Nasopharyngeal teratomas and dermoids: a review of the literature and case series.

OBJECTIVES: Review the clinical differences between nasopharyngeal (NP) true teratomas and dermoids, with the addition of a case series to the literature. Additionally, review the impact of prenatal diagnosis on the management and outcome of these lesions. STUDY DESIGN: Retrospective review of cases from the Children's Hospital Medical Center (Seattle, WA) and Madigan Army Medical Center (Tacoma, WA) with the histopathologic diagnosis of nasopharyngeal teratoma or dermoid. Review of medical literature for cases reported since 1977, when prenatal diagnosis of these lesions was first reported. Only tumors of the nasopharynx were considered. METHODS: Charts and pertinent literature were reviewed and data presented with respect to age at diagnosis, signs and symptoms, perinatal diagnosis and management, preoperative evaluation, surgical treatment, and outcome. RESULTS: The majority of lesions were diagnosed at birth, with the most common presenting symptom being respiratory distress. Teratomas had a higher incidence of maternal polyhydramnios, preterm birth, need for emergent airway management, and associated congenital abnormalities. Complete surgical excision remains the treatment of choice. Recurrences were rare, occurring only in the teratoma group. Prenatal diagnosis did not have a significant impact on the diagnosis and treatment of these lesions in our review. CONCLUSIONS: Inconsistent use of a standard classification system has made differentiating between NP teratomas and dermoids difficult, although the clinical implications can be critical. NP teratomas have a higher incidence of preterm birth, neonatal airway distress, associated congenital abnormalities, need for more extensive surgical procedures, and recurrence. Prenatal diagnosis has made little impact on the overall diagnosis and treatment of these lesions.

Presence of 22q11 deletion in postadenoidectomy velopharyngeal insufficiency.

BACKGROUND: Velopharyngeal insufficiency is an uncommon complication of adenoidectomy. Persistent velopharyngeal insufficiency following adenoidectomy (VIA) may occur in association with an unrecognized syndrome, such as velocardiofacial syndrome (VCFS). Although the diagnosis of VCFS is primarily a clinical one, a test has been developed to identify the underlying chromosomal abnormality, ie, deletion of 22q11. OBJECTIVE: To describe characteristics and occurrence of the 22q11 deletion in a population with VIA. SETTING: Three tertiary referral centers. DESIGN: Retrospective case series of 23 patients with VIA who required intervention and had follow-up for more than 1 year. These patients' medical records were reviewed for indications for adenoidectomy, the presence of 22q11 deletion and whether a 22q11 deletion test was obtained, phenotypic evidence for VCFS, presence of a submucous cleft palate, velopharyngeal closure pattern, and type of speech intervention. RESULTS: Of the 23 patients, 9 underwent adenoidectomy for otitis media, 9 for obstructive sleep symptoms, and 5 for sinusitis therapy. Fourteen of the 23 patients were tested for a 22q11 deletion. Of these 14 patients, 9 had a 22q11 deletion with 5 having phenotypic evidence for VCFS. Six of the 23 patients had a submucous cleft palate, 2 of whom had a 22q11 deletion. CONCLUSIONS: Although VIA is uncommon, its occurrence should alert the otolaryngologist to the possibility of an underlying syndrome diagnosis. The 22q11 deletion test is beneficial in diagnosing patients with genotypic, but not phenotypic, VCFS in this population. In tested subjects of our patient population, 28% (4 patients) had the genotype for VCFS, without clinical evidence of VCFS.

Surgical excision of pilomatrixoma of the head and neck: a retrospective review of 26 cases.

The objective of this article is to describe our experiences in treating patients for head and neck pilomatrixoma at our institution during a 5-year period and to compare our findings with previously published results. To that end, we conducted a 5-year retrospective chart review to identify those patients who had a confirmed histopathologic diagnosis of pilomatrixoma involving the head and neck area. We reviewed medical records for presenting signs and symptoms, lesion characteristics, treatment rendered, and outcomes. We identified 26 patients, aged 6 to 77 (mean: 33), who met the criteria for inclusion in our study. All had been treated for solitary tumors with simple surgical excision and closure. We found no reported adverse outcomes and no tumor recurrences at the surgical sites. These findings support the use of simple surgical excision as the treatment of choice for these tumors.

Effect of the Palmaz balloon-expandable metallic stent in the trachea of pigs.

Endoscopically placed airway stents offer a viable option in primary or adjunctive treatment of severe pediatric tracheobronchial stenoses. Optimistic clinical reports substantiate the need for experimental studies to more effectively evaluate their clinical role. Development of an animal model comparable with the pediatric airway, amenable to endoscopic instrumentation, and capable of assessing effect on growth was the purpose of this pilot project. Nine 4-week-old piglets underwent endoscopic midtracheal placement of the balloon-expandable Palmaz metallic stent. Initial expansion and stent position were verified fluoroscopically and by direct videobronchoscopy. Serial endoscopic examination and stent reexpansion were performed 2 and 4 weeks after stent insertion. Animal weight, clinical tolerance, tracheal growth, and stent integrity were observed. Tracheal inflammation was evaluated grossly and by objective histopathologic criteria. Successful endotracheal stent placement and expansion were accomplished in all piglets. One pig died of anesthesia complications less than 24 hours after stent insertion. The remaining pigs exhibited excellent clinical tolerance through experiment completion. No detrimental effect on growth was noted, and effective dilatation of the stented tracheal region was observed. Stent incorporation was evident with significant mucosal ingrowth. Inflammation in the form of nonobstructing granulation tissue was present, and no evidence of necrosis or cartilage invasion was evident. The piglet trachea appears to be an excellent model for evaluation of expandable metallic airway stents in management of congenital and acquired tracheobronchial stenoses.

Combined rigid and flexible endoscopic removal of a BB foreign body from a peripheral bronchus.

Aspirated foreign bodies (FB) in the peripheral tracheobronchial tree may present challenging management problems for the bronchoscopist. Critical to successful removal is maintaining airway control while minimizing endoscopy time. An innovative approach utilizing rigid and flexible bronchoscopy in removal of a distal impacted airway is presented. Difficulties encountered as well as advantages of this combined approach are discussed.

Potential pitfalls of initiating a newborn hearing screening program.

OBJECTIVE: To examine the efficacy of a universal screening program for infant hearing. DESIGN: Retrospective analysis. SETTING: Tertiary care center. PATIENTS: We examined 2289 (90.2%) of 2537 infants born at our institution from April 1, 1995, to June 30, 1996, for hearing loss (HL). INTERVENTIONS: We used a 3-stage protocol with transient evoked otoacoustic emissions (TEOAE) in stages 1 and 2 and diagnostic evaluation in stage 3. Infants without reproducible TEOAEs in either ear after stage 2 were referred to stage 3. MAIN OUTCOME MEASURES: Specificity of TEOAE, incidence of HL, prevalence of risk factors for HL, cost of TEOAE screening, and identification of barriers to universal screening. RESULTS: Of the infants undergoing TEOAE screening, 91.1% passed stage 1. Of infants needing repeated testing, 73.7% passed and 26.2% failed. Of the 43 infants referred for diagnostic evaluation, 5 (11.6%) had HL. The combined incidence of conductive and sensorineural HL was 2.18 per 1000 newborns. The prevalence of at least 1 risk factor for HL was 10.4%. The estimated cost of TEOAE screening was $24.48 per infant. Ten percent of infants did not undergo screening due to program deficiencies. CONCLUSIONS: Screening with TEOAE was sufficiently specific for universal screening. However, we were unable to achieve truly universal screening. This is probably the reason for our lower incidence of sensorineural HL. We were unable to continue our universal newborn screening program, due to lack of funding, difficulties with program implementation, and our low incidence of detected HL.

Iatrogenic airway stenosis with recurrent respiratory papillomatosis.

OBJECTIVE: To describe the presentation of, factors contributing to, and treatment of iatrogenic airway stenosis (IAS) associated with recurrent respiratory papillomatosis (RRP). DESIGN: Retrospective case series. SETTING: Pediatric tertiary care center. PATIENTS: The charts of patients treated for RRP in our institution from 1980 to 1995 (N=50) were reviewed. Seven patients were identified as having IAS based on endoscopic findings. MAIN OUTCOME MEASURES: Prevalence and types of IAS within our RRP patient population, methods used to treat IAS, and successful treatment of IAS. RESULTS: Of the 7 patients identified, 3 had isolated posterior glottic stenosis (PGS) and 1 had isolated subglottic stenosis. The other 3 had multiple areas of IAS as follows: PGS with bronchial stenosis, supraglottic stenosis with PGS, and tracheomalacia with tracheal stenosis from a suprastomal granuloma. The factors associated with IAS were extensive papilloma growth in the posterior glottis, prolonged periods of frequent laryngoscopies, and the use of nonstandard therapies, which in our series included topical podophyllum resin or photodynamic therapy. Six patients, all of whom had tracheal RRP at some point in their disease process, required tracheotomy. Five patients required laryngotracheal reconstruction. Laryngotracheal reconstruction permitted decannulation in all cases. Tracheal papillomas became sessile and nonobstructive after decannulation. Laryngotracheal reconstruction with rib grafting was most frequently performed. Of our 50 patients, none who did not have IAS required a tracheotomy. Of the 44 patients who did not require a tracheotomy, only 1 had tracheal papillomas. CONCLUSIONS: Occasionally, therapy for RRP is complicated by IAS. In our series, PGS was most common. Tracheotomy was associated with the presence of both IAS and distal RRP. In selected cases, laryngotracheal reconstruction can be successfully accomplished when RRP is present, and subsequent regression of tracheal RRP is likely.

Acute right heart failure due to adenotonsillar hypertrophy.

A case is presented in which a child with underlying chronic lung disease, developed cor pulmonale and severe pulmonary hypertension as a result of adenotonsillar hypertrophy. His cardiac function acutely decompensated with an upper respiratory infection which exacerbated his obstructive sleep symptoms. Pre and postoperative documentation of cardiopulmonary function was critical in the peri-operative management of this patient. His severe pulmonary hypertension was stabilized using a nasopharyngeal airway and medications pre-operatively, in order to minimize his risk of anesthesia. He continued to require careful monitoring and manipulation of his medications after adenotonsillectomy and bronchoscopy. Serial echocardiograms documented the effects of the various interventions implemented in this patient. Severe acute right heart failure is an unusual complication of obstructive sleep apnea. However, this may become more common as more children survive prematurity and its associated chronic lung disease. These children may have subclinical lung disease and/or chronic pulmonary hypertension even after they no longer require supplemental oxygen and bronchodilators. Because these children are often tenuous, with regard to their cardiopulmonary function, they may be at increased risk to develop significant complications related to obstructive sleep apnea. It is important that a physician familiar with the management of pulmonary hypertension be involved in the care of these patients.

Airway management in children with craniofacial anomalies.

Craniofacial anomalies (CFA) predispose children to airway obstruction. A retrospective study was conducted to describe airway intervention required to manage patients with craniofacial syndromes and diseases involving the midface and mandible (i.e., Pierre Robin, Apert, Treacher Collins, Saethre-Chotzen, CHARGE, Nager, Stickler, Goldenhar, and Pfeiffer). The type of airway intervention, duration of intervention, and associated physical and medical conditions were evaluated. One hundred nine patients had charts available for review and met inclusion criteria. Sixty-five of these patients required airway management, most commonly in the first month of life, ranging from positioning to tracheotomy. Nineteen patients required a tracheotomy. Associated medical conditions and feeding difficulties were associated with airway obstruction. This study evaluates factors that predispose children with CFA to have airway problems that need treatment, as well as the types of airway management that are necessary.

The haemostatic effectiveness of autologous platelet rich plasma sequestered after heparin administration and institution of cardiopulmonary bypass.

Preoperative harvesting and postoperative reinfusion of autologous platelet rich plasma (PRP) has been reported to decrease blood loss as well as the requirement for homologous blood transfusion following cardiopulmonary bypass (CPB). We have developed a technique of intraoperative PRP sequestration which occurs during the initial period of CPB after the patient's circulation is supported and heparin has been given (PRP+). This process does not require any additional hardware, personnel or expense and it is performed without difficulty or complication. To evaluate the effect of PRP+ sequestration and reinfusion on blood loss and homologous blood requirement after CPB, we randomly assigned 126 consecutive patients undergoing elective open heart surgery into the experimental group 1 (PRP+) (n = 64) or the control (no platelet pheresis) group 2 (n = 52). A third group (n = 10) were not included in the randomization. Patients in group 3 had PRP prepared by conventional techniques (PRPc) prior to heparin administration and given to the patient after protamine infusion. Aggregation and activation studies were performed on the PRP+, PRPc, and blood bank platelets (BBP). Per cent aggregation of PRP in response to ADP was superior to that of BBP. There were no significant differences in ADP induced aggregation between PRP+ and PEPc. There was no significant difference in platelet activation (CD62) or number between the three groups. Patients infused with PRP+ showed significantly increased aggregation to ADP when compared with untreated patients 120 minutes after return to the ICW. Furthermore, more homologous haemostatic components (platelets/fresh frozen plasma) were required in the control group. We have demonstrated that collection of autologous PRP+ after administration of heparin does not interfere with its haemostatic effectiveness compared with PRPc prepared before the initiation of bypass. Moreover, this can be performed universally in haemodynamically unstable patients without any additional costs.

Cardiopulmonary bypass with adequate flow and perfusion pressures prevents endotoxaemia and pathologic cytokine production.

Endotoxin and cytokine inflammatory mediators comprise the afferent and efferent limbs of the 'acute phase response'. During cardiopulmonary bypass (CPB) there may be gut translocation of endotoxin and contact activation of lymphocytes. It has been hypothesized that the haemodynamic instability encountered following CPB is caused by the 'acute phase response'. In this study we attempted to quantify the acute phase response in patients undergoing open-heart surgery and determine the influence of these cytokines on perioperative morbidity. Four perioperative blood samples were drawn from 20 consecutive patients undergoing coronary artery bypass grafting (CABG). These samples were assayed for endotoxin and four cytokines. In all cases the cardiac index was maintained > 2.4 l/min/m2 during nonpulsatile normothermic bypass (37 degrees C) and > 1.8 l/min/m2 during nonpulsatile hypothermic bypass (28 degrees C), and the perfusion pressure > 60 mmHg. Endotoxin was not detected in any of the test samples despite positive nonpatient controls. Interleukin 6 (IL-6) and tumour necrosis factor (TNF) were not detected despite an assay sensitivity of 80 and 10 pg/ml, respectively. TNF was detectable with an assay sensitivity of 0.5 pg/ml although there were no significant differences within the group. Interleukin 1 (IL-1) was detected (range = 0.98 - 9.09 ng/ml) in patients and again there were no trends within the group. The platelet activating factor (PAF) values peaked at crossclamp release (1.3 ng/ml versus a baseline of 0.2 ng/ml); however, there was no significant difference within the study.

Intraoperative procurement of autologous fibrin glue.

A method of intraoperative procurement of autologous fibrin glue is described. The relative efficacy of our autologous preparation is compared with that of fibrin glue made with homologous cryoprecipitate. Experimentally, the fibrinogen content and the strength are less than those found in cryoprecipitate and appear related to the fibrinogen content of the autologous plasma used as substrate in the fibrin glue reaction. Clinically, no significant differences are noted in the performance of autologous fibrin glue. We believe the absence of the risk of blood-borne infection with the autologous product is a major advantage.

Bowel obstruction from retained inner bumper following removal of gastrostomy tube: a case report.

Percutaneous endoscopic gastrostomy is an effective means of obtaining enteral access in patients who need chronic nutritional support. We describe a complication resulting from the inability to remove an inner bumper. This bumper migrated into the terminal ileum, where it lodged, causing bowel obstruction. Literature review revealed no reports of similar complications.

A comparative study of the neutrophil stimulatory activity in vitro and pro-inflammatory properties in vivo of 72 amino acid and 77 amino acid IL-8.

IL-8, a potent neutrophil-activating protein, can be produced by many cell types including monocytes, lymphocytes, fibroblasts, neutrophils, and endothelial cells. Depending on the cell source, the N-terminal amino acid sequence of IL-8 displays heterogeneity that has been shown to confer differences in its neutrophil stimulatory activity in vitro. Despite these observations the relative potency of different IL-8 molecules in vivo is unknown. To address this question we have investigated the biologic activity of the two predominant forms of IL-8, the 72 and the 77 amino acid proteins, in vitro and in vivo. In vitro, human rIL-8(72) and human rIL-8(77) dose dependently induced adherence of rabbit peritoneal neutrophils and human neutrophils to laminin-coated plates and elevated cytoplasmic levels of Ca2+ ([Ca2+]i) in fura-2 loaded neutrophils. In these in vitro assays human rIL-8(72) was more potent than human rIL-8(77) while inducing comparable responses to human rC5a. With respect to enhancing [Ca2+]i, neutrophils desensitized to human rIL-8(72) failed to respond to human rIL-8(77). However, neutrophils fully desensitized to human rIL-8(77) could exhibit a partial response to human rIL-8(72). Further, human rIL-8(72) was approximately 10-fold more effective than human rIL-8(77) in displacing human [125I]rIL-8(72) from rabbit peritoneal neutrophils in a receptor-binding assay. In vivo, intradermally administered human rIL-8(72) and human rIL-8(77) induced 111In-neutrophil accumulation and edema formation in rabbit skin. In contrast to the in vitro studies, the two forms of IL-8 gave identical responses in vivo although they were less potent than human rC5a. Our results demonstrate that, in vitro, human rIL-8(72) is more potent than human rIL-8(77) in stimulating neutrophils. It may be that IL-8)72) has a greater affinity and/or efficacy for the neutrophil IL-8 cell-surface receptors. One possibility for the observation that both forms of IL-8 are equipotent in inducing inflammatory responses in vivo is that the extended form is proteolytically cleaved to the more biologically active IL-8(72).

How does service drive the service company?

"How Does Service Drive the Service Company?" presents commentators on Leonard A. Schlesinger and James L. Heskett's September-October article. Commentators include Michael R. Quinlan, Ron Zemke, Jim Snider, Dinah Nemeroff, Steven S. Reinemund, Robert Ayling, Karmjit Singh, James A. Perkins, Joseph E. Antonini, and Walter F. Loeb.

Identification of a second neutrophil-chemoattractant cytokine generated during an inflammatory reaction in the rabbit peritoneal cavity in vivo. Purification, partial amino acid sequence and structural relationship to melanoma-growth-stimulatory activity.

The intraperitoneal injection of zymosan in the rabbit results in the generation of an inflammatory exudate containing oedema-forming and chemoattractant activities. Previous studies demonstrated the early appearance of the complement fragment C5a, followed by the generation of two mediators related to the cytokine interleukin-8 that were separable by cation-exchange h.p.l.c. N-Terminal amino acid sequencing identified one of these mediators as rabbit interleukin-8. This paper describes the purification of the second cytokine by cation-exchange, gel-filtration and reversed-phase h.p.l.c. The purified material had both oedema-forming and chemoattractant activity when assayed in rabbit skin in vivo. On SDS/PAGE a single 6-8 kDa band was observed and N-terminal amino acid sequencing of the reduced and alkylated protein positively identified 36 amino acids. This sequence revealed the rabbit homologue of melanoma-growth-stimulatory activity. The identification of these two cytokines in vivo will provide an opportunity to investigate the importance of their co-release in the inflammatory process.

A longitudinal study of total and free thyroid hormones and thyroxine binding globulin during normal puberty.

Thyroid hormones are essential for normal pubertal growth, yet the changes in total and, especially, free thyroid hormones and thyroxine-binding globulin during puberty have not been adequately defined. Serum from 39 normal children (20 girls, 19 boys) between the ages of 10 and 15 years were assayed for total T4, free T4, free T3 and thyroxine-binding globulin at 6-monthly intervals; the free hormone assays were valid, non-analogue methodologies. In the girls, free T4 levels fell from 15.7 +/- 0.6 pmol/l at 10 years to 13.0 +/- 0.6 (p less than 0.001) at 12.5 years before rising to 15.9 +/- 0.7 at 15 years; this nadir occurred at puberty stages 3-4. Changes in total T4 followed a similar pattern with a slight delay in the nadir (13 years, puberty stage 4). In the boys, free T4 fell from 16.3 +/- 0.6 pmol/l at 10 years to 14.3 +/- 0.3 at 13.5 years, then rising to 15.4 +/- 0.5 at 15 years; the nadir again occurred at puberty stages 3-4. The corresponding nadir in total T4 which occurred at puberty stages 4-5 was not apparent by age analysis. Thyroxine-binding globulin concentrations remained unchanged in the girls, but fell slightly in the boys during later puberty. Free T3 concentrations in the girls showed a progressive fall after 12.5 years which was significant by the age of 14 when most had been in puberty stage 5 for more than 1 year. The boys showed no change of free T3 concentration throughout the study.(ABSTRACT TRUNCATED AT 250 WORDS)

Nasal polyps: a manifestation of allergy?

The cause of nasal polyps is speculative. In the 1930s nasal polyps were considered to be a manifestation of allergy. This theory was challenged in the 1970s. Many patients with nasal polyps have no identifiable allergic disease. The purpose of this study is to better define the relationship between allergy and nasal polyps. Twelve patients with nasal polyps were evaluated by history, physical examination, skin testing, nasal smear for eosinophils, specific IgE determination from serum, nasal secretions, and nasal polyp fluid. Similar data were collected from three control groups: (1) 10 patients with allergic rhinitis, (2) 10 patients with nonallergic rhinitis, and (3) 10 patients with no known nasal disease. By comparing the incidence of markers for allergic disease in patients with nasal polyposis to each of the three control groups, the role of allergic disease in nasal polyposis was delineated.