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Biomedical subjects

K Mancer

Publications and source records attributed to K Mancer.

At least 37 records · Page 2Linked to original sources

Congenital hepatic fibrosis with saccular dilatation of intrahepatic bile ducts and infantile polycystic kidneys.

A case report is presented of a child who died in the neonatal period and who had the sonographic findings of multiple, tubular, fluid-filled spaces in the liver characteristic of Caroli's disease. There was an echodense renal cortex and pyramids. Necropsy findings were consistent with Caroli's disease but the liver also showed evidence of congenital hepatic fibrosis and there was infantile type polycystic disease of the kidneys.

Bile Duct Diseases↗

Malignant sacrococcygeal teratoma--endodermal sinus, yolk sac tumor--in infants and children: a 32-year review.

Fifteen cases of malignant sacrococcygeal teratoma were treated at our hospital over the last 32 years. These included five cases of endodermal sinus (yolk sac) tumor that arose in otherwise benign or immature teratoma, and ten cases of pure endodermal sinus (yolk sac) tumor. Most were females, and only one was a neonate. The presence of symptoms almost always indicated local extension or distant metastases. According to Altman's classification, all but two of these malignant tumors were Types III and IV. The resectability was low, but with aggressive radiotherapy and chemotherapy, second look operations in five have been possible with increasing optimism for survival. Two of five patients with secondary resections are alive and tumor-free almost two years after therapy was discontinued. So far, only the newborn is a long-term (14 years) cure.

Child, Preschool↗

Ileocaecal atresia.

Meconium ileus is one of the commonest causes of distal small bowel obstruction in neonates. Within the last six years at our hospital two newborns presented with identical signs and symptoms of meconium ileus. Abdominal x-rays showed distal small bowel obstruction with ground glass opacity in the right lower quadrant. Gastrografin enema in both cases demonstrated a microcolon, but there was no reflux of this contrast material into the terminal ileum. Laparotomy in both babies revealed atresia of the ileocaecal valve with dilatation of the terminal ileum loops which contained normal meconium. Ileocolic resection was carried out in both neonates. Sweat chlorides and eventual recovery were normal. Pathology in one of the specimens showed muscularis passing across the atretic area with a telescoped pattern of the muscularis propria suggesting intussusception in utero. Although the presentation of a newborn may be that of meconium ileus, the fact that the Gastrografin enema is unable to enter the terminal ileum in a retrograde fashion should be an indication for immediate laparotomy rather than persisting in this mode of treatment. It is obvious that occasionally the radiological diagnosis of meconium ileus is incorrect.

Cecum↗

Thirty years of experience with pediatric primary malignant liver tumors.

Since 1950, 48 infants and children from 10 weeks to 16 years of age presented with primary hepatic malignancy. Signs and symptoms ranged from asymptomatic to those of malignant disease. All patients had a palpable abdominal mass. Jaundice was seen in five patients, four of whom had preexisting cirrhosis. Three male children had evidence of precocious puberty. Whereas liver function tests were usually normal, alpha-fetoprotein levels, when elevated, proved useful diagnostically and as a tumor marker in follow-up. Hepatic angiography and computed tomography (CT) scans have provided the most valuable preoperative assessment of hepatic architecture. Sixteen infants and children underwent resection for cure. Eleven of these patients are alive and disease free 6 months to 23 years later. Six additional patients had incomplete resection with subsequent radiotherapy and/or chemotherapy; only one such patient is disease-free past 3 years. Twenty-six tumors could only be biopsied; most of these patients died within 12 months regardless of what treatment they received. The histology was hepatoblastoma in 39 patients, hepatocellular carcinoma in 4, fibrolamellar carcinoma in 4, and malignant mesenchymal tumor (mesenchymoma) in 1. The patients with hepatocellular carcinoma and mesenchymoma all died. Three of four patients with fibrolamellar carcinoma are alive and disease-free following resection up to 3 years; this histology seems favorable. The other survivors had hepatoblastoma. The role of adjunctive chemotherapy and/or radiotherapy has not yet been determined.

Adolescent↗

The problem of recurrent thyroglossal duct remnants.

The removal of a thyroglossal duct or sinus is a common pediatric surgical operation. It has been taught that if the duct remnant is removed down to and including the middle third of the hyoid bone, the "correct" operation has been done. The three authors have an experience totaling over 60 years in pediatric surgery during which time they operated on 270 thyroglossal duct cysts and sinuses. In this group were 27 recurrences of which three belonged to the authors. Most recurrent thyroglossal duct remnants were found to have the middle third of the hyoid bone still in place, and with its removal the patients were cured. However, during this period of time, there were thyroglossal duct cysts and sinuses which recurred in spite of the "correct" surgical procedure having been done. These patients had all been operated on between three and five times thus creating a difficult and unusual problem. The solution to these recurrent thyroglossal duct cysts and sinuses were deeper excisions to remove residual tracts deep to the previously removed hyoid bone, and/or wider excisions to excise previously missed respiratory epithelial remnants which deviated laterally from the midline. The latter tissue was found to be a more centrally directed branchial cleft sinus.

Adolescent↗

Solitary intestinal fibromatosis with perinatal bowel obstruction.

Three newborn male infants presented with bowel obstruction in the first day of life and at surgery were found to have solitary tumors involving the small or large intestine. Histologic examination in each case showed a transmural infiltrative spindle cell lesion having the morphologic features of fibromatosis. Ultrastructural studies in one case revealed the tumor to be composed of myofibroblasts. The patients are all alive and well without recurrences 26 months to 10 years after surgery. Only 3 previous cases of solitary congenital fibromatosis of the intestinal tract have been reported. Some of the other congenital spindle cell tumors cited in the literature under various names have morphologic and biologic similarities to our cases and may in fact be examples of congenital fibromatosis. The appropriate treatment of this unusual lesion is local excision, and the prognosis is excellent.

Colonic Neoplasms↗

Yolk sac tumor of the bladder.

A 1-year-old white boy presented with hematuria and was found to have a large polypoid bladder mass. Light and electron microscopic, and immunohistochemical diagnosis was a yolk sac (endodermal sinus) tumor. This is the first case of a primary yolk sac tumor of the bladder to be described in the English literature.

Humans↗

The temporary stent technique: an easier method of micro-venous anastomosis.

The microvascular repair of small veins is technically difficult. A new method using a temporary "stent" has been studied experimentally in microvenous anastomoses of the femoral veins in twenty-four rats. The technique is described in detail. The results were evaluated by gross observation and by histological section. Improved patency rates were demonstrated and the advantages of the technique are discussed. The technique could be very useful in the anastomoses of small, fragile veins, particularly by the less experienced microsurgeon.

Animals↗

Lipoblastoma in infants and children.

Lipoblastoma and lipoblastomatosis are rare benign tumors of embryonal fat with a tendency to local invasion but not to metastasize. To date, there have been 60 cases described in the international literature. There has never been a report in a child older than 8 yr. The main clinical characteristics are the presentation, usually below 3 yr of age, the rapid growth of the mass and the peripheral location, mainly in the extremities. Although CT scan may show a fatty tumor, there is no single test to make the differential diagnosis (which includes benign lipoma, liposarcoma and myxoliposarcoma) and the treatment should be based on clinical findings. We have treated four patients with this condition. Two were intrathoracic, one was intraperitoneal, and one was a tumor of the upper arm. All presented as rapidly-growing tumors and were clinically assumed to be malignant. We recommend complete but conservative excision of the tumor; there are reported recurrences after inadequate excision. This is a benign tumor and radical cancer surgery should be avoided.

Abdominal Neoplasms↗

Tracheoplasty--a new operation for complete congenital tracheal stenosis.

This is a report of a case of complete congenital tracheal stenosis confirmed by tracheobronchogram. The stenosis also involved the origin of the right main bronchus. The membranous portion of the trachea was absent. It was repaired through a sternotomy and right thoracotomy aided by partial cardiopulmonary bypass. The posterior trachea was opened from larynx to carina and on into the right main bronchus, and each posterior tracheal edge was sewn to the anterior wall of the esophagus with a running Prolene suture. Three months after repair bronchoscopy showed that the new membranous trachea was epithelialized and the entire airway was of good caliber; the only problem was a diffuse tracheomalacia. He died in his seventh postoperative month after a major airway complication due to tracheotomy, which occurred after an elective bronchoscopy. It is obvious that this operation is technically feasible. It was hoped that his airway would become sufficiently stable to allow the tracheotomy tube to be removed at some time in the future.

Bronchi↗

Thymic changes in histiocytosis.

In order to arrive at a hypothesis of the pathogenesis of thymic changes in histiocytosis, the thymus glands from thirty-two children with histiocytosis were studied. In general, the changes were a severe loss of cortical and medullary structures rather than stress type changes in which only the cortical structure changes. The thymic changes were then classified into three main groups: severe dysplasia equivalent to that seen in congenital immune deficiency, severe nonspecific involution, and a third group designated as a dysmorphic type which resembled those with dysplasia, but, in addition, showed smaller lobules and interstitial histiocytic infiltration. The thymic changes correlated with the type of histiocytic disease. For the purposes of this study, the disease was categorized as Type I or Type II, as previously described. Dysplastic changes were only seen in those patients with Type I histiocytic disease, while involution occurred in patients with Type II histiocytic disease. Dysmorphic change was apparent in both, but it had a much higher incidence in Type II.

Child↗

Intestinal stricture after necrotizing enterocolitis.

Between 1974 and 1979 inclusive, 20 of 175 neonates with previous necrotizing enterocolitis (NEC) developed obstruction from intestinal stricture 1-20 mo after the diagnosis of NEC. Seven were found in the small bowel, 19 in the colon. Treatment included proximal decompressive enterostomy with subsequent resection in 6 and primary resection in 11. Two died during attempts to restore GI continuity and one died from cardiac disease before any GI surgery. Morbidity occurred in six. Various stages of wound healing from acute inflammation to dense fibrosis were found in pathologic specimens. Fibrosis was most marked in the submucosa and most consistently found in specimens resected 3 mo after the acute episode of NEC. Intestinal stricture develops in 6%-33% of the infants with previous NEC. Eighty percent of these strictures are colonic, 30% are left sided, and 15% are multiple. Multiple lesions are exclusively colonic. Histology varies with age of lesion. The predominant feature of stricture is submucosal fibrosis. Strictures less than 3 mo old still have acute inflammatory disease, older strictures are mature and fibrotic. The best diagnostic tools are a high index of suspicion and a barium enema. An intestinal stricture should be suspected in any infant with a past history of NEC and: (1) symptoms of obstruction or failure to thrive; (2) previous exteriorization or proximal diversion of acute or chronic NEC lesions; or (3) peritoneal drainage under local anesthesia for NEC perforation. Treatment should be tailored to the infant's condition and the time elapsed since the acute NEC episode. Resection of the stricture must be complete otherwise recurrence or leak is unlikely. Intestinal diversion with a 3-6-mo delay before reconstruction appears safer in the child with little margin for error. Earlier restoration of blood flow and cessation of intestinal function during the acute episode of NEC might reduce the incidence of stricture.

Colonic Diseases↗