【Introduction】

PNL (percutaneous nephrolithotripsy) is the priority surgical option for large burden renal stones over 20 mm. Although, it has problems associated with the complexity of the surgical procedure and severe complications.

【Case Presentation】

An eight-year-old girl with Chiari malformation type 2 was referred to our hospital to treat staghorn stones with 45 mm long diameters in her right kidney. The range of leg joint movement was limited. Mini-PNL with 17.5 Fr outer sheath was performed in the prone position with Holmium Laser and a 14 Fr nephrostomy tube was inserted at the closing operation. Stone analysis was struvite. She developed acute pyelonephritis with high grade fever and drowsy consciousness despite preoperative oral medication. Pseudomonas aeruginosa was identified from nephrostomy urine and the intravenous antibiotics was altered to ceftazidime. We performed Second-look PNL with a flexible fiberscope on the 10th day and extracted the nephrostomy tube on the 13th day after the first PNL.

【Discussion】

  Mini-PNL is useful for the patients with comorbidities and disadvantages for retrograde approaches. The body position is important for success in PNL, and it should be selected properly upon each patient’s conditions. Careful management of urinary tract infection is necessary.

The patient is now a 4-year-old boy who has intellectual disability and multiple minor anomalies. He was prenatally diagnosed as the left hydronephrosis (SFU grade 4), and the left hydroureter and horseshoe kidney were also found just after birth. He had suffered from urosepsis twice, at 11 days and 8 months of age. Vesicoureteral reflux was not observed in repeated voiding cystourethrography. Retrograde ureterography under cystoscopy at 10 months of age revealed the left ureterovesical junction obstruction (UVJO), the left incomplete duplicated collecting system, and the left upper pole ureteropelvic junction obstruction (UPJO). We planned to treat UVJO at first, aiming to prevent severe urinary tract infection (UTI)/urosepsis, followed by pyeloplasty for the left upper pole UPJO if necessary. At the age of 11 months, the left ureteral reimplantation (Cohen’s procedure) with ureteral plication was performed. Thereafter, no urosepsis occurred although mild UTI was frequently observed, and the left upper pole grade 4 hydronephrosis and the left hydroureter persisted. At the age of 32 months, laparoscopic left upper pole pyeloplasty was performed. Postoperative course was uneventful except for one episode of UTI just after pyeloplasty, and the left hydronephrosis and hydroureter resolved at 15months after pyeloplasty.

Two-staged surgeries, including the left ureteral reimplantation (Cohen’s procedure) with ureteral plication and laparoscopic left upper pole pyeloplasty, were successful for our patient associated with the left UVJO and the left upper pole UPJO.

Lower urinary tract dysfunction (LUTD) is frequently observed in children with Down syndrome (DS).

Renal function in children with Down syndrome is low, at approximately 80% of that seen in healthy Japanese children, and LUTD may pose a risk of further renal decline. However, clinical guidelines for children with Down syndrome, both in Japan and internationally, contain only limited information regarding the assessment of renal function and LUTD.

We report a case of a child with Down syndrome in whom severe renal impairment was incidentally detected during routine blood draws for a hematological condition, leading to a diagnosis of acute renal failure caused by LUTD.

[Case] A 4-year-10-month-old girl. Routine testing revealed a serum creatinine level of 0.91 mg/dL (eGFR 34.1 mL/min/1.73 m²), indicating marked renal impairment. Physical examination revealed few obvious abnormalities, but she appeared to strain during urination.　Although she was able to void spontaneously, ultrasound examination revealed bilateral hydronephrosis and bladder wall thickening. Urodynamic study (UDS) showed findings suggestive of a low-compliance bladder, detrusor overactivity, and voiding with abdominal pressure; intermittent self-catheterization was initiated, resulting in improved renal function.

Patients with Spina bifida may experience recurrent urinary tract infections, renal deterioration, and urinary leakage due to poor bladder compliance. These conditions are usually managed with anticholinergic medications and intensified clean intermittent catheterization. When symptoms do not improve, augmentation cystoplasty is considered. However, careful patient selection is necessary because the procedure is highly invasive and may cause postoperative complications.

In patients who require cutaneous vesicostomy at a young age because of refractory bladder dysfunction, bladder storage function is often severely impaired, and augmentation cystoplasty is frequently required at the time of vesicostomy closure or as an additional procedure.

Vibegron selectively stimulates β3-adrenergic receptors and improves bladder storage function by relaxing the bladder.

We experienced two boys with myelomeningocele who underwent vesicostomy closure without augmentation cystoplasty after preoperative treatment with vibegron.

Both patients had undergone cutaneous vesicostomy during infancy because of uncontrolled urinary tract infections. During school age, both patients and their families desired urinary continence. Vibegron was initiated before surgery, and vesicostomy closure was performed without augmentation cystoplasty. Both patients have remained free from febrile urinary tract infections and maintained good symptom control for more than one year after surgery.

Introduction

Proximal hypospadias is frequently associated with a bifid scrotum. While some patients require scrotoplasty for aesthetic improvement, its optimal timing remains a subject of debate. Previously, some reported performing scrotoplasty during first-stage urethroplasty, whereas we performed it as a separate procedure after the completion of staged repair. We have recently been performing it simultaneously with the second-stage urethroplasty. We present our surgical procedure and discuss the strategic timing of scrotoplasty for complex hypospadias.

Case report

A 31-month-old boy underwent scrotoplasty during a second-stage urethroplasty. He presented with scrotal hypospadias and a bifid scrotum. Since his glans width was 11mm, testosterone was administered twice before the first surgery. The first-stage urethroplasty using a Byars flap had been performed 6 months prior to the second procedure. In the second-stage repair, the urethroplasty was performed according to the Thiersch-Duplay technique, followed by scrotoplasty and bilateral orchiopexy. He was discharged on postoperative day 2, and the catheter was removed 12 days after surgery. Four months postoperatively, no complications, such as urethral fistula or skin necrosis, were observed, and an excellent cosmetic outcome was achieved.

Conclusion

Since thorough degloving during the first-stage surgery can alter the position of the scrotal component, and meticulous surgical attention to flap perfusion is necessary when urethroplasty is performed using the inner preputial flap, simultaneous scrotoplasty is not advisable at that stage. However, scrotoplasty can be safely combined with the second-stage urethroplasty. This approach reduces the total number of surgeries and achieves satisfactory cosmetic outcomes with fewer complications.