Lateral thoracic expansion for Jeune's syndrome: midterm results.

BACKGROUND: In 1995, we reported the use of lateral thoracic expansion in a patient with symptomatic Jeune's asphyxiating thoracic dystrophy. We have subsequently used lateral thoracic expansion 16 times on 10 patients during 7 years. This article reports our outcomes and provides surgical details. METHODS: Charts of all patients undergoing lateral thoracic expansion were reviewed. Eight of the 10 patients had symptomatic Jeune's syndrome. The other 2 had similar thoracic deformities limiting thoracic capacity. In half of the patients the procedures were performed bilaterally. RESULTS: All patients older than 1 year of age were symptomatically benefited by lateral thoracic expansion. Functional and anatomic measurements documented thoracic enlargement in several patients who had comparable preoperative and postoperative studies. However, 2 infants with significant underlying airway disease did not improve and went on to succumb to that aspect of their disease despite enlargement of the thorax. Fracture of the titanium ministruts has been a recurrent problem, and we now use larger struts. CONCLUSIONS: Lateral thoracic expansion is a safe and effective procedure in selected patients with Jeune's syndrome older than 1 year of age as judged by short-term and midterm follow-up. More experience and longer follow-up are required to discern the place of the lateral thoracic expansion in the overall management of these patients.

Focused abdominal sonography for trauma (FAST) in children with blunt abdominal trauma.

BACKGROUND: Focused abdominal sonography for trauma (FAST) has been well reported in adults, but its applicability in children is less well established. We decided to test the hypothesis that FAST and computed tomography (CT) are equivalent imaging studies in the setting of pediatric blunt abdominal trauma. METHODS: One hundred seven hemodynamically stable children undergoing CT for blunt abdominal trauma were prospectively investigated using FAST. The ability of FAST to predict injury by detecting free intraperitoneal fluid was compared with CT as the imaging standard. RESULTS: Thirty-two patients had CT documented injuries. There were no late injuries missed by CT. FAST detected free fluid in 12 patients. Ten patients had solid organ injury but no free fluid and, thus, were not detected by FAST. The sensitivity of FAST relative to CT was only 0.55 and the negative predictive value was only 0.50. CONCLUSION: FAST has insufficient sensitivity and negative predictive value to be used as a screening imaging test in hemodynamically stable children with blunt abdominal trauma.

Neonatal intrapericardial teratomas: clinical and surgical considerations.

Intrapericardial teratomas are rare primary cardiac tumors of infancy and childhood. We describe three neonates with intrapericardial teratomas diagnosed during fetal life and treated after birth. Clinical and anatomic considerations suggest that cardiopulmonary bypass provides for safe tumor dissection and complete excision of the tumor, thereby decreasing the risk of recurrence.

Evidence that colitis is initiated by environmental stress and sustained by fecal factors in the cotton-top tamarin (Saguinus oedipus).

Parallel changes in spontaneously occurring inflammation in colonic Thiry-Vella loops and the in-line colon of cotton-top tamarins were studied in a colitis-inducing environment at 8 and 15 months following surgical preparation of the loops. Gross disease severity and numbers of inflammatory/immune cells per unit area of lamina propria in histological sections from endoscopic biopsies were analyzed. Cell counts and severity of colitis declined over time in the Thiry-Villa loops while the disease followed its characteristic course in the remaining large bowel and in the colons of controls. Perfusion of the loops with the animals' feces increased the density of the cellular infiltrate in the lamina propria in parallel with increased severity of inflammation. Electron micrographs of the colonic mucosa showed invasion by microorganisms. The predominant microorganism had characteristics of Helicobacter sp. The results implicate the fecal stream as a factor in the persistence of colitis in the tamarin model. Nevertheless, fecal factors appear not to be the primary trigger, as evidenced by findings that the disease is not expressed in wild-living tamarins and that it enters remission when affected animals are transferred to natural conditions from a colitis-inducing environment. Both an adverse environment and the fecal contents appear to be required for expression of the disease.

Metastatic paraganglioma and paraneoplastic-induced anemia in an adolescent: treatment with hepatic arterial chemoembolization.

Mediastinal paragangliomas are rare neoplasms in children. Anemia, as a paraneoplastic syndrome, has been described in adults with metastatic paraganglioma. The management of paraneoplastic anemia from metastatic paraganglioma has been problematic, with no reports in the literature describing successful treatment. This article describes a 17-year-old Jehovah's Witness with a mediastinal paraganglioma, hepatic metastases, and severe anemia. The patient and his family refused blood products and the anemia was refractory to erythropoietin and elemental iron therapy. Serial chemoembolization of the hepatic lesions resulted in resolution of the anemia, allowing subsequent debulking of the mediastinal paraganglioma.

Bronchopexy for congenital bronchomalacia in the newborn.

Congenital bronchomalacia is a very unusual cause of respiratory distress in the newborn. The surgical management of this anomaly is challenging. The authors report on a newborn with congenital bronchomalacia successfully treated with bronchopexy.

Duodenum inversum mimicking mesenteric artery syndrome.

BACKGROUND: Duodenum inversum is an often unrecognized anomaly of duodenal rotation/fixation at upper gastrointestinal (UGI) contrast study because the duodenojejunal junction appears normally located. OBJECTIVE: This anomaly is important to diagnose because it may result in obstructive gastrointestinal symptoms. CONCLUSION: We describe a case of duodenum inversum mimicking superior mesenteric artery (SMA) syndrome that improved after surgical therapy.

Pulmonary homograft repair of a mycotic aortic aneurysm in an infant.

Thoracic aortic aneurysms are rare in children and even more unusual in infants. The vast majority are mycotic. Frequently, those with mycotic thoracic aortic aneurysm do not survive and the diagnosis is made at autopsy. We present the case of an asymptomatic infant found to have a mycotic thoracic aortic aneurysm. The clinical course, diagnosis, and surgical repair of the aneurysm with pulmonary homograft are discussed.

Preliminary experience with focused abdominal sonography for trauma (FAST) in children: is it useful?

BACKGROUND/PURPOSE: Most pediatric surgeons and pediatric radiologists consider computed tomography (CT) the best radiological test for the evaluation of children with suspected intraabdominal injury. The majority of injured children evaluated with CT will be found to have a normal scan. Focused abdominal sonography for trauma (FAST) has been shown to be a useful screening test in the evaluation of adult patients with suspected intraabdominal injury. Limited data exist regarding the use of FAST in children. Our aim was to evaluate the usefulness of FAST as a screening test in the evaluation of children with suspected intraabdominal injury in an attempt to minimize the number of normal CT scans performed. METHODS: Hemodynamically stable children evaluated for suspected intraabdominal injury were prospectively screened with FAST. FAST, real-time sonography at four sites, was performed by staff pediatric radiologists. The average duration of the examination was 2 minutes. Positive and negative FAST scan findings were defined prospectively. The result of each FAST was recorded (positive or negative) and then all patients underwent CT as a control. All management decisions were based on CT results. RESULTS: Forty-six patients were included in the study. FAST identified four children with positive findings (free intraperitoneal fluid), whereas CT showed 13 children with injuries (nine with associated free intraperitoneal fluid and four with only solid organ injury and no associated intraperitoneal fluid). There were nine false-negative and no false-positive FAST scans. The sensitivity of FAST was 0.3 and the specificity was 1.0. Injuries missed by FAST included liver laceration, adrenal hematoma, renal laceration, small bowel injury and splenic laceration. CONCLUSION: Preliminary results suggest that FAST alone is not a useful screening test in the evaluation of children with suspected intraabdominal injury.

Primary intrathoracic gastric volvulus in a newborn.

Primary gastric volvulus in the neonatal period is extremely rare, with only 18 cases reported in the literature. The authors describe the case of a newborn with intrathoracic gastric volvulus and discuss its management.

Penetrating neck trauma in children: a reappraisal.

The management of penetrating neck injuries in adults is controversial, with a trend toward selective neck exploration. These injuries are uncommon in children, and only limited information exists regarding their management. To assess the management of these injuries in the authors' geographic region, they reviewed the records of children with injuries penetrating the platysma muscle who were treated between 1980 and 1994. Forty-six children (aged 2 to 16 years) suffered a total of 55 penetrating neck injuries. The injuries were classified according to type and location. Fifty-two percent were caused by missiles, 30% by stab wounds, and 18% by dog bites. Fifty-eight percent of injuries were in zone II, 31% in zone I, and only 11% in zone III. The diagnostic workup, including arteriography, esophagography, or endoscopy, was performed preoperatively in 10 patients. Overall, 21 patients had exploration, and the rate of negative explorations was 48%. All cases explored for bleeding or a positive diagnostic workup result were found to have significant injury. On the other hand, all neck explorations performed solely because of injury to zone II were negative. The overall morbidity and mortality rates were 31% and 7%, respectively. A more selective approach, similar to that used for adult patients, emphasizing preoperative diagnostic evaluation, is recommended to decrease the rate of negative neck explorations among children.

PEG ileus. A preventable complication.

Percutaneous endoscopic gastrostomy (PEG) tube placement is an established procedure in the nutritional management of patients. There are numerous reports in the literature describing the techniques for placing PEG tubes. However, there are few reports that discuss the proper methods for removing these devices and the complications that may result from incorrectly removing a PEG tube. An increasing number of patients with PEG tubes are being cared for by individuals who are not familiar with these devices and their proper method of removal. The use of an incorrect method to remove a PEG tube may result in significant morbidity to the patient. We report a case of bowel obstruction resulting from the incorrect removal of a PEG tube that required laparotomy. To prevent similar complications, PEG tubes should be removed using the appropriate method by individuals familiar with the device.

Xanthogranulomatous pyelonephritis in renal allografts: report of 2 cases.

We report 2 cases of xanthogranulomatous pyelonephritis that occurred in renal transplant recipients. Both cases were successfully treated with antibiotics. The traditional treatment of choice has been nephrectomy. No graft was lost as a result of xanthogranulomatous pyelonephritis and no nephrectomy was required in either patient. The course of these 2 patients is discussed and literature on this disease is reviewed.