William J. Pokorny

Morgagni hernias during infancy: Presentation and associated anomalies

Morgagni hernias during infancy are associated with significant respiratory symptoms as well as other congenital anomalies, particularly congenital heart disease. During the past 6 years we have had the opportunity to treat five infants less than 1 year of age with Morgagni hernias. A review of the literature revealed an additional seventeen detailed case reports of infants with Morgagni hernias. Each of our patients was symptomatic as were all but two of those previously reported. These patients presented with tachypnea, dyspnea, and cyanosis which was very similar to the presentation of Bochdalek hernias seen during infancy. Thirteen of 16 patients in whom detailed descriptions of associated anomalies were given including four of our own patients, had significant congenital anomalies. Nine involved the heart, including three infants with dextracardia, three with ventricular septal defects, and two with anomalous pulmonary venous return. Five infants were retarded, including three with Trisomy 21. Two of the previously reported patients had large omphaloceles and other stigmata of Cantrells syndrome. All but one of the patients reported, including ours, had a hernia sac. The liver, colon, and small bowel were most commonly found in the hernia sac; however, the presence of the stomach and spleen have been reported. While both the transabdominal and transthoracic approaches to Morgagni hernias have been advocated and, indeed, repair of the defect can be accomplished through both approaches, the transthoracic approach allows better exposure of incarcerated viscera, particularly the frequently found anomalous left lobe of the liver.

Acute appendicitis in preschool age children

Appendicitis in preschool children is not uncommon. A duration of greater than 40 hours usually implied perforation. The most common findings were abdominal pain, tenderness, temperature elevation and vomiting. In the presence of perforation or abscess formation, appendectomy followed by copious wound irrigation, abscess drainage and delayed secondary wound closure is the procedure of choice.

Current management of laryngeal and laryngotracheoesophageal clefts

Laryngeal and laryngotracheoesophageal clefts (L-LTEC) are uncommon anomalies in neonates that cause significant morbidity secondary to aspiration, pneumonia, and respiratory distress. Other anomalies of development, such as esophageal atresia and tracheoesophageal fistula (EA-TEF), are observed in 20% of patients with L-LTEC and often confuse the radiographic and clinical picture. Repair of L-LTEC depends on the length and location of the cleft, associated anomalies, and concurrent systemic illness. For type I L-LTEC, endoscopic repair occasionally is possible with microsurgical instrumentation. With types II to IV L-LTEC, an open approach must be used. Tracheotomy is a universal requirement, often for extended periods of time. Reported here is our experience with four cases of L-LTEC managed over the past 7 years at Texas Childrens Hospital.

Necrotizing enterocolitis: Incidence, operative care, and outcome

At Jefferson Davis Hospital, the incidence of necrotizing enterocolitis (NEC) was three per 1,000 live births, and 30 per 1,000 low birth weight births. The occurrence of NEC was sporadic and no epidemics occurred. NEC occurred most frequently in infants weighing between 750 and 1,500 g, and the smaller infant with NEC was more likely to require surgical intervention. As the survival of small birth weight infants improved over the 4 years of the study, the patient population developing NEC became smaller. The age at operation also increased in the period between 1982 and 1984. Those infants who developed NEC after 30 days of age typically had more extensive disease and a less favorable prognosis. In this series, 31% of infants with acute NEC required surgical intervention. An additional 11% of those infants treated nonoperatively eventually required surgical intervention for late sequelae of NEC. The overall survival of infants with NEC was 75%. While the survival of all infants operated for NEC was 68%, the survival for those with the acute syndrome was 63% and those operated on for late sequelae was 87%. Primary anastomosis in selected patients did not adversely affect mortality and simplified the postoperative care of these infants with severe complications. Indeed, enterostomy closure in an infant who had previously had NEC was an extensive procedure that carried significant risk. Our results indicated that the trained pediatric surgeon could predict at the operating table which infants could safely undergo resection and anastomosis and that, with experience, the percent undergoing primary anastomosis increased to approximately 50%.

Gastroschisis complicated by intestinal atresia

Five of 22 infants operated upon in the last 4 yr for gastroschisis had an associated loss of intestinal continuity. Four of the infants had an intestinal atresia and one had a gangrenous segment of ileum. Each had primary closure of the gastroschisis and decompression of the proximal intestine. Four had cutaneous enterostomy and one had long tube intubation. Four infants were returned to the operating room between 2 and 4 wk of age and their intestinal continuity was established. At the second operation the intestine, which had presented as a matted mass at birth, appeared normal with resolution of the serosal edema and few adhesions. The length of the intestine that initially had appeared shortened may be more accurately evaluated and is usually considerably greater than was appreciated at birth. If distal atresias are present, they will become apparent. A primary anastomosis can be carried out using the principles of repair for an isolated intestinal atresia in the newborn. All five of these infants have survived.

Thoracic trauma in children.

Thirty-seven children were admitted for the treatment of thoracic injuries during a 15-year period. Two thirds suffered blunt trauma. Of these children with blunt injuries, three quarters were male, with a median age of 6 years. Half of all victims of blunt trauma were between the ages of 4 and 6 years, and most were involved in motor vehicle crashes. Thirteen children (35%) were victims of penetrating trauma. Sixty-nine percent were male. The median age was 13 years, with 9 of 13 patients in their teenage years. Injuries were evenly distributed between stab and gunshot wounds. Eight of the 37 patients (22%) required thoracotomy. Fourteen had associated intra-abdominal injuries. Twelve of these patients had injuries that required exploratory laparotomy and two were managed expectantly. There were 27 patients with lung injuries, three quarters of whom were managed with tube thoracostomy alone. Seven children suffered injuries that resulted in their deaths. Six had been struck by a motor vehicle. Four of these six died of closed head injuries. The overall mortality of patients with blunt trauma was 25%.

Cholelithiasis and cholecystitis in childhood

In this review of 50 children with gallbladder disease in Houston, Texas, there were an equal number of patients with and without hemolytic disease and a slight predominance of males in both groups. Nearly all had been symptomatic for a prolonged period before diagnosis and cholecystectomy. Common early diagnoses were hemolytic crisis and appendicitis. Ultrasonography has become a very accurate tool for the preoperative evaluation of these children. Once the diagnosis of cholelithiasis is established, elective cholecystectomy is the treatment of choice.

A totally implanted venous access system used in pediatric patients with cancer.

A safe and effective method of venous access is important in the care and treatment of patients with malignancies. A recently available totally implantable venous access system offers advantages over traditional central vein catheters (Broviac and Hickman, Houston). We report our experience with the implanted venous access system used in 31 pediatric patients with malignancies. The mean age of the patients was 7 years (range, 6 months to 17 years), and the mean indwelling time of the catheters was 232 days (range, 14 to 607 days; total patient days, 7,198). The catheters were used to administer chemotherapy, drugs, blood products, and parenteral nutrition, as well as to draw blood. Clotting occurred in the catheters on four occasions, requiring removal of two catheters. Fever occurred in eight patients; one developed a local infection at the site of implantation and four developed bacteremia. Our use of the implanted venous access system in children resulted in a lower rate of infection compared with that when the traditional Broviac and Hickman catheters were used, and simplified patient management.

Late presentations of midgut malrotation in children

Malrotation of the intestine may become symptomatic in the older child and may manifest itself in atypical presentations. Older children may present with symptoms of less than 72 hours duration which are typical of acute duodenal obstruction. More frequently, however, the older child with malrotation will present with chronic abdominal pain with or without vomiting or chronic diarrhea. The diagnosis of malrotation should be considered in any child with intermittent abdominal pain, vomiting, diarrhea, or malabsorption. Surgical intervention is curative and should be implemented as soon as possible after the diagnosis is made.

Experience with a total muscle-sparing approach for thoracotomies in neonates, infants, and children

We have adopted a total muscle-sparing technique for thoracotomies in infants and children. The technique preserves the latissimus dorsi and serratus anterior muscles and provides excellent exposure for most thoracic and mediastinal operations. Thirty-two such procedures have been performed with only one complication, a small wound seroma. Use of this technique may not only decrease postoperative pain and splinting, but may decrease the incidence of scoliosis and muscle dysfunction found in children having undergone thoracotomies as infants.