Testicular regression syndrome: a clinical and pathologic study of 11 cases.

CONTEXT: The vanishing or regressed testis is an entity well known to urologists and pediatric surgeons, affecting approximately 5% of patients with cryptorchidism. However, there is little review and discussion of this entity among pathologists with only 2 of 40 published reviews of testicular regression syndrome (TRS) found in the pathologic literature. OBJECTIVES: To assess recognition of TRS among a subset of pathologists and to determine the applicability of histologic criteria for TRS as published. DESIGN: An 8-year retrospective review of cases submitted as atrophic or regressed testis was performed. Original diagnosis and diagnosis after review were compared to assess pathologic recognition of TRS. Pathologic assessment included identification of vas deferens, epididymis, dystrophic calcification, hemosiderin, dominant vein, pampiniform plexus-like vessels, and vascularized fibrous nodule formation. At minimum, the presence of a vascularized fibrous nodule (VFN) with calcification or hemosiderin or VFN with cord element(s) was required for diagnosis. SETTING AND PARTICIPANTS: Medical records and pathologic specimens of patients undergoing surgery for cryptorchidism or with specimens reviewed at a medium-sized university hospital were analyzed. RESULTS: The original diagnosis in 3 (23%) of 13 cases was that of TRS. On secondary review, 11 (85%) of 13 cases showed features consistent with TRS. The diagnoses both before and after review showed a concurrence of 23% (3/13 cases). Two (15%) of 13 cases were correctly recognized and diagnosed as TRS at primary review; 1 case originally thought to represent TRS was not confirmed. Pathologic features correlated well with those reported in the literature. Among all 13 cases, the 11 confirmed TRS cases showed VFN in 11 (85%), intranodular calcification in 8 (62%), intranodular hemosiderin in 9 (69%), vas deferens in 9 (69%), epididymal structures in 5 (38%), and a dominant venous structure in 11 (85%). The average size of the VFN was 1.1 cm. CONCLUSION: A urologic and pediatric surgical problem, TRS may be unrecognized by many practicing pathologists. In the typical situation in which a blind ending spermatic cord is submitted for tissue analysis, characterization of such cases as consistent with regressed testis is desirable and achievable in a high percentage of cases. Pathologists may play a pivotal role in management of these patients since histologic confirmation of the testis as regressed reassures the surgeon and the family of the correctness of diagnosis and can eliminate the necessity for further intervention.

Separation of complex pygopagus conjoined twins.

Pygopagus twins were born with a unique spectrum of anomalies including a conjoined distal spinal cord, single kidney (in twin A), single rectum (in twin A), single vagina (in twin B), and severe central nervous system anomalies in twin B that precluded her independent survival. Separation at 10 weeks of age was tailored toward Twin A's survival. This report discusses the surgical modifications necessary in view of the unique anatomy, including salvaging the distal spinal cord and vagina for twin A.

Locally invasive aspergillosis of the bowel.

Locally invasive aspergillosis of the bowel and peritoneal cavity is a rare complication of immunosuppression, broad-spectrum antibiotic therapy, and corticosteroid administration. We present the case of a 9-year-old boy with acute lymphocytic leukemia who presented with a small bowel obstruction. Surgical treatment of the aspergillosis required multiple aggressive resections of all involved bowel segments. Parenteral nutrition and intravenous Amphotericin B and 5-Fluorocytosine therapy were also instituted. Achievement of a hematologic remission is another prerequisite for cure.

Broviac central venous catheters inserted via the saphenous or femoral vein in the NICU under local anesthesia.

We present our experience with 92 Broviac central venous catheters inserted into 84 infants over a 31-month period. Our technique specifies placement in the neonatal intensive care unit under local anesthesia, with insertion to the inferior vena caval-atrial junction via the saphenous or femoral vein, with a subcutaneous tunnel to an exist site on the anterior thigh. We conclude this technique to be safe, efficient, convenient, cost-effective, and minimally uncomfortable to the infant, with no increase in morbidity or mortality in comparison to previously described methods.

Ventricular sepsis and abdominally related complications in children with cerebrospinal fluid shunts.

In a retrospective study of 300 children who underwent placement or revision of cerebrospinal fluid (CSF)-peritoneal shunts during a 10-year period, 15 (5%) developed shunt-related abdominal complications with ventricular sepsis and two developed acute perforated appendicitis. Abdominal complications and associated shunt infections suggested two potential modes of development: (1) descent of contaminated CSF from an infected shunt into the abdomen (CSF ascites--four patients, CSF pseudocysts--four patients, and shunt-induced abscess/peritonitis--five patients); and (2) ascent of bacteria into the shunt from an abdominal source (visceral perforation by the shunt catheter--two patients and acute perforated appendicitis--two patients). Three types of shunt systems were placed during the study period; five of the seven (71%) most serious septic complications were associated with the use of Raimondi spring-reinforced catheters. Bacteria isolated in this series were associated with differing modes of sepsis: those involving descent of bacteria into the abdomen from an infected shunt were predominantly gram-positive, cutaneous microorganisms, whereas those associated with ascent of bacteria from the abdomen into the shunt were mixed, gram-negative intestinal microorganisms. Appendicitis did not result in shunt infections. Aggressive treatment resulted in no operative or complication-related deaths. Removal of the shunt catheter from the abdomen and intravenous antibiotics were essential for eradication of sepsis; laparatomy was required only for cases with suspected peritonitis. In eight of the 17 (47%) patients, reestablishment of CSF-peritoneal shunts was performed after resolution of shunt-related complications. In recent years improved shunting materials and supportive care have reduced the incidence of the most serious of these complications.

Ischemic extremities due to compartment syndromes in a septic neonate.

The clinical presentation and diagnostic evaluation of a septic neonate with sclerema neonatorum and impending tissue loss secondary to compartment syndromes is presented. Vascular disease leading to tissue loss is uncommon in children. To our knowledge, this is the only reported case of a compartment syndrome leading to tissue loss in a neonate.

Effects of total parenteral nutrition and semi-starvation on the liver of beagle puppies.

The effects on hepatic growth of fat-free TPN and semistarvation have been studied in newborn beagle puppies. Our data indicate that liver growth during TPN is characterized by normal deposition of protein and minerals, with increased glycogen and decreased lipid content. While the lipid pattern of TPN livers is normal, the phospholipid fraction contains an abnormal fatty acid pattern, consistent with the development of essential fatty acid deficiency. The exact physiologic significance of this is unknown, but it is tempting to speculate that changes in integrity of the hepatic cell membrane as a result of this abnormal fatty acid pattern might account for some of the clinical abnormalities of hepatic function during TPN.