Journal of Pediatric Surgery Case Reports最新文献

Introduction

During cloacal reconstruction, vaginal replacement with an intestinal graft is considered when native vagina does not reach the perineum. Risks include need for revision surgery, mucus production, colitis, and rarely dysplasia. Given these concerns, alternative vaginal reconstructive procedures are being considered. We present a novel case using AlloDerm for vaginal replacement in a female infant undergoing cloacal reconstruction.

Case presentation

A 17-month-old female with a complex medical history including Stage 5 chronic kidney disease (CKD) underwent repair of a 4.5 cm cloaca via a posterior sagittal approach. Although the rectum was adequately mobilized off tension, the vagina remained 4 cm from the perineum when off tension. Because vaginal replacement using sigmoid colon would have required exploratory laparotomy in this medically complex child, decision was made to proceed with vaginoplasty using an AlloDerm graft. Two 2 × 4 cm pieces of AlloDermwere placed longitudinally to create the anterior and posterior vaginal walls. The lateral walls and surrounding fat were then secured and the introitus was created. Vaginoscopy was performed three months post-operatively and confirmed an intact graft approximately 2–3 cm long with a patent anastomosis to the native vagina.

Conclusion

AlloDerm is an acellular tissue matrix that supports tissue regeneration. This is a promising vaginal replacement option for patients with cloaca who are not good candidates for a colonic vaginoplasty. Future studies are needed to assess long-term outcomes of this graft for prepubertal vaginoplasties.

Introduction

Case presentation

Conclusion

Introduction

There is no true consensus on the optimal management strategy for spontaneous bile duct perforation in neonates, but previous studies favor surgical drainage over primary repair. Management of subsequent high-volume biliary drainage has not yet been reported, particularly bile refeeding after surgical drain placement.

Case presentation

We present a case of spontaneous bile duct perforation in a previously healthy 16-day-old female who presented with abdominal distension, acholic stools, and direct hyperbilirubinemia. Intraoperative cholangiogram demonstrated a perforation at the proximal common bile duct, which was patched with omentum. A surgical drain was placed in the sub-hepatic space. She recovered well and was discharged home, but continued to have high volumes of bile drainage. She required readmission due to dehydration, electrolyte derangement, and weight loss. Upon readmission, a nasogastric feeding tube was placed, and she was refed bile from the drain every 8 hours while continuing to breast and bottle-feed. Over the next five days, drain output decreased and she was again discharged home. At one-week follow up, there was no additional bile drainage, she was well-appearing, and gaining weight. The drain was removed in clinic. She was subsequently seen approximately four months from her operation at which time she was well and thriving.

Conclusion

Intraoperative cholangiogram and drain placement are safe and effective for spontaneous bile duct perforation. Bile refeeding can be used in the setting of excessive biliary losses after surgical drainage for neonatal spontaneous bile duct perforation and may decrease bile drainage.

Introduction

Large masses in the pleural cavity or mediastinum in pediatric patients may present an operative challenge, not only in terms of obtaining adequate space in the small chest for safe dissection, but in attaining adequate chest closure.

Case series

Three adolescent males presented with large masses in the mediastinum or thoracic cavity. Their presenting signs were primarily chronic cough and chest pain. Patient 1 was a 13-year-old male with a history of spina bifida and a 12 × 10 × 9 cm malignant germ cell tumor in the mediastinum/right chest. Patient 2 was an 18-year-old male with a 24 × 15 × 11 cm right thoracic synovial sarcoma in the posterior mediastinum/right hemithorax. Patient 3 was a 14-year-old male with a 19 × 14 × 10 cm lymphovascular malformation in the posterior mediastinum/right hemithorax. In all three patients, after the resection of the tumor via a clamshell throacotomy, sternal plating was employed for sternal wound closure. Post operative complications included a superficial surgical site infection and a chyle leak, but no wound dehiscence or breakdown.

Conclusion

The bilateral clamshell thoracotomy is a suitable approach for the resection of extensive mediastinal and thoracic masses in adolescents. The use of a sternal plate leads to adequate sternal healing.

Introduction

Plasma free hemoglobin (PFH) is a product of intravascular hemolysis associated with certain disease states as well as the shearing effects of mechanical support devices, such as extracorporeal membrane oxygenation (ECMO). There are detrimental effects of intravascular hemolysis secondary to ECMO including severe hyperbilirubinemia, thrombus within the ECMO circuit causing inability to provide adequate support, and as a result, a need for ECMO circuit exchanges. Neonates can experience renal failure, kernicterus, and other significant morbidities as a result of hemolysis secondary to ECMO.

Case series

We present three cases of neonates with a gestational age of 35–37 weeks who had a left-sided CDH and were cannulated to VA-ECMO on the first day of life using an 8 French arterial cannula and a 10 French venous cannula. Within the first 10 days of the ECMO run all patients developed markedly elevated PFH of (peaks of 465, >500 and > 500 mg/dL), and creatinine, and underwent therapeutic plasma exchange (TPE). TPE was done using frozen fresh plasma (FFP) replacement in line with the ECMO circuit. All patients showed a remarkable decrease in the PFH levels after the TPE treatment. The number of TPE rounds ranged from 1 to 6, depending on the evolution of the PFH levels. One patient developed hypocalcemia that resulted in decreased cardiac pulsatility during the first round of TPE that resolved with aggressive calcium repletion. The total duration of the ECMO runs ranged from 16 to 29 days. None of the patients required continuous renal replacement therapy. All patients were discharged home at ages ranging from 2 to 6 months.

Conclusion

Mechanical cell hemolysis is a significant source of morbidity in neonatal patients on ECMO. This case series exemplifies a safe and impactful intervention to address hemolysis in a critically ill patient population.

Introduction

Paratubal cysts are rare cystic structures that are usually asymptomatic; rarely, they may cause torsion of the adnexa.

Case presentation

We report the case of a 13-year-old premenarchal female who presented with left lower quadrant abdominal pain for 4 days. She had a history of a previous right fallopian tube cyst and torsion 9 months prior, treated at an outside hospital with diagnostic laparoscopy, right tubal detorsion, and paratubal cystectomy. Abdominal ultrasound demonstrated a right-sided para-ovarian fluid filled structure. She underwent diagnostic laparoscopy, which demonstrated bilateral 5 cm paratubal cysts attached to the fimbriae of both fallopian tubes, with bilateral fallopian tube torsion. Both ovaries and fallopian tubes had good blood flow. Both fallopian tubes also had findings of multiple small cysts budding from the tubular mesothelium. Bilateral detorsion was performed. The large cysts were excised, and the multiple small cysts were drained. We preserved both fallopian tubes and most of the fimbriae. Pathology resulted in benign paratubal cysts.

Conclusion

Adnexal torsion due to paratubal cysts can occur in the premenarchal pediatric population, affect both fallopian tubes, and recur. This case report aims to highlight unusual cause of adnexal torsion and its potential harm if not diagnosed in a timely manner.

Introduction

The advent of laparoscopic and robotic surgery has revolutionized many operations throughout the surgical world, leading to smaller incisions, reduced post-operative pain, shorter hospital stays, and accelerated post-surgical recovery. However, these advancements come with drawbacks such as limited accessibility, high costs, and sometimes the requirement of multiple incisions. We present an alternative approach for an open “incisionless” colostomy, using an intra-rectal Foley catheter to help identify the descending colon.

Case series

Two patients underwent colostomy creation utilizing this minimally invasive open technique. The technique involves placement of a 24-french Foley catheter intra-rectally prior to prepping and draping the patient, making a circular incision, generally less than 3 cm in diameter over the left lower quadrant where the ostomy will be matured, and identifying of the colostomy limb through palpation of the intra-rectal catheter. This technique was utilized in two patients with minimal previous abdominal surgery and a normal or low body mass index. The first patient was 16-year-old male who had suffered a spinal cord injury, which was complicated by neurogenic bowel. He had considerable difficulties in emptying his colon appropriately, refused utilizing an enema regimen, and requested a permanent stoma. The second patient was a 10-year-old male with developmental delays, and severe idiopathic constipation refractory to medical intervention. In this case the patient's family requested a permanent stoma.

Conclusions

The open “incisionless” minimally invasive approach to colostomy creation provides an alternative approach to laparoscopic methods, with the benefit of minimal trauma to the abdominal wall and absence of a post-operative incision other than the colostomy itself.

Introduction

Intussusception, primary or secondary, is a common cause of mechanical obstruction in infants and children. Rarely, however, an intussusception is caused by a lesion of the appendix.

Case presentation

A thirty-two-month-old female was brought to the emergency department with abdominal pain and passage of red currant jelly stool. The abdominal exam revealed a palpable mass in the upper abdomen. Ultrasound examination of the abdomen showed the classical signs of an ileocolic intussusception: target sign in the transverse view and pseudokidney sign in the longitudinal view. A hydrostatic barium enema reduction was done. Successful reduction of the intussusception was confirmed by seeing the contrast reflux into the ileum. However, several hours later the abdominal pain recurred, and a follow-up ultrasound showed recurrence of the intussusception. The patient was taken to the operating room for an exploratory laparotomy. Following the manual reduction of the intussusception, a palpable mass was identified at the base of the appendix protruding into the cecum. An enterotomy was made on the cecum near the base of the appendix, and a polyp was found protruding from the inside the appendix into the cecum. We did an appendectomy, which included the polyp. The patient recovered uneventfully. The pathology analysis was consistent with a juvenile polyp.

Conclusion

Although rare, appendiceal polyps can serve as a lead point for intussusception of the ileocecal region. Such type of intussusception may recur after a reduction enema and may require a surgical exploration.

Introduction

Laparoscopic appendectomy is a common procedure for pediatric surgeons, primarily employed for acute appendicitis. However, this case report presents a unique scenario of a 12-year-old male with symptoms suggestive of appendicitis, diagnosed with an appendiceal mass causing torsion, due to a vascular malformation or reactive vascular proliferation.

Case presentation

A 12-year-old male presented with three days of abdominal pain, diarrhea, and emesis. The clinical exam was concerning for appendicitis. Ultrasonography revealed a mildly dilated appendix terminating in a hypervascular, lobulated mass, suggestive of a large solid appendiceal neoplasm. Subsequent CT scan further characterized this lesion that encased the mildly dilated appendix, compressing it and causing reactive appendiceal wall thickening. All laboratory studies were within normal limits. Diagnostic laparoscopy revealed a torsed, hemorrhagic mass originating from the distal aspect of the appendix. He underwent laparoscopic appendectomy, and the entire specimen was removed intact. Pathology reported a non-malignant vascular malformation or reactive vascular proliferation. The patient had an uncomplicated postoperative course and was managed like a standard postoperative appendectomy. One year following this procedure, he was readmitted with a large abdominal mass found to be Burkitt's lymphoma and was started on treatment. Pathology from the original appendiceal resection was reviewed and confirmed to be unrelated.

Conclusion

Vascular malformation/proliferation of the appendix is rare and may present similarly to appendicitis. Any mass may serve as a lead point for appendiceal torsion. Simple appendectomy is likely sufficient for such vascular lesions, and many pediatric appendiceal neoplasms.

Introduction

Spindle cell lipomas are a rare, benign soft tissue mass that are easily treated with surgical excision. Although typically occurring in males and much later in life, in this case report, we present a case of a spindle cell lipoma occurring in a female teenager.

Case presentation

The patient is a healthy 16-year-old Female who presented with a soft, mobile, and poorly defined posterior flank mass on physical exam. As it was not well-visualized by ultrasound, a magnetic resonance image (MRI) was obtained, revealing a well-defined, T1 and T2 bright, ovoid lesion in the subcutaneous tissue. On MRI, the mass measured 7.4 x 4 × 4.5 cm and was consistent with a benign fatty mass. The mass was thought to be a lipoma and it was recommended that it be excised to confirm the diagnosis. However, the patient elected to defer due to concerns about undergoing surgery. Two years later she re-presented with a much larger mass in the same region and a surgical excision was then performed. On excision, the mass appeared smooth, glistening, and fatty, measuring 13.5 x 9.8 × 4.5 cm. Histology revealed encapsulated fibroadipose tissue containing both adipocytes and CD34-staining spindle cells, consistent with a diagnosis of spindle cell lipoma. The patient continues to do well 6 months later with no evidence of recurrence.

Conclusion

Spindle cell lipoma cannot be differentiated from lipomas pre-operatively.