, case reports contribute substantially to medical advance by sharing remarkable or unexpected findings. For this reason, case reports should be prepared with vigilance, and current conventions on good medial writing practice should be observed. This guideline aims to assist clinicians and surgeons in the successful publishing of a compelling case report in pediatric surgery that is read and understood by the intended audience.

Indocyanine green (ICG)-guided near-infrared fluorescence has been recently adopted in pediatric surgery, although its use in the treatment of congenital hyperinsulinism has not been reported. We present a case of focal congenital hyperinsulinism in which ICG-navigation with ICG was used during surgical treatment. A 3-month-old infant was referred to our institution from a peripheral hospital for episodes of persistent hypoglycemia since birth, with no response to intravenous treatment with diazoxide, octreotide, or hydrochlorothiazide. An abdominal positron emission tomography-computed tomography scan showed a hypermetabolic nodule in the proximal portion of the body of the pancreas, compatible with focal congenital hyperinsulinism. A heterozygous mutation in the ABCC gene (Ala1516Glyfs*19) frameshift type inherited from the father was identified, which supported this diagnosis. Laparoscopy-assisted surgery was performed with ICG-guided near-infrared fluorescence, with intravenous injection of 16 mg ICG (2 mg/mg), which allowed localization of the focal lesion in the body of the pancreas. The lesion was resected with bipolar electrocautery and intraoperative histological study confirmed complete resection. Plasma glucose values normalized 6 hours after surgery and the patient was discharged 5 days later. In conclusion, the use of ICG in the treatment of congenital hyperinsulinism helps to identify hypermetabolic pancreatic nodules, decreasing the likelihood of incomplete resection.

Congenital mature teratomas of the umbilical cord are extremely rare. We report on a girl who presented with a ruptured omphalocele and a 7 cm mass connected to the umbilicus, which we resected on the first day of life. Histology revealed mature umbilical cord teratoma . On the 29th day of life, a secondary laparotomy was necessary to address the associated intestinal malformations (megaduodenum, stenotic small bowel with duplication and malrotation). After a prolonged hospital stay, we discharged the patient in age-appropriate conditions. Antenatal diagnosis of an umbilical cord tumor can be challenging in the presence of an omphalocele. Given the high prevalence of associated malformations, the finding of umbilical cord teratoma should be followed by a detailed and comprehensive neonatal workup for additional abnormalities.

Ewing's sarcoma is the second most frequent primary malignant bone tumor in adolescents and young adults. Locations on the thoracic wall represent up to 20% of primary and secondary locations. We present the case of a 13-year-old patient treated with the use of a radiolucency porous bioceramic prosthesis as a sternal replacement for a wide tumor resection in an oncologic context. Focal radiation therapy was not possible due to the high risk of severe myocardial injuries caused by the sternal location of the tumor. The sternum CERAMIL® (I.CERAM, Limoges, France), in porous alumina (Al ₂ O ₃ ) has already been implanted into adults in sternal replacement during its invasion by a tumor or its infectious destruction. There were no complication concerning the surgery. The last follow-up at 2 years postoperatively reveals a satisfactory clinical situation with any functional thoracic complaint and nor any functional respiratory symptoms. The porous alumina sternal prosthesis offers a reliable alternative for sternal replacement indications for children in an oncologic context.