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.

Compression of the trachea by the innominate artery in a 2-month-old child.

SUMMARY. We utilized improved methods for assessing airway structure and function to define the clinical significance of the innominate artery syndrome. Both infant pulmonary function tests (IPFT) and noninvasive controlled ventilation computed tomography (CVCT) were used, along with traditional diagnostic techniques in a 2-month-old child with compression of the trachea by the innominate artery. These tests provided objective documentation of functional impairment before surgery and improvement postoperatively. These tests should aid in the understanding of this controversial syndrome and help to further define treatment options.

Functional urinary tract obstruction developing in fetuses with isolated gastroschisis.

OBJECTIVE: To evaluate the frequency and natural history of urinary tract abnormalities developing in fetuses presenting with initially isolated gastroschisis. METHODS: Serial ultrasounds were performed prospectively on fetuses identified by our prenatal diagnosis program as having a gastroschisis. When abnormalities in the urinary tract were identified prenatally, newborns were evaluated by a pediatric urologist. RESULTS: Over a 1-year period four out of 12 fetuses with gastroschisis developed deformations of the urinary tract. In three fetuses the bladder herniated through the abdominal wall defect. Two also had upper tract dilatation. A fourth fetus developed bilateral hydronephrosis with a normally situated bladder. Once the gastroschisis was repaired none of the newborns had evidence of structural obstruction of the urinary tract, however, hydronephrosis with or without reflux persisted for several months. CONCLUSIONS: Deformations of the fetal urinary tract can develop secondary to gastroschisis. They do not appear to represent separate malformations and evaluation with fetal karyotyping may not be indicated. When hydronephrosis is present ongoing urologic evaluation of the neonate is indicated.

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.

Infarction of the choledochus, liver, gallbladder, and pancreas: a unique complication of the hemolytic uremic syndrome.

Hemolytic uremic syndrome (HUS) is associated with multiple nonrenal manifestations. A unique case is described of a 3-year-old boy who presented with a classic diarrheal prodrome followed by massive necrosis of the biliary tree and common bile duct, pancreas, and the left lobe of his liver. This complication of HUS has not been reported in the English-language literature.

Pediatric airbag injuries: the Ohio experience.

BACKGROUND/PURPOSE: We sought to determine if properly restrained children, less than 13 years of age, placed in the front passenger seat are at greater risk for trauma from airbag deployment than unrestrained children. METHODS: The charts of children treated at any of 3 regional pediatric trauma centers in Ohio were reviewed for airbag injuries sustained in motor vehicle crashes between January 1995 and September 1998. Injury Severity Scores (ISS) were compared with Mann-Whitney Rank Sum Test and outcome data with Fisher's Exact Tests. Statistical significance was set at P< or =.05. RESULTS: Twenty-seven children aged 1 month to 12 years sustained airbag-related injuries. Sixty-one percent were girls. ISS ranged from 1 to 75 with a mean score (+/- SD) of 10 (14.5). All crashes were at reported speeds of less than 45 mph, and 64% were head-on collisions. No significant differences in the mean ISS (P = .074) occurred between groups. Both groups had similar closed head, ocular and facial injuries, extremity fractures, and number of deaths (P = 1.0). Abdominal organ injury was exclusive to the restrained group. Decapitation only occurred among unrestrained children. CONCLUSION: Our data showed that airbags, with or without proper safety restraints, can lead to mortality or serious morbidity in children.

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.

Nonteratomatous germ cell tumors in children.

BACKGROUND/PURPOSE: Totipotential germ cells may give rise to a broad range of tumors. The teratomatous variety of germ cell tumors has been the subject of several large studies. The goal of the current study was to describe the clinical features of nonteratomatous germ cell tumors (NTGCT) by reviewing a large series of patients. METHODS: Between 1945 and 1997, there were 78 cases of nonteratomatous germ cell tumors (NTGCT's) in children at The Children's Hospital, Columbus. Their records were reviewed retrospectively. There were 35 boys and 43 girls (M:F ratio 0.8). Mean follow-up was 87 months. RESULTS: Histological subtypes included germinoma (33 cases, 42%), endodermal sinus tumor (24 cases, 31%), embryonal carcinoma (12 cases, 15%), gonadoblastoma (4 cases, 5%), mixed histology (4 cases, 5%), and choriocarcinoma (1 case, 2%). Forty-two tumors were in gonadal sites, but a significant percentage were extragonadal (36 cases, 46%). Forty-six patients (59%) had localized disease, 18 (23%) had regional disease, and 14 (18%) had metastases. Treatment consisted of surgery and selective chemotherapy and radiation. Complete tumor resection was more likely for gonadal (29 of 42, 69%) than extragonadal primaries (15 of 36, 41%; P < or = .05). Forty-nine (63%) of all patients survived, whereas 29 (37%) died of their disease. Survival in patients with gonadal primaries (32 of 42, 76%) exceeded that in patients with extragonadal primaries (17 of 36, 47%; P < or = .01). Survival in patients with localized disease (34 of 46, 74%) exceeded that in patients with regional extension or metastases (15 of 32, 47%; P < .05). CONCLUSIONS: This study highlights the fact that tumor location, gonadal versus extragonadal, was important in determining prognosis, whereas tumor histology was not. This may be the result of a higher rate of complete tumor resection for gonadal primaries and underscores the important role of surgery in the optimal treatment of these unusual tumors.

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.

Analysis of a nonpeptide antagonist for substance P on myenteric neurons of guinea-pig small intestine.

CP-96,345 [(2S,3S)-cis-2-(diphenylmethyl)-N-[(2-methoxyphenyl)-methyl]- 1-azabicyclo[2.2.2.]octan-3-amine], a novel nonpeptide antagonist of the substance P receptor, was evaluated for blocking action at substance P receptors on myenteric neurons of guinea-pig small intestine. Intracellular electrophysiological recording was used to determine actions of the drug on excitatory responses to substance P, on slow and fast excitatory postsynaptic potentials and action potential initiation and propagation in the neurons. CP-96,345 suppressed responses to substance P. It also suppressed spike initiation and propagation in the neuronal processes, as well as in the somal membranes. The effects of the drug on substance P responses could not be attributed to an action at substance P receptors. The mechanism of action appeared to be a nonselective local anesthetic effect on initiation and propagation of action potentials.