Franklin J. Harberg

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.

Vascular trauma in infants and children.

A 20-year retrospective evaluation of vascular trauma in infants and children was undertaken. The study included 53 cases of blunt and penetrating vascular injuries in pediatric patients. There were 36 males and 17 females ranging in age from 24 days to 14 years (average, 10 years). The most frequently encountered sites of arterial trauma were the brachial or superficial femoral artery, and of venous trauma the inferior vena cava. Any patient presenting to the Emergency Center with an injury in proximity to a major vessel, hematoma formation, audible bruit, or palpable thrill underwent prompt arteriography or immediate operative exploration of the injury sit. All patients in the series were managed operatively. There were 41 major arterial and 32 major venous injuries. No patient required a major amputation. Most injuries were repaired by primary closure or segmental resection and end-to-end anastomosis; interposition vein grafts and substitute conduits were used in four patients with more extensive injuries. A 13% operative mortality was encountered: the most frequent cause of death was intraoperative exsanguinating hemorrhage. The triad for successful management of vascular trauma in pediatric patients is: 1) a high index of suspicion, 2) performance of aggressive diagnostic studies when indicated, and 3) prompt surgical intervention.

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.

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.

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.

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.

Psoas Abscess in Children

In children, psoas abscess does not head the list in the differential diagnosis of the child who presents with a limp or lower abdominal pain. Therefore, the road to this diagnosis can be long and complicated leading to numerous studies and specialty consultations. Over a 7-year period, seven psoas abscesses have been drained surgically. All were Staphylococcal though one was mixed. In each case, the original admitting diagnosis was that of septic arthritis of the hip. In general, this diagnosis was ruled out by negative hip aspirations and bone scans. Often, the severity of symptoms led to persistent evaluation with noninvasive tests such as gallium scan, intravenous pyelogram, or barium enema. Though these tests were often suggestive, a positive ultrasound or CT scan was the key studies diagnostic enough to warrant surgical exploration and drainage. During this time period, there have been no negative explorations for psoas abscess. Upon surgical drainage, all patients improved, with subsequent recovery of hip function. The child who presents with a limp or painful hip should be considered for ultrasonography or computerized tomography once hip pathology is ruled out. We feel that the results of other tests such as gallium scan, IVP, or barium enema are not sufficiently specific to indicate surgery.

Treatment of uncomplicated meconium heus via T-tube ileostomy

There are many treatment modalities for uncomplicated meconium ileus. However, for neonates with intestinal obstruction unrelieved by nonoperative measures we advocate T-tube ileostomy with postoperative irrigation of the intestine utilizing pancreatic enzyme. Our series includes 11 infants with uncomplicated meconium ileus who underwent T-tube ileostomy 24--96 hr after birth. Of the 7 females and 4 males only 2 infants failed to pass meconium. One infant required reexploration for persistent obstruction and 1 infant, the only death encountered, had overwhelming pulmonary infection and intracranial hemorrhage. The remaining 9 infants passed meconium between the 1st and 11th postop days. The T-tube was removed by pulling it out between the 10th and the 14th postop day with the T-tube tract closing spontaneously in all patients. Our experience with T-tube ileostomy suggests that resection of dilated but viable ileum is not necessary for prompt intestinal function in the neonate. With the use of pancreatic enzyme irrigation, the tenacious meconium in these patients can be rapidly liquified and expelled per rectum or T-tube and the necessity of mechanical removal during the operative procedure avoided.

Cloacal Duplication: Genitourinary and Lower Intestinal Implications

Abstract Complete duplication of the bladder, urethra and hindgut is a rare congenital anomaly. We report a case of complete duplication of the genitourinary and lower intestinal tracts with a brief discussion of the pertinent embryogenesis and therapeutic options.