Robyn M. Hatley

Interferon-alpha-2a for the treatment of complex hemangiomas of infancy and childhood.

ObjectiveThe authors describe the use of interferon–alpha-2a (IFN–α-2a) in the treatment of complex hemangiomas and review the role of interferon (IFN) in this example of an angiogenic disease. Summary Background DataHemangiomas are the most frequent tumors of infants and children. They grow rapidly for 6 to 8 months and then resolve over a period of years. Approximately 5% produce life-, sight-, or limbthreatening complications, with mortality rates between 20% and 50%. Aggressive therapy with steroids, arterial ligation or embolization, or surgery has been used in these situations with variable results and high morbidity. Recently, IFN–α was found to be effective treatment in these complex hemangiomas. MethodsFour infants and one child were treated with IFN–α-2a at an initial subcutaneous dose of 1 million units/m2/day and a sustained dose of 3 million units/m2/day for 5 to 11 months. Appropriate laboratory values were monitored and adverse reactions and ultimate response to therapy were recorded. ResultsTwo patients experienced minor complications that were managed easily. Three patients had total or near-total regression of the hemangioma, one had partial (50%) regression, and one had stabilization but no regression after an average of 7.1 months of IFN therapy. ConclusionInterferon–α inhibits angiogenesis and endothelial cell migration and proliferation in vitro. The patients in this study add to the growing number who have benefited from IFN therapy. As such, IFN–α should be considered as a first-line agent in treating complex hemangiomas of infants and children.

Successful management of an infant with a giant hemangioma of the retroperitoneum and Kasabach-Merritt Syndrome with α-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.

Prostaglandin-induced foveolar hyperplasia simulating pyloric stenosis in an infant with cyanotic heart disease

Prostaglandin infusion is used to maintain patency of the ductus arteriosus in infants with cyanotic congenital heart disease. Recently, gastric outlet obstruction as a result of prostaglandin infusion has been described. In our case, an upper gastrointestinal contrast study seemed to depict the typical appearance of pyloric stenosis in an infant who had received an infusion of prostaglandin for a prolonged period. Serial ultrasonograms, however, disclosed progressive elongation of the antropyloric channelwithout wall thickening. This report is the second to illustrate prostaglandin-induced gastric outlet obstruction in a vomiting infant with a gastrointestinal series diagnosis of pyloric stenosis.

Splenic microabscesses in the immune-compromised patient

Four immune-compromised children who were receiving antineoplastic chemotherapy (three for leukemia), presented with recurrent episodes of fever and left upper abdominal pain. Blood cultures grew enteric gram-negative organisms in three children. Multiple blood cultures were negative for fungus although three patients had mucocutaneous and urinary candidiasis. All remained febrile and symptomatic despite treatment with broad spectrum antibiotics and antifungal chemotherapy. Computed tomography (CT) scans in all patients showed 2- to 10-mm focal defects in the spleen. The larger defects could be seen by ultrasonography but not on the live-spleen nuclear scan. A splenectomy was performed 2 to 4 weeks after the onset of symptoms in each child, and the cut surface of the spleens showed multiple small abscesses. All operative cultures were negative. A histological examination confirmed Candida infection in two patients and Aspergillus in one. Necrotizing granulomas strongly suggestive of fungus were seen in the fourth child. The patients defervesced and appeared well within three days. Antifungal therapy was continued. One child remains in remission from acute lymphocytic leukemia; one continues on chemotherapy; and one has recurrent widespread tumor. The patient with Aspergillus died following a bone marrow transplantation 6 months after the splenectomy. He had disseminated aspergillosis. An immune-compromised patient with persistent unexplained fever should have a CT scan of the abdomen. The presence of multiple splenic lesions strongly suggests fungal disease. If antifungal therapy does not result in complete resolution of fever and the splenic lesions, a splenectomy is indicated.

Cervical thymic cysts: CT appearance of two cases including a persistent thymopharyngeal duct cyst

The differential diagnosis of cervical cysts in children includes common entities such as branchial cleft cysts, thyroglossal duct cysts, and cystic hygromas. Congenital thymic cysts are uncommon and often misdiagnosed as either branchial cleft cysts or cystic hygromas. However, they may have an appearance on CT that can be characteristic. The course of the descent of embryologic thymic tissue in the neck to the mediastinum indicates the potential site of deposition of an ectopic cervical thymic cyst. In a child, a cystic lesion that has an intimate relationship to the carotid sheath is likely to be a thymic cyst.Of the approximately 100 cases of vestigial cervical thymus or thymic cysts that have been reported in children, only 5 cases of a persistent thymopharyngeal duct cyst have been described [1–5]. In two of these five, the persistent thymopharyngeal duct cyst was demonstrated by CT [1, 2]. We report one additional case of a cervical thymic cyst and one case of a persistent thymopharyngeal duct cyst both depicted by CT.

Thoracic Empyema in a Patient With Acute Appendicitis: A Rare Association

Thoracic empyema and appendicitis rarely are concomitant. This is the first report of ultrasonography and computed tomography being used preoperatively to establish the diagnosis of ruptured appendicitis in a child with thoracic empyema. The perforated appendicitis was identified after gastrointestinal flora were cultured from the thoracostomy drainage of the empyema.

Popliteal pseudoaneurysm caused by Nora's lesion of the femur in a young child: A rare presentation and first report

Bizarre parosteal osteochondromatous proliferation (BPOP), or Noras lesion, was first described by Nora et al. in 1983 as a rare, tumor-like lesion involving the bones of the hands and feet. Popliteal artery pseudoaneursyms in the pediatric population are also unusual. Here, we present a case of a young male with a popliteal artery pseudoaneurysm and distal femur lesion originally thought to be an osteochondroma. A 10-year old, Caucasian male was referred to our facility following an MRI concerning for a popliteal artery pseudoaneurysm. On physical exam, there was a palpable 5 × 5-cm pulsatile mass in the upper popliteal fossa with a normal pulse exam bilaterally. A computed tomographic angiogram demonstrated a 4.5-cm by 1.8-cm by 3.6-cm pseudoaneurysm adherent to a 3.5-cm thick, exostotic lesion of the posterior right femur. He was taken to the operating room for repair of the popliteal pseudoaneurysm and resection of his bone lesion. The final pathology was consistent with a popliteal pseudoaneurysm, osteochondroma, and bizarre parosteal osteochondromatous proliferation (BPOP), otherwise known as Noras lesion. The location of the lesion and the age of our patient were both atypical for BPOP and to our knowledge, this represents the first report of a resulting popliteal artery pseudoaneurysm.

Heterotopic gastric mucosa of the upper esophagus following repair of esophageal atresia with tracheoesophageal fistula

A term female newborn underwent uncomplicated repair of esophageal atresia with tracheoesophageal fistula (type C) then coarctation of the aorta. Subsequently, she developed symptomatic esophageal strictures, which required serial dilations. In addition, she suffered feeding intolerance from esophageal dysmotility and gastroesophageal reflux disease, necessitating Nissen fundoplication with gastrostomy. At 6 years of age, surveillance esophagogastroduodenoscopy revealed mild stenosis and heterotopic gastric mucosa of the upper esophagus at the level of the anastomosis. This is the second report of heterotopic gastric mucosa of the upper esophagus at the level of the anastomosis following repair of esophageal atresia with tracheoesophageal fistula.

Neonatal pharyngeal pseudodiverticulum mimicking esophageal atresia.

Pseudodiverticulum of the hypopharynx is an infrequent but potentially serious complication of orogastric or nasogastric tube insertion and endotracheal intubation. We report two cases of injury to the hypopharynx resulting in a pseudodiverticulum of the hypopharynx that was initially diagnosed as esophageal atresia. Both cases were managed successfully by conservative therapy. We also review the literature regarding neonatal pharyngeal pseudodiverticulum.

Rectovulvar fistula in a child secondary to an unusual foreign body

Foreign bodies in the female genital tract are well recognized as a cause of pain, discharge, secretions, and infection. In the small child, the presence of a vaginal discharge is usually associated with either a common object which has been inserted or sexual abuse complicated by a sexually transmitted disease. A 3-year-old child presented with a recurrent labial secretion and drainage due to an unusual foreign body. It was only during a second operation that the possibility of a foreign body was entertained, and diagnostic testing was begun. A third operation permitted removal of the foreign body, a large bone probably of animal origin. The fistula tract was closed after a colostomy was performed. This represents the first reported case of a rectovulvar fistula not of a congenital nature.