Joseph S. Janik

Recurrent inflammatory pseudotumors in children

BACKGROUND/PURPOSE pulmonary (PPT) and extrapulmonary pseudotumors (EPPT) are uncommon benign tumors, which, in general, do not recur after complete resection. Recurrence rates for both types of pseudotumors are undocumented in a large population of children, and the salient features of potential recurrences are unspecified. METHODS This is a report of 15 children with PPT and EPPT; 3 children had a recurrence. These pseudotumors recurred despite adequate primary resection of all gross disease at first presentation. The literature was reviewed to determine rate of recurrence for PPT and EPPT and also to document features common to recurrent pseudotumors. RESULTS Overall recurrence rate for pseudotumors was 14%. PPT and EPPT, which were not confined to a single organ, had a high chance of recurrence (46% and 30%, respectively) compared with PPT and EPPT, which were confined to a single organ (1.5% and 8%, respectively). Recurrences have appeared between 3 months and 7 years. Intraabdominal EPPT accounts for more than 75% of the EPPT recurrences. CONCLUSIONS PPT and EPPT recur more frequently than anticipated. All pseudotumors, which on initial presentation extend beyond the confines of a single organ, have a high chance of recurrence despite what appears to be adequate resection. Children with pseudotumors that extend beyond a single organ, require frequent postoperative evaluation for recurrence and may be candidates for chemotherapy or radiotherapy at the time of initial resection.

Nonsurgical management of appendiceal mass in late presenting children.

Thirty-seven late presenting children with appendiceal mass were treated between 1965 and 1975 with i.v. fluids, alimentation according to the state of gastrointestinal function, and no antibiotics. They ranged in age from 18 mo to 16 yr and all had had symptoms for at least 5 days (mean 8.7), an abnormal WBC (mean 19.9), and a fixed palpable mass without rebound tenderness. Children were discharged when clinical findings resolved. All returned for interval appendectomy. Eighty-one percent (31 children) had clinical improvement within 5-22 days (mean 10.9). Nineteen percent (7 children) had recurrence or worsening of symptoms and required abscess drainage within 2-10 days after observation began. No child in either group received antibiotics nor did any die. Only one recurrence of symptoms after discharge was recorded before interval appendectomy. Pathologic specimens revealed fibrosis in 46%, subacute inflammation in 35%, and acute inflammation in 19%. Nonoperative management of the appendiceal mass without antibiotics in children is safe as long as diligent observation is maintained. Interval appendectomy can be performed as late as 20 wk after symptom resolution or drainage, however, over 50% of the interval appendectomy specimens reveal acute and subacute inflammation.

The vulnerability of the vas deferens (II): The case against routine bilateral inguinal exploration

The results of operative manipulation of the vas deferens have unknown results. In order to document early and late structural changes in the vas after simple operative manipulations, the vasa deferentia of 120 Wistar rats were subjected to 4 different manipulations. Subsequently, they were removed at intervals (up to 6 mo) and examined after appropriate staining. Finger or vascular clamp grasping resulted in mild inflammation at 6 wk, which resolved thereafter. Forceps grasping disrupted the muscle layers in 30%-40% of the vasa. Hemostat clamping damaged muscle layers in 100% of the vasa and resulted in abscess formation in 30%. Healing in forceps grasped and hemostat clamped specimens was negligible and haphazard with minimal evidence of normal collagen or elastin at 6 mo. All differences were significant (p less than 0.05). These results suggest that operative manipulations of the vas should be minimized and limited to digital maneuvers. Damage from other manipulations is appreciable, long-term, and only minimally repaired at 6 mo.

An assessment of the surgical treatment of adhesive small bowel obstruction in infants and children

At The Hospital for Sick Children, Toronto, Canada, adhesive small bowel obstruction (SBO) ranks seventh as a cause of pediatric bowel obstruction. Between January 1968 and December 1979, 131 infants and children had adhesive SBO proven at laparotomy or autopsy: 123 had 1; 7 had 16 adhesiotomies; 1 died without surgery; 100 had 1 prior operation; and 31 had multiple operations. Over 80% of the SBOs developed within 2 yr of the prior operations. Appendectomy and subtotal colectomy were the most common prior operation. Postoperative morbidity occurred in 29 children; 20 were observed longer than 24 hr before laparotomy. The rate of wound infection ranged from 4% to 50%; it was lowest for those children who had lysis of adhesions only, and highest for those who had lysis and decompressive enterotomy or perforation repair. Results indicate that delaying adhesiotomy and entering the GI tract during adhesiotomy are associated with increased morbidity (p less than 0.01), and therefore should be avoided. Prophylactic antibiotics may have a protective role during anterolysis.

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 infants 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.

Peritoneal drainage under local anesthesia for necrotizing enterocolitis (NEC) perforation: A second look

Between 1974 and 1979, 15 extremely ill neonates with necrotizing enterocolitis (NEC) were initially treated with peritoneal drainage under local anesthesia for intestinal perforation. They weighed 600 to 3040 g with half less than 1000 g. Most had other serious illnesses (RDS, PDA, jaundice, CNS abnormalities). There were no immediate complications such as hemorrhage or bowel evisceration from the local drainage procedures. Seven of the 15 (46%) survived. Three (20%) died because of unrelated problems (CNS, liver failure) with an intact gastrointestinal tract, while another 8 (34%) died from intestinal sepsis. Seven (87%) of the neonates weighing less than 1000 g had an adequately functioning GI tract after this drainage procedure. Half of the neonates requiring additional surgery within 24 hr of initial peritoneal drainage survived and half of the neonates requiring subsequent surgery survived. Five of 15 infants developed strictures one died before excision. This technique is contrary to standard practice and was employed in less than 10% of the neonates with NEC treated at our institution. These results indicate that this method is effective in possibly temporizing the very ill neonate with NEC. An added bonus, however, is that 40% of the neonates treated in this fashion had complete resolution of their disease without residual scarring of the gastrointestinal tract requiring further surgery. It is our continued conclusion that this form of peritoneal drainage under local anesthesia is warranted in certain carefully selected instances.

Perioperative management of children with sickle hemoglobinopathy

During the 11 years between July, 1967 and June, 1978, 35 children with a major hemoglobinopathy (SS, SC, S-thalassemia) underwent 46 operations without any mortality or unusual morbidity. All of these children were prepared for surgery by preoperative transfusion of 15-20 cc/kg packed erythrocytes and a hematocrit of at least 36%. In urgent and emergent situations, the operation was performed as soon as the transfusion was completed. For elective patients, the operation was the following morning. The ease of application and the absence of morbidity and mortality suggest that this method of preoperative transfusion preparation of children with a major hemoglobinopathy is more desirable than a two-volume exchange or transfusions beginning 10-15 days prior to surgery.

A standard of comparison for acute surgical necrotizing enterocolitis

We reviewed 187 cases of documented neonatal necrotizing enterocolitis (NEC) from 1976 to 1988. Of these patients, 111 infants underwent celiotomy for acute surgical complications. The following protocol of operative indications was employed: pneumoperitoneum, localized mass, abdominal wall erythema, portal venous air, and clinical deterioration, singly or in any combination. Clinical deterioration was defined as falling platelet count, rising or falling white blood cell count, left shift in the myeloid series, persistently or progressively low pH, and increasing frequency of apnea or bradycardia. Overall mortality was 15% (28 of 187). For the patients who underwent celiotomy, all had histologic confirmation of NEC. Ninety-five had localized disease, and 16 had diffuse disease. All of the former had resection and diverting enterostomy with 85 (89.5%) surviving; none with diffuse disease survived, P less than 0.0001. Forty-one infants with NEC weighed less than 1,000 g; 25 underwent surgery and 15 (60%) survived. Fifty-one of the 159 surviving neonates (32%) developed intestinal strictures. All neonates with strictures have had resection and successful reconstruction of their gastrointestinal tract. These indications and surgical principles resulted in a high degree of diagnostic accuracy and a low degree of surgical mortality.

Occult coin perforation of the esophagus

Only a small number of ingested foreign bodies perforate the esophagus and even a smaller fraction migrate extraluminally with no symptoms. Both of these events are even rarer after coin ingestion. Between 1972 and 1984, three children (16 months to 5 years), who had unabating upper respiratory symptoms, were found to have ingested coins. In only one child could the time interval between ingestion and appearance of symptoms (3 years) be established, and in this child the diagnosis was delayed because of failure to x-ray the chest after an ingestion episode. Surgical evaluation included chest/neck films: tracheal and esophageal shadows were separated by the object and a soft tissue mass; esphagogram: deviation of the esophagus, irregularity of the lumen, and no leak or tracheoesophageal fistula (TEF) and esophagoscopy: mucosa was intact with no direct visualization of the coin. Treatment consisted of exploration: cervical, one; thoracic, two; localization: coin in extraluminal granulomatous soft tissue; removal: without resection of the soft tissue mass or esophagus, and drainage: penrose, one; chest tube, two. There was no morbidity or mortality from 1 to 13 years later. Though generally harmless, ingested coins are capable of penetrating the esophagus. Sporadic literature reports confirm that the clinical findings and operative results are typical of this seemingly intermediate stage between perforation with mediastinitis and perforation with TEF.

Ileal atresia with total colonic aganglionosis

This is the 17th report of the case of an infant who had ileal atresia associated with Hirschsprungs disease, and the second with ganglion cells distal to the atresia. Experience suggests that Hirschsprungs disease should be suspected in all forms of bowel atresia.