Robert J. Izant

Gastrostomy without laparotomy: A percutaneous endoscopic technique

A new technique has been developed to establish a tube feeding gastrostomy without a laparotomy. The procedure is particularly useful in high risk patients because general anesthesia is not usually required. The procedure is simple, safe, and rapid. It has been employed in 12 children (and 19 adults) with minimal morbidity and no mortality.

Paravertebral malignant rhabdoid tumor in infancy. In vitro studies of a familial tumor.

Two female siblings died within three months after presenting with paravertebral tumors in the first year of life. The pathology of the two tumors was identical and characteristic of a malignant rhabdoid tumor. There were no identifiable tumor patterns within the kindred which have been associated with any hereditary cancer or precancer syndromes. Fibroblasts were cultured from skin biopsies obtained from the second patient and both parents. Assays of growth kinetics associated with cellular transformation revealed that fibroblasts from the affected sibling can be distinguished from those of the parents and age‐matched controls by increased in vitro occurrence of tetraploidy. Such evidence suggests that increased in vitro tetraploidy occurring spontaneously in cultured fibroblasts is an expression of a cancer‐prone gene. Increased in vitro tetraploidy has previously been demonstrated in some kindreds with heritable colon cancer syndromes, and may extend our understanding of the genetic etiology of some childhood cancers. Cancer 52:290‐296, 1983.

Transverse testicular ectopia

In transverse or crossed testicular ectopia, the affected gonad lies in the same canal as the normally descended testis. During a 5-yr span, three children with this form of ectopia were seen. All presented with a symptomatic right inguinal hernia and an empty scrotum on the left side. The ages at operation were 1, 3, and 5 mo. Only the first patient was reoperated. In this child, the diagnosis of transverse testicular ectopia was made during the herniorrhaphy and the ectopic, but otherwise normal, gonad returned to the abdominal cavity. A subsequent left orchidopexy through a celiotomy was done. In the last two patients, the correct diagnosis was made preoperatively. Both gonads were of equal size and normal, occupying the same hemiscrotum. A herniorrhaphy with fixation of the ectopic gonad to the opposite hemiscrotum was done in both. All three children are otherwise normal. Cases collected from the literature are discussed. The condition should be suspected if a unilateral hernia is associated with a contralateral, nonpalpable testis and may not be as rare as formerly thought.

Duplication of the stomach

Abstract Duplications of the stomach are being reported with increasing frequency. A series of nine patients with their clinical, radiographic, pathologic and operative findings is presented. Certain consistent clinical features are indicated which should alert the clinician to consider strongly the diagnosis. Surgical intervention is recommended, though procedures will vary. Simple excision should be employed whenever possible.

Symptomatic Hepatic Disease In Cystic Fibrosis: Incidence, Course, And Outcome Of Portal Systemic Shunting

Fifteen (2.2%) of 693 patients with cystic fibrosis seen over an 18-year period developed clinical hepatic disease. In 13 patients all symptoms were secondary to portal hypertension. Ten had hypersplenism and 6 had variceal bleeding, including 3 who developed both conditions. All 5 patients who survived the initial episode of gastrointestinal bleeding underwent portal systemic shunting. A shunting procedure also was performed on 1 patients with hypersplenism but no variceal bleeding. No subsequent deterioration of intellectual function occurred in either the shunted or unshunted patients. Only 1 of the shunted patients showed progression of hepatic disease after surgery. These results suggest that portal systemic shunting is useful in the treatment of bleeding esophageal varices in cystic fibrosis. A sweat test to rule out cystic fibrosis should be included in the evaluation of any teenage or young adult patient with unexplained portal hypertension.

Congenital mesocolic (paraduodenal) hernia. Embryologic basis of repair.

Abstract Paraduodenal hernia is a misnomer and the term “congenital mesocolic hernia” preferred. The etiologic features of mesocolic hernias are explained by an abnormal rotation of the gut during embryonic development. Three types of congenital mesocolic hernias are described. A safe method for surgical reduction of a left mesocolic hernia is described and discussed.

Nonoperative management of liver injuries following blunt abdominal trauma in children

In a 4-year span, 12 patients with hepatic trauma were treated at our institution. After initial resuscitation, three underwent emergency celiotomy because of the severity of their injuries. This communication analyses the other nine. All of these had significant liver injuries and were managed nonoperatively. Hepatic injury was diagnosed and the severity defined by ultrasonography, radionuclide scintigraphy, or computed tomography (CT). These stable patients were initially admitted to the intensive care unit (ICU) and upon transfer to the ward, maintained on restricted activities. Seven of the nine children received blood transfusions. Although liver enzymes were initially elevated in each case, they returned to normal in 1 week. In-hospital and post-discharge imaging documented healing of the hepatic injuries in each case. Eight children are well at 3 months to 4 years follow-up. Only one of the nine nonoperatively managed patients died and this was secondary to severe head injuries. To date, there are no complications. Our nine children are added to the 23 nonoperatively treated pediatric hepatic injuries found in the literature.

Pathologic Childhood Aerophagia: A Recognizable Clinical Entity

Pathologic childhood aerophagia is a rarely recognized, often poorly treated entity that has remained almost undescribed in either the surgical or pediatric literature. In only 1 of 9 children the condition was recognized at presentation. The initial diagnosis of the others was Hirschsprungs disease (2), malabsorption syndrome (3), gastric outlet syndrome (1), constipation (1), and esophagitis (1). Five were hospitalized and two underwent surgical procedures. History disclosed a remarkably constant triad: previous normal stooling pattern, visible and often audible air swallowing and excessive flatus. Physical examination often demonstrated a markedly or intermittently distended and tympanitic abdomen. Abdominal musculature was thinned in children with chronic aerophagia. Roentgenographic evaluation showed massively distended loops of intestine throughout without associated air-fluid levels. There was marked compression of the diaphragm with limited excursion in some. Laboratory and malabsorption testing was normal. Treatment is limited to recognition of the problem, nasogastric decompression in severe cases and psychologic counseling when symptoms persist in the older child. The recognition of this condition may lead to a better understanding of its pathophysiology and will reduce the number of unnecessary admissions or surgical procedures.

Undifferentiated (embryonal) sarcoma of the liver with alpha-1-antitrypsin deposits: Immunohistochemical and ultrastructural studies

Periodic acid‐Schiff‐positive diastase‐resistant globules are described in an undifferentiated (embryonal) sarcoma of the liver in a 14‐year‐old boy. These globules were shown to contain alpha‐1‐antitrypsin (A‐1‐AT) protein by immunofluorescence techniques. Further immunohistochemical studies revealed albumin and gamma globulins, but no alpha‐feto protein (AFP). Electron microscopic studies showed large, electron‐dense bodies consistent with phagolysosomal structures, and distinct from that described in alpha‐1‐antitrypsin deficiency (A‐1‐ATD). These findings suggested that the globules in this tumor may represent trapping of serum proteins by the malignant cell. These observations are discussed in connection with recent studies linking the deficiency disease and epithelial hepatic tumors.

Antenatal ultrasonography: The experience in a high risk perinatal center

During a 52-month span, 14,324 ultrasonographic examinations were performed on 9,453 pregnant patients. One-hundred and fifty-one anatomical malformations were found in 122 fetuses (1.29%). Our analysis of patients referred to the perinatal center for ultrasonography indicates that the number of high risk patients has increased, and a parallel increase of neonatal surgical anomalies has resulted. An analysis of fetuses concluded that anomalies of the: gastrointestinal tract had improved care, deaths occurred due to associated anomalies or severe prematurity; genitourinary system received earlier diagnosis and treatment; central nervous system/musculoskeletal system/hydrops--no difference in management, treatment or outcome was noted; teratoma/cystic hygroma--did not effect treatment; cardiovascular system--inutero medical treatment by digitalization of the mother was possible. Paradoxically, an increase in the mortality of diaphragmatic hernia patients was noted and concluded to be secondary to the extremely early detection of this anomaly.