Biomedical subjects
Publications and source records attributed to J Maddalozzo.
We describe a case of enteric duplication arising from the floor of the mouth and base of the tongue of a 7-year-old child. This mass was asymptomatic and was detected on routine dental examination. The unusual location, possible etiology, and a brief review of the literature are discussed.
BACKGROUND: Cystic hygromas are characterized by a proliferation of small vessels and lymphatics with intervening fibrous tissue. Studies have shown malignant tumors and some benign neoplasms are dependent on angiogenesis, the induction of new capillaries from preexisting vessels. Growth and progression of these tumors are associated with a disturbance in the balance of angiogenic inducers and inhibitors. We have postulated that cells derived from cystic hygromas are angiogenic due to secretion of higher levels of angiogenic inducers that promote vascular proliferation. DESIGN: A large cystic mass was surgically removed and a portion of the sterile tumor was immediately placed in the medium. The tissue was minced, washed in phosphate-buffered saline, and grown to near confluence. Conditioned medium was collected under serum-free conditions after 48 hours. Secreted proteins were concentrated, quantitated, and analyzed in an in vitro endothelial cell migration assay and by Western blot. Antibody to factor VIII-related antigen was performed to confirm endothelial cell origin of the cultured cells. MAIN OUTCOME MEASURES: In vitro angiogenic activity of secreted proteins in a capillary endothelial migration assay was tested by using blocking antibodies to angiogenic inducer, basic fibroblast growth factor, and angiogenic inhibitor, thrombospondin-1. Total protein levels of thrombospondin-1 were determined by Western blot. RESULTS: Cells isolated from cystic hygroma are angiogenic in vitro and this angiogenic activity is due to secretion of high levels of angiogenic inducer, basic fibroblast growth factor, and lower levels of naturally occurring angiogenic inhibitor, thrombospondin-1. CONCLUSIONS: Cystic hygromas may represent another neoplasm dependent on angiogenesis. The angiogenic activity is due in part to elevated levels of potent angiogenic inducer, basic fibroblast growth factor. Antiangiogenic therapy directed at the endothelial cell may help suppress the growth of cystic hygromas.
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UNLABELLED: We assessed the safety and efficacy of oral transmucosal fentanyl citrate (Fentanyl Oralet; Abbott Laboratories, Abbott Park, IL), administered preoperatively to provide both preoperative sedation and postoperative analgesia, in a randomized, double-blind, placebo-controlled study in 40 children, 2-10 yr of age, scheduled for tonsillectomy. In the preoperative holding area, one group (Group O) received Fentanyl Oralet (fentanyl 10-15 micrograms/kg), and the other (Group IV) received only the candy matrix. Patients in Group O received an i.v. injection of saline, and those in Group IV received an i.v. injection of fentanyl (2 micrograms/kg) after removal of the first tonsil. Except for the opioid, patients received a standard anesthetic. Preoperative sedation and cooperation were assessed. Postoperative pain was evaluated using an objective pain scale. Patients in Group O were more sedated but no more cooperative at the induction of anesthesia compared with those in Group IV. No patient vomited preoperatively or experienced preoperative or postoperative desaturation. Time to postanesthesia care unit (PACU) discharge was not different between groups. There was no significant difference in the number of patients requiring morphine in the PACU (6 of 21 in Group O versus 10 of 19 in Group IV). Plasma fentanyl concentrations were not a reliable indicator of the need for postoperative morphine. Among the patients who required morphine postoperatively, there was an 11-fold variation in plasma fentanyl concentrations at the time of morphine administration. Derived pharmacokinetic parameters were similar to those previously reported in children; bioavailability of the fentanyl in Fentanyl Oralet was 0.33. We conclude that premedication with Fentanyl Oralet did not differ with i.v. fentanyl in regard to the induction of anesthesia and postoperative analgesia. IMPLICATIONS: In this double-blind, randomized study, we studied the efficacy of Fentanyl Oralet (10-15 micrograms/kg) preoperatively for providing postoperative analgesia in children undergoing tonsillectomy. We found no incidence of preoperative desaturation or vomiting in any patient. This is in contrast to other studies, in which there was a longer time interval between Fentanyl Oralet completion and induction of anesthesia. The bio-availability of the fentanyl in Fentanyl Oralet was estimated to be 33%, which is less than that reported in adults (approximately 50%). There was no difference in postoperative opioid requirements between patients who received 2 micrograms/kg of fentanyl i.v. and those who received Fentanyl Oralet.
Pseudotumor of infancy (POI) occurs within the first month of life as a benign neck mass and affects 0.4% of all newborns. Differentiating these lesions from a malignancy or a congenital anomaly requiring surgical intervention can represent a diagnostic challenge for the clinician. While 80-90% of these masses resolve spontaneously within the first six months of life, there is still considerable time expended and cost incurred in the evaluation of POI. We have established and implemented a protocol for the evaluation of these patients consisting of a complete history and physical exam, ultrasonography and routine follow-up every four to six weeks. Seventeen patients were identified who had been evaluated and followed in the aforementioned manner. Two patients underwent diagnostic imaging studies prior to referral, consisting of magnetic resonance imaging (MRI) and computed tomography (CT). Ultrasonography appeared identical in 80% of the cases of POI with a heterogeneous pattern of internal echogenicity and a hypoechoic surrounding rim. The remaining ultrasound studies differed only subtly with regard to the internal echo pattern. There were no patients with pseudotumor of infancy in whom the ultrasound was normal. We concluded that a work-up including a history and physical exam and ultrasound is an accurate and cost-effective method for evaluating newborns with a neck mass, when suspicious for POI. Ultrasonography has a 100% sensitivity for the diagnosis of POI in this clinical scenario.
Aggressive juvenile fibromatosis, though allegedly a benign process, is as frustrating to manage as it is perplexing to comprehend. The treatment is primarily surgical, with chemotherapy and radiation therapy recently finding support as adjuncts in selected circumstances. Though there is no agreement regarding the etiology of fibromatosis, many have suspected hormonal or traumatic influences. There has been historical, clinical and experimental data demonstrating that fibromatosis seems to be under the influence of estrogen. There have also been anecdotal reports that this tumor has regressed with the use of tamoxifen. To our knowledge, no one has tested these tissues for the presence of estrogen/progesterone receptors. Recently, over a short period of time, we at Children's Memorial Hospital, Chicago, have treated four youngsters with this disorder. All operative specimens were submitted for estrogen and progesterone assays. Although these cases were indistinguishable on histologic examination, two of the four exhibited the presence of estrogen/progesterone receptors. We propose, that from these observations, there potentially may be derived a histochemical classification based upon the presence or absence of estrogen/progesterone receptors. This would serve as an added reference in the definition and treatment of this disease. Should hormonal receptors be present, agents such as tamoxifen conceivably could be employed as part of a post-operative maintenance regimen similar to those protocols applied in the management of hormonally responsive breast cancer.
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Chondrosarcoma of the nose and paranasal sinuses is extremely rare. We report a case of a child with a massive chondrosarcoma of the sphenoethmoid complex who presented with a change of visual acuity. Clinical and histologic characteristics of this lesion are discussed, along with treatment options and factors affecting prognosis.
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A previously undescribed deficiency of the posterior cricoid at the midline, wherein the mucosa overlying this deficiency is intact, is described. Clinically this presents as a variety of subglottic stenoses wherein the narrowing is primarily in the transverse subglottic diameter; the symptoms are, therefore, those of airway obstruction not laryngeal incompetence. It is suggested that this deformity has been previously overlooked by laryngologists and pathologists by virtue of a postmortem routine in which the larynx is routinely split in the posterior midline. Demonstration in these cases was by serial horizontal section of the intact, unsplit, whole larynx.
There is always concern for a malignancy or other serious disease when a child presents with a chief complaint of a neck mass. However, a neck mass in child is commonly inflammatory or congenital rather than neoplastic. A complete history and physical exam will often provide enough information to guide management and reassure the concerned parents. In some cases, laboratory tests, radiologic studies or a surgical procedure may be necessary to determine a specific diagnosis and treatment plan. An organized and systematic approach is recommended and detailed in this review.
The experience with laryngotracheal reconstruction in 20 children in Chicago is reviewed. Nine of the 20 children (45%) operated upon had congenital subglottic stenosis. Ten (50%) had acquired stenosis. One child was classified as having combined types. All patients underwent laryngotracheal reconstruction with autogenous costal cartilage grafts. Eight patients had both anterior and posterior costal cartilage grafts with stent insertion. One had a posterior costal cartilage graft only. Sixteen of the 20 children (80%) have been decannulated. Midtracheal or lower tracheal disease concomitant with laryngotracheal stenosis is an indication that endoscopic management of severe subglottic stenosis is likely to fail. Early laryngotracheal reconstruction is indicated for patients with a high likelihood of failure of endoscopic management and for those with severe cricoid cartilage deformities. Our results support the use of laryngotracheal reconstruction as an alternative to conservative (endoscopic) management of severe subglottic stenosis in carefully selected patients.