Mark A. Rich

Predisposing factors in bladder calculi: review of 100 cases

One hundred patients, aged twenty to ninety-two years, underwent 111 procedures for removal of bladder calculi. Most patients (88) had some type of bladder outlet obstruction. Two types of stones were identified: those that had apparently formed in the upper tract and been trapped in the bladder (17 cases) and those that appeared to have formed in the bladder in the presence of various types of outlet obstruction. Stone analysis revealed uric acid stones in 50 percent, calcium oxalate stones in 19 percent, and stones of mixed composition in 31 percent. Five patients had metabolic abnormalities predisposing to stone formation; in 2 cases, these abnormalities were discovered during the evaluation for stone disease. Treatment depended on stone characteristics, associated pathology, and the general health of the patient. A review of the literature with regard to the morbidity and mortality of combining treatment of vesical calculi and bladder outlet obstruction secondary to prostatic obstruction is included.

Association of Sickle Cell Disease, Priapism, Exchange Transfusion and Neurological Events: Aspen Syndrome

Priapism and acute neurological events are believed to be unrelated complications of sickle cell hemoglobinopathy. We describe a syndrome based on our experience and a review of the literature of significant neurological events after partial exchange transfusion to treat priapism in sicklemic patients. Severe headache is often the initiating symptom of this complex. The ensuing neurological events range from seizure activity to obtundation requiring ventilatory support. The proposed pathophysiology of these neurological events is related to cerebral ischemia after an acute increase in per cent total hemoglobin, concomitant decrease in per cent hemoglobin S and subsequent release of vasoactive substances during penile detumescence. We have termed this constellation of events the ASPEN syndrome, an eponym for association of sickle cell disease, priapism, exchange transfusion and neurological events. Early recognition and aggressive medical management resulted in complete reversal of neurological sequela.

Spectrum of genitourinary malformations in patients with imperforate anus

A retrospective review of 244 cases of imperforate anus demonstrated a 48% incidence of associated genitourinary (GU) anomalies. The incidence and severity of associated GU anomalies was directly related to the level of the fistula between the blind-ending rectum and the GU tract. High-level fistulae to the bladder neck in males and the cloaca in females demonstrated a 90% incidence of associated genitourinary anomalies. In contrast, lower-level fistule to the perineum revealed only a 14% incidence of associated GU anomalies. Classification of anorectal anomalies by fistula level provides an accurate means of predicting the likelihood of an associated GU malformation.

Persistent cloaca and phallic urethra

During the last 8 years 54 children with a persistent cloaca have been treated by one of us (A. P.). The most common associated anomalies involved the urinary tract, with renal agenesis, renal dysplasia, vesicoureteral reflux and megaureter encountered most frequently. Four patients demonstrated the rare entity of an opening at the tip of a pseudophallus and 2 also had an accessory urethra in association with the cloacal malformation. These children exhibited some form of masculinization of the external genitalia with the accessory urethra or cloacal channel assuming a phallic position in an enlarged clitoris. No adrenal, metabolic or chromosomal abnormalities were detected and virilization was limited to the genitalia. These cases illustrate the greater predilection of these patients to more complex congenital malformations than patients with an isolated cloaca. The posterior sagittal approach has been used effectively for repair of these congenital malformations.

Autosomal Dominant Transmission of Ureteral Triplication and Bilateral Amastia

We report a case of ureteral triplication as part of an autosomal dominant syndrome comprising bilateral amastia, pectus excavatum, umbilical hernia, patent ductus arteriosus, dysmorphic low set ears, ptosis, epicanthic folds with an antimongoloid slant to the eyes, hypertelorism, high arched palate, flat broad nasal bridge, tapered digits, cubitus valgus and syndactyly.

Applications of the Peel-Away Introducer Sheath

The peel-away introducer sheath set, originally designed for the antegrade insertion of the silicone rubber Universal stent, also is useful for antegrade or retrograde basketing of ureteral stones, negotiation of a dilated renal pelvis and tortuous ureter with minimal trauma, retrograde flushing of ureteral stones for percutaneous extraction, removal of dislodged stents from the renal pelvis and ureteral examination with a flexible endoscope or rigid ureteropyeloscope.

Stentless pediatric robotic pyeloplasty

Objectives: Open dismembered pyeloplasty remains the standard of care for the correction of ureteropelvic junction obstruction in children. We describe our experience with a tubeless, stentless pediatric robotic pyeloplasty technique. Methods: Between October 2008 and September 2009, 12 consecutive children underwent robotic dismembered pyeloplasty. Ureteral stents or nephrostomy tubes were not used. Operative time, hospital stay, days of Jackson–Pratt drainage, and complications were analyzed. Postoperative renal ultrasonography was obtained at 4–6 weeks after surgery. Results: The mean patient age was 9.1 years (3.5–16). The mean operative and console times were 178 (122–250) and 129 (96–193) minutes, respectively. The Jackson–Pratt drain was removed after a mean of 1.8 days (1–4). The mean hospital stay was 2.4 days (1–4.5). There were no complications. Mean follow up was 16 months (12–24 months). All patients had complete resolution of symptoms. Hydronephrosis either completely resolved or significantly decreased in all cases. In cases without complete resolution of hydronephrosis, 99m Tc-MAG-3 diuretic renography showed preservation of renal function without obstruction. Conclusions: Robot-assisted laparoscopic pyeloplasty can be safely performed without internal indwelling stent drainage. In children, this avoids the need for additional anesthesia and stent-related morbidity.

N-myc Oncogene Expression in Porcine Renal Development and Oncogenesis

ABSTRACT: N-myc oncogene expression was characterized in porcine kidneys to investigate the potential role of this gene in normal renal development and oncogenesis. N-myc RNA expression was detected in porcine kidneys from birth until 5 wk of age, which corresponds to the time when glomerular differentiation is completed. Immunohistochemical studies revealed that N-myc protein was selectively expressed in the primordia of renal proximal tubule epithelial cells. These cells were cultured in vitro and continued to express N-myc for a limited time. Comicroinjection of a mutant ras oncogene and N-myc into these cells led to focus formation in soft agar, loss of contact inhibition, and the establishment of an immortalized cell line. These findings support a multistep model of renal oncogenesis that involves overexpression of N-myc.

Latex allergy and anaphylaxis

The incidence of intraoperative anaphylactic reactions has been estimated to be between 1 in 1,500 and 1 in 5,000; approximately 5 to 10% of patients developing intraoperative anaphylaxis die as a direct result [1]. Triggering agents implicated in these reactions include neuromuscular blocking drugs, intravenous anesthetics, intraoperative antibiotics, radiocontrast material, blood products, and protamine. No etiology is identified, however, in approximately 16% of these cases [2]. In the past 10 years, case reports have been published in the literature linking latex exposure to severe, immediate, type I allergic reactions in the operating room [3]. At present, this linkage is inferred and direct proof is lacking. However, latex-mediated immune reactions appear to be real and warrant consideration whenever unexplained anaphylactic or allergic reactions occur in the operating room.

Flexible cystoscopy in surgical repair of traumatic urethral strictures in children

Flexible cystoscopy via a suprapubic cystostomy tract was utilized to locate the proximal segment of a complete traumatic disruption of the bulbar urethra secondary to a pelvic fracture. With the fiberoptic light of the instrument visible in the perineum, the proximal urethra was easily and safely dissected free from the enveloping fibrotic scar tissue and a simple one-stage urethroplasty performed. The patient is voiding normally eight months postoperatively.