Successful management of an infant with a giant hemangioma of the retroperitoneum and Kasabach-Merritt syndrome with alpha-interferon.

Giant vascular neoplasms in neonates generally require aggressive medical or surgical therapy for treatment of complications. Steroids, chemotherapy, embolization, radiation, and surgery have all been used with short-term beneficial and sometimes unknown long-term side effects. A new modality of treatment, alpha-interferon, has recently been described. The majority of hemangiomas in children involute by 8 years of age. Occasionally, hemangiomas can endanger vital structures and are associated with a consumption coagulopathy and thrombocytopenia (Kasabach-Merritt Syndrome). These hemangiomas occasionally do not respond to steroids, radiation therapy, cytotoxic drugs, or embolization. The mortality rates approach 50% in nonresponders. Alpha-interferon has been used in these children with life-threatening complications of hemangiomas with relief of symptoms. This case illustrates the potential use of alpha-interferon in the management of giant hemangiomas in children. This emerging form of biological therapy avoids the risks of radiation therapy, embolization, and surgery with only minimal side effects.

Proteus syndrome: diagnosis and surgical management.

The congenital hamartomatous syndrome known as the "Proteus syndrome" (PS) manifests itself with regional giantism, lymphangiomatous hamartomas, and other variable features. Review of the medical literature shows approximately 50 cases reported to date. Since this syndrome has only recently been defined, the management of these patients has been speculative and often children are not treated. This report summarizes the characteristics of the PS and presents eight additional cases. All of the eight children had regional giantism with macrodactyly and skeletal hypertrophy. Asymmetrical leg length was pronounced in five children. All children had large lymphangiomas, the majority of which involved the trunk. Three of the children have been followed through adolescence, two into late childhood, and three into early childhood. In contrast to previous reports, we believe that early surgical reconstruction is necessary to reduce deformities due to the giantism and the large hamartomas. During extensive excisions, residual abnormal tissue is often needed in the reconstruction and it is not unusual for postoperative leakage of lymph to be prolonged. All of the children in this series have benefited both physically and emotionally from extensive surgical reconstruction.

Recent experience with diaphragmatic hernia and ECMO.

In the past 4 years at the Medical College of Georgia, a total of 74 patients underwent extracorporeal membrane oxygenation (ECMO) with 62 (84%) survivors. Forty-seven of these infants had meconium aspiration syndrome and 11 had diaphragmatic hernia. The use of ECMO, when indicated, after reduction and repair of the diaphragmatic hernia, results in normal oxygen delivery, allows time for pulmonary maturation, and increases survival. A total of 27 referrals for diaphragmatic hernia were studied. Six infants had surgical repair and did not require ECMO. Eleven patients, after surgical repair, were treated with ECMO and seven survived. More importantly 10 patients died before the use of ECMO. Six infants died either before or during transport from referring hospitals and four died while in the delivery room or neonatal unit before ECMO. Of these 10 infants, eight were potential candidates for ECMO. Thirteen of the twenty-seven (48%) infants survived. Seven of eleven (64%) infants who received the benefit of ECMO survived. Eight infants who met the criteria for ECMO died before its use. Had ECMO been used in those eight infants, our data suggests that at least four may have survived. The data from this report support the concept that infants undergoing surgical repair of diaphragmatic hernia, when ECMO is not available, should be referred to an ECMO center in the early postoperative period. Furthermore infants with prenatal diagnosis of diaphragmatic hernia should be delivered at a center where surgical as well as ECMO expertise are available.

Childhood farm injury: the role of the physician in prevention.

Farm-related trauma occurs in more than 25,000 children per year with almost 300 patients dying from these preventable injuries. The incidence of associated farm-machinery injuries and fatalities is increasing at an alarming rate with the majority occurring in young boys during the summer months. Between 1979-1985, 234 patients with traumatic injuries were admitted to our Pediatric Surgery Service. Seven of these, six boys and one girl, age ranges from 2-15 years were treated for severe farm-related injuries. Modified injury severity score was calculated with an average score of 31.5 (range 9-66). Four patients sustained or required amputation of a major extremity(ies) as a result of the injury. Two patients had multiple long bone fractures with associated neurologic or abdominal injury. One patient exsanguinated from massive liver lacerations. Five of the six surviving patients are disabled to varying degrees because of their injuries. This report provides support for the establishment of federal safety standards for farm-related equipment. Guidelines for prevention are outlined. We believe that the child physician is obliged to encourage education programs in farming communities on safety measures and further should endorse the application for farms.

Esophageal stricture secondary to Stevens-Johnson syndrome.

We report a 7-year-old male with ampicillin-induced Stevens-Johnson syndrome with subsequent extensive skin, conjunctival, oropharyngeal, and laryngeal involvement. Over the next 5 months, he developed complete blindness and dysphagia. A barium swallow revealed absence of both right and left pyriform sinus, and a stricture involving the entire esophagus. Retrograde dilatations, complicated by malignant hyperthermia, have subsequently allowed for the difficult progression from an eight to a 40 French bougie. Eighteen months since the diagnosis of esophageal stricture, he has a normal appearing esophagus and is swallowing without difficulty.

Primary repair of esophageal atresia: how long a gap?

Primary esophagoesophagostomy is the treatment of choice for repair of esophageal atresia, particularly the more common type C atresia. Debate continues, however, regarding repair of the type A, or long-gap esophageal atresia. Since the pioneering work of Livaditis, Howard and Myers, the frequency of primary repair of long-gap atresia has increased. Interposition grafting, however, remains as the treatment for gaps longer than 6 cm or with absence of a distal intrathoracic esophageal segment. We report a case of primary esophagoesophagostomy with proximal esophagomyotomy in a 1,900-g infant with an absent distal intrathoracic esophageal segment and an eight vertebral body gap. Utilizing daily bougienage of the proximal and eventually the distal segment, a 1-cm gap was present at 6 months of age. With intraoperative fluoroscopy, the distal intra-abdominal esophageal segment was manipulated thru the esophageal hiatus. Thru an extrapleural approach with a proximal esophagomyotomy, a delayed primary anastomosis was successfully performed. She is currently 3 years old and has a normal barium swallow without stricture or gastroesophageal reflux.

Gastroschisis.

Twenty-five newborn infants were treated for gastroschisis between 1971 and 1979 at the Eugene Talmadge Memorial Hospital. Primary closure of the defect was accomplished in 17 patients and eight required staged procedures with Silon. The overall mortality rate was 32 per cent. The major causes of death were related to the status of the bowel at the initial procedure as well as weight and body temperature on admission. Respiratory function was not influenced by the type of repair. Since gut dysfunction in the postoperative period is likely to be prolonged, total parenteral nutrition continues to play a major role in survival.

Use of preoperative chemotherapy and radiation therapy in patients with Wilm's tumors.

Four patients underwent preoperative chemotherapy and radiotherapy for Wilm's tumor, and three were without pathological diagnosis. Three of the four are currently free of disease. Patients with massive abdominal tumors and with the clinical and radiological characteristics of Wilm's tumor should receive preoperative chemotherapy and radiation. This treatment plan should provide the best chance of resectability and will, it is hopes, improve the chance of survival in these children.

Perforation of the peritoneum and intra-abdominal hemorrhage: a complication of umbilical vein catheterizations.

We report a major hemorrhage into the peritoneal cavity caused by perforation of the umbilical vein by a venous catheter in a critically ill neonate. A lateral roentgenogram was necessary to confirm the position of the umbilical vein catheter and the peritoneal perforation. Conservative management, including replacement of lost volume and correction of coagulation abnormalities, controlled the hemorrhage, and surgical intervention was not necessary.

Recurrent arterial thrombosis in nephrosis.

Recurrent arterial thrombosis without inciting trauma occurred in an 8-year-old boy with the nephrotic syndrome. Thrombosis of the left femoral artery was successfully managed by early thrombectomy and postoperative anticoagulation therapy, but the right leg finally had to be amputated. The development of hypovolemia in the nephrotic child receiving long-term steroid therapy apparently increases the tendency to a hypercoagulable state.