Blair Carpenter

Intraabdominal Pulmonary Sequestration

A left upper quadrant fetal abdominal mass was detected at 24 weeks gestation. The mass was again confirmed in a postnatal ultrasound. Pathological analysis of the excised mass demonstrated an intraabdominal lung sequestration with Stocker type II congenital cystic adenomatoid malformation (CCAM). The sonographic characteristic of these lesions are those of a homogeneous echogenic mass with variable shape passing through or arising from the diaphragm. Surgical excision is recommended because of the uncertainty of the preoperative diagnosis and the possibility of malignant changes in CCAM.

Improved Movat Pentachrome Stain

Movat in 1955 developed a staining method which demonstrates collagen fibers, mucin, muscle fibers, elastic fibers, fibrin and fibrinoid changes in a single section. His procedure was considered excellent by Lynch et al. (1969)

Fibromatosis in infancy and childhood : the spectrum

Fibromatoses form an interesting group of tumors occupying a midposition in the spectrum of fibrous tissue neoplasms. Within the fibromatoses subgroups there exists a variety of tumors whose clinical behavior spans the range from completely benign to locally aggressive. Four case histories are presented to illustrate this point. All four patients were initially seen with asymptomatic head or neck masses. The first had complete excision with no recurrence to date. The second underwent spontaneous regression after confirmation of diagnosis with biopsy. The third case had a very locally aggressive disease requiring three surgical resections to date, while the last case had one recurrence after the initial resection. With certain exceptions, initial wide local excision offers the best possible chance for cure.

Surgical complications of Kawasaki disease

Four of 10 cases of Kawasaki disease (KD), mucocutaneous lymph node syndrome, seen from 1975 to 1980 had serious surgical complications. These included gallbladder obstruction, massive necrosis of gallbladder, liver, duodenum, jejunum, and necrosis of digits of both hands. Less serious necrosis of adrenals, testes, bladder, and stomach were seen. The operative mortality rate was 25%. The etiology is unknown. The average age was 2 yr. There was no sex preponderance. The essential lesion is an arteritis and periarteritis initially involving small arteries and later the medium and large vessels.

Stain Technology: A Combined Elastic, Fibrin and Collagen Stain

A method is described which demonstrates nuclei, elastic fibers, red blood cells, collagen and fibrin. Nuclei and elastic fibers are stained by a modified VerhoefPs elastic tissue stain which was previously developed and used in the elastic-Masson combination. Both early fibrin and red blood cells are shown by Hssamine fast yellow. Mature fibrin, some types of collagen and other cytoplasmic changes are stained by a combination of acid fuchsia, Biebrich scarlet and ponceau 2R, while old fibrin is demonstrated by the collagen stain. This method takes about 1 hr to perform and has the added advantage that several entities are clearly shown in a single slide.

Melanotic Neuroectodermal Tumor of Infancy

Melanotic neuroectodermal tumor of infancy is a specific but unusual tumor of infancy for which only sporadic cases have been reported in the literature. This paper presents a case in an infrequent site, the epididymis, and summarizes the literature on the subject.

Optimal biopsy techniques in the diagnosis of primary ciliary dyskinesia

OBJECTIVE Adequate biopsy specimens that clearly demonstrate cilia, and therefore enable the determination of the presence or absence of primary cilia dyskinesia, may be difficult to obtain. This study is an attempt to identify the optimal sampling technique to best examine respiratory tract cilia. DESIGN A prospective comparison of the four sampling techniques was carried out: nasal brushing, nasal biopsy, bronchial brushing, and tracheal biopsy. SETTING Tertiary care pediatric hospital: Childrens Hospital of Eastern Ontario. METHODS Ten consecutive patients booked for bronchoscopy and tracheal biopsy underwent all four procedures. Specimens were examined under light microscopy for an assessment of quality. RESULTS The nasal brushing and tracheal biopsy specimens provide superior quality (p = .22); however, nasal brushing is more cost efficient. Nasal biopsy samples frequently are metaplastic and therefore are inferior to nasal brushing samples (p = .02). CONCLUSION With equal efficiency demonstrated, the reduction in potential morbidity and health care costs suggests nasal brushings to be the optimal initial investigation for primary ciliary dyskinesia.

Long-term prostaglandin E1therapy in congenital heart defects

Seventeen neonates received an intravenous infusion of prostaglandin E1 for an average of 39 days (range 8 to 104). Seven (group 1) had transposition of the great arteries with no ventricular septal defect or a small one; eight (group 2) had ductus-dependent pulmonary flow (pulmonary atresia or stenosis in six and tricuspid atresia in two); and two (group 3) had aortic coarctation, one with no ventricular septal defect, the other with ventricular septal defect, isthmus hypoplasia and descending aortic flow supplied mainly by the ductus. An increase in the arterial partial pressure of oxygen (PO2) was seen in groups 1 and 2. Six patients from group 1 and two from group 2 developed heart failure; cortical hyperostosis of long bones was seen in three patients from group 1 and three from group 2; one from group 1 had refractory diarrhea. Other side effects seen at the beginning improved as the rate of infusion diminished. In group 3, the patient with complex coarctation had a decrease in blood pressure in the arms, an increase in pressure in the legs and restoration of renal function; in the patient with no ventricular septal defect, heart failure worsened during therapy. Histologic changes seen in three ductus were attributed to the closing process. When delaying surgery in selected ill infants with heart defects is deemed advantageous, long-term infusions of prostaglandin E1 are feasible.

Definitive treatment of focal nodular hyperplasia of the liver by ethanol embolization.

Focal nodular hyperplasia (FNH) is a benign, usually asymptomatic liver tumor with no predisposition to spontaneous hemorrhage. Treatment is not mandatory unless large size, compression symptoms, or fear of traumatic hemorrhage indicate otherwise. Ethanol embolotherapy offers a safe and effective alternative to surgery. FNH lends itself particularly well to embolotherapy because it is usually fed by a single end-artery with no intratumoral arteriovenous shunting or parasitic blood supply. An illustrative case is presented. Diagnosis was established by ultrasound, computed tomography (CT) scan, radionuclide scan, and arteriography and was confirmed by biopsy. Ethanol embolization resulted in shrinkage of a 10 x 14 cm tumor to a 1.5-cm calcified nodule over an 18-month period.

Tissue viral DNA is associated with chronic allograft nephropathy

Abstract:  Viral infections post‐renal transplant (Tx) impact on outcome. Increased rejection rates and decreased renal function secondary to acute CMV, EBV and HHV‐6 infections are well described. However, the clinical significance of a mere presence of these viruses on kidney tissue biopsy remains questionable. Thirty‐six kidney biopsies obtained from 17 renal transplants (five females) and two combined liver–kidney recipients (one female) were retrospectively evaluated. Age at Tx ranged from 1.7 to 17.2 yr (median = 7.4). Biopsies were performed as protocol biopsies or when renal function deteriorated, between 6 weeks and 11 yr post‐Tx (median = 1.2 yr). Immunosuppression included steroids and combination of tacrolimus/cyclosporin, mycophenolate mofetil/azathioprin and induction therapy. Fourteen patients received antiviral prophylaxis (ganciclovir/valganciclovir/acyclovir). Renal tissue was classified according to Banff ’97 criteria. Tissue CMV, EBV, HHV‐6 and HHV‐7 was analyzed by PCR. We used an estimation of GFR from average plasma Cystatin C (CysC) and slopes of 1/CysC to assess renal function. The 16/36 biopsies were positive for one virus; 5/36 biopsies were positive for two viruses. In the infected group, Banff ’97 scores for interstitial fibrosis (ci) and tubular degeneration/atrophy (ct) were significantly higher (p < 0.03 vs. the non‐infected group for both). The slope of 1/CysC, or the proportion of patients on antiviral prophylaxis, did not differ significantly between both groups. In conclusion, a significant number of kidney biopsies showed PCR positivity for CMV, EBV, HHV‐6 and HHV‐7. This was associated with a significantly higher Banff score for ci and ct; while renal function was not affected. Further controlled studies are required.