Morton M. Woolley

ESOPHAGEAL ATRESIA AND ASSOCIATED ANOMALIES

One hundred and two patients with esophageal atresia possessed 237 additional anomalies. Risk grouping of these patients was useful and showed a direct relationship between the increased incidence of anomalies, low birth weight, and nonsurvival. Fifty-seven per cent of the patients with additional anomalies possessed multiple anomalies, most frequently occurring in the gastrointestinal, cardiovascular, and musculosketetal organ systems. Fifty-five per cent of the deaths appeared to result from the coexistent severe anomaly, while the remaining 45% of the deaths appeared to occur as a result of the presence of esophageal atresia. It is our suspicion that the insult producing esophageal atresia with a high incidence of associated anomalies occurs at a more critical time in organogenesis than that event that produces the esophageal atresis alone or with one moderate anomaly.

Pancreatitis in childhood

Thirty cases of pancreatitis in children are reviewed. Diagnosis is based on the clinical findings of pancreatic inflammation, laboratory confirmation with elevated serum and/or urinary amylase values, and in some cases surgical or postmortem tissue substantiation. Four major classes of pancreatitis are defined in children: (1) traumatic; (2) systemic-disease associated; (3) drug-induced, caused by drug therapy for various life-threatening diseases, and (4) chronic pancreatitis, with or without an anatomic abnormality. If cases of traumatic pancreatitis are excluded, females outnumber males 5 to 1. A high index of suspicion is necessary to make the diagnosis, and this is very important if the mortality for pancreatitis in children is to be lowered. Awareness and consistent early aggressive intervention should increase the salvage rate.

Diagnosis of biliary atresia: relative accuracy of percutaneous liver biopsy, open liver biopsy, and operative cholangiography.

Following necropsy or a minimum of three years of clinical observation an ultimate diagnosis of either biliary atresia or neonatal hepatitis was established in 108 infants with obstructive neonatal jaundice. In this group, the diagnostic accuracy of contemporary percutaneous (needle) liver biopsy and operative (open) liver biopsy was approximately the same. One third of the cases were not correctly identified. Operative cholangiograms usually dictated a correct course of clinical management, but 20 per cent suggested the incorrect diagnosis.

Sacrococcygeal teratomas in infants and children

From 1941 through 1983, a total of 66 patients with sacrococcygeal teratoma were seen, representing 41 percent of the total of 162 patients with teratomas from all anatomic sites seen over this period. Forty-six (70 percent) of the patients with sacrococcygeal teratomas were female and 34 (52 percent) were neonates. Younger patients had a significantly better prognosis. Approximately half (48 percent) of the patients had benign tumors. Of the other 34 patients, 19 (29 percent) had frank malignancy, being either a purely malignant tumor or a tumor with malignant mixed with benign elements, and 15 (23 percent) patients had tumors containing embryonic but no malignant components. Patients with tumors containing mature adult or embryonic tissues have a good prognosis. However, malignant sacrococcygeal teratoma portends a bleak prognosis irrespective of therapy (in this series only one patient survived among 19 patients with an average survival of 16 months after diagnosis).

Hydrops of the Gallbladder Associated With Kawasaki Syndrome

Hydrops of the gallbladder is recognized as a major component of the abdominal crisis occurring in children with Kawasaki syndrome. Sixteen patients with hydrops of the gallbladder secondary to Kawasaki syndrome have been diagnosed and treated at the Childrens Hospital of Los Angeles. One patient was treated by cholecystectomy and 15 nonoperatively without untoward sequelae. Nonoperative management with serial ultrasonic evaluation and close clinical monitoring is a safe method of treatment for this entity. Pathologic and clinical data are presented and discussed. Review of diagnosis and treatment of 41 reported cases of hydrops of the gallbladder in Kawasaki syndrome from the English language literature is also presented.

Postoperative functional and manometric evaluation of patients with Hirschsprung's disease

Sixty-two patients with Hirschsprungs disease (operated on according to Duhamel, Swenson, or Soave) were evaluated for quality of defecation and studied manometrically. Follow-up was 30 years. Stools were normal in 30% to 50% of patients, while the rest had either constipation, loose, or increased frequency of stools per day. Continence was complete in 30% to 60%. Enterocolitis was seen in 12 patients preoperatively and in 20 patients postoperatively, regardless of type of operative procedure. This complication improved with age. To correct some of these problems, 18 had post pull-through internal sphincter myectomy with 50% improvement. Manometrically, 50% to 70% experienced a sensation of fullness and an urge to defecate following rectal balloon inflation. The resting external sphincter pressure was high in the majority of patients and further increased following rectal balloon inflation. The internal sphincter pressure showed a normal anorectal reflex in only 10% of patients postoperatively and did not seem to be related to clinical fecal continence. We concluded from the study that an appreciable number of patients in this select group with Hirschsprungs disease suffered from a variety of stooling disorders postoperatively. The majority of these complications were managed medically and/or socially but some were quite incapacitating.

Duodenal hematoma in infancy and childhood: changing etiology and changing treatment.

It is estimated that between 2300,000 and 4,000,OOO cases of active child abuse occur in the United States annually [I], and estimated deaths due to child abuse vary from 2,000 [2] to 50,000 annually [I]. The extreme variation in estimates is due to the difficulties inherent in proving or disproving child abuse. The child is either too young to testify on his own behalf or too frightened to do so. The abusive adult is not apt to admit to the abusive act, and those who live with him or her will assume a protective posture toward the abuser, thus denying all such allegations. Although the number of reported cases of intramural duodenal hematoma is increasing, there has been no consistent reportage of the association of active child abuse with this injury. Some of the case reports indicate “trauma of unknown etiology.” Such injuries may, in retrospect, be due to child abuse [3-81. In 1969 we reported our experience with seven patients who were treated for intramural duodenal hematoma [9]. Traumatic injury was denied by the parents of one patient, and in another the details of the trauma were not available. In retrospect, these two patients may have been injured by an abusive adult; however, at the time the manuscript was completed none of the patients was recognized as having been actively abused. During the subsequent seven years (1969-1977) eight patients have been treated for duodenal hematoma at the Childrens Hospital of Los Angeles. A retrospective review of these patients forms the basis of the present report.

Kasabach-Merritt syndrome treated by therapeutic embolization with polyvinyl alcohol

The authors describe a 6 1/2-month-old boy with a large hemangioma involving the left buttock associated with a severe consumptive coagulopathy. Cosmetic improvement and resolution of the coagulation defect followed occlusion of the nutrient vessels to the hemangioma with polyvinyl alcohol. This is the first report of successful treatment of this condition with therapeutic embolization.

Indirect calorimetry in mechanically ventilated infants and children : measurement accuracy with absence of audible airleak

Objective:To establish the effect of an audible airleak (around an endotracheal tube) on oxygen consumption (Vo2) measurements in pediatric ICU patients. Design:Prospective trial comparing Vo2 measurements before and after deflation of the endotracheal tube cuff. Setting:Pediatric ICU in a large pediatric tertiary care center. Patients:Twenty critically ill infants and children receiving mechanical ventilatory support via cuffed endotracheal tube. Interventions:Deflation of endotracheal tube cuff. Measurements and Main Results:The presence (group 1, n = 9) or absence (group 2, n = 11) of an audible airleak with the cuff deflated was confirmed by two independent observers. The percent difference in Vo2 was calculated for both groups using the following formula: ([Vo2cuff up -Vo2 cuff down]/Vo2 cuff up × 100. An audible airlelk associated with cuff deflation (group 1) caused a significant (p = .0012) reduction of Vo2 by 45.6% (mean difference in Vo2 = 45.6%). In contrast, with no audible airleak after cuff deflation (group 2), only minimal changes in Vo2 (mean difference in Vo2 = −0.4%) were observed. Conclusions:These data suggest that if no audible airleak is detected, Vo2 determined by indirect calorimetry may be reliably measured in infants and children with a noncuffed endotracheal tube.

Esophageal atresia and tracheoesophageal fistula: Management of the uncommon types

Summary During the past six years, among 108 hospital admissions for esophageal atresia, 31 (29 per cent) have had one of the four unusual varieties, i.e., Type A, B, D, or E (“H”). In five patients with an initial diagnosis of atresia without fistula (Type A), primary esophageal anastomosis was performed following a planned delay of from four to ten weeks. This was accompanied in three infants by an elongation procedure to lengthen the proximal esophageal segment. This regimen of delayed primary anastomosis, following proximal pouch elongation, has replaced colon interposition in the management of Type A atresia in this center; and is probably the procedure of choice in premature infants with Type C atresia. Recognition of the upper tracheoesophageal fistula has proved difficult in five infants admitted with double fistula (Type D); and anastomotic leak has resulted in a high morbidity and mortality. When lying high in the neck, division of the upper fistula through a cervical incision as a separate procedure may be preferable to complete repair via thoracotomy. Major diagnostic problems are presented by the tracheoesophageal fistula without atresia, Type E (“H”). When this anomaly is seen in newborn infants with respiratory distress, the diagnosis is most readily established by the use of contrast studies of the upper esophagus. Reduced motility in the distal esophagus is a suggestive sign. In older infants and children presenting with intestinal (or abdominal) symptoms, in contrast, endoscopic studies are most helpful. All such fistulae have been divided through cervical incisions.