[Costello syndrome associated to a neuroblastoma. Presentation of a case]. / Síndrome de Costello asociado a neuroblastoma. Presentación de un caso.

We present the case of a newborn with Costello syndrome who died due to heart arrhythmia. In the autopsy, a neuroblastoma was found. The male patient was born at term. During the first hours of life, he developed severe respiratory failure requiring mechanical ventilation. Phenotypic features included cranial and facial dysmorphia, short thorax, tachycardia, heart murmur, abdominal distention, hepatomegaly, short extremities, widespread petechias, diminished muscular tone, ungueal hypoplasia in toes, bilateral cryptorchidia, and generalized redundant skin. In the evolution he presented several sepsis episodes, difficulty for feeding, supraventricular arrhythmia, two heart arrests, and opisthotonos, and died at 65 days of life due to heart arrhythmia. The autopsy revealed hydrocephaly, a neuroblastoma, and a heart without anatomic alterations. Costello syndrome was diagnosed. Costello syndrome is not frequent; in this patient, the diagnosis was suspected in life and was confirmed postmortem, the topic is reviewed, the important aspect in this case is the association with a neuroblastoma.

The effects of the M40 protective mask on sleep.

There are very few data available on the effects of respiratory protective masks on sleep. This study was to undertaken to investigate the effects of the M-40 protective mask on sleep architecture. We asked 21 healthy volunteers (18-35 yr) to participate in a two-night study. During the first night, subjects underwent standard polysomnography without the M40 mask. The second night entailed sleeping while wearing the mask, without the hood. Specific variables measured on each night included sleep stage distribution, time awake, number of awakenings, oxygen saturation, number of apneas and hypopneas, and time to sleep onset. Statistical analysis was performed using a two-tailed, paired t-test for all variables except REM sleep, for which a one-tailed, paired t-test was used. There was a significant difference between mask and non-mask nights when measuring total sleep time, total awake time, percentage of time in REM, and number of awakenings. It is concluded that sleep in the M40 mask is disrupted compared to sleep without the mask, with significant decreases in total sleep time and REM sleep.

Effects of topical oral antiseptic rinses on bacterial counts of saliva in healthy human subjects.

Wound infections remain a significant source of morbidity in patients undergoing major head and neck operations that invade the aerodigestive tract. Infection rates have been significantly reduced by the administration of perioperative intravenous antibiotics; however, the incidence of infection remains unacceptably high. This study was undertaken to help identify an oral antiseptic that could significantly reduce the bacterial colony count of human saliva. A randomized, prospective clinical trial was conducted to analyze and compare the effects of Listerine antiseptic and Peridex oral rinse on the aerobic and anaerobic bacterial counts in healthy human subjects. Thirty healthy adult volunteers between the ages of 18 and 61 participated in the study. The patients were randomized to receive normal saline solution, Listerine antiseptic, or Peridex oral rinse. Aerobic and anaerobic bacterial colony counts of saliva were measured before treatment and at 1 and 4 hours after treatment. Both Listerine antiseptic and Peridex oral rinse significantly reduced bacterial counts at 1 hour after treatment in our volunteers. At 4 hours after treatment, Peridex oral rinse showed a further reduction in the bacterial colony count whereas Listerine antiseptic showed no difference compared with normal saline solution. At 4 hours after treatment, Peridex oral rinse reduced the total bacterial colony count by 85%.

Gender and racial variations in cephalometric analysis.

Cephalometric analysis has earned a vital role in the evaluation of obstructive sleep apnea. However, the normal measurements cited in the literature are not sex or race specific. Skeletal differences in sexes and races have been established. This study was initiated to examine whether race and sex variations in soft tissue and skeletal measurements exist in cephalometric analysis. A total of 89 volunteers of different race and sex participated in this study. The data support the hypothesis that there are statistically significant differences in (1) sella-nasion-subspinale angle (SNA) between black men, and both Caucasian and Hispanic men, (2) sella-nasion-supramentale angle (SNB) between black men and Caucasian men, (3) posterior airway space between Caucasian men and women, and (4) mandibular plane to hyoid distance between Caucasian men and women. These data suggest that only SNA and SNB need racial specificity. Furthermore, Caucasian women need a separate set of normal values from men, specifically posterior airway space and mandibular plane to hyoid bone.

Pediatric thyroid carcinoma in the military population.

A 30-year review of the Armed Forces Tumor Registry revealed that of 3,126 thyroid cancers on file, 82 were in the pediatric population. All of these cases were reviewed, and the mean age was found to be 13.8 years. There as a male-to-female ratio of 1:2.3. At the time of diagnosis, 33% had regional nodes and 6% had distant metastasis. The malignancies were 60% papillary, 13% follicular, 10% papillary-follicular variant, and 6% medullary. Treatment included 41% lobectomies with or without isthmectomy, 27% total thyroidectomies with or without node sampling, 20% total thyroidectomies with neck dissections, and 12% surgical treatment not otherwise specified. Of the surgical patients, 30% received postoperative 131I ablation. An average patient follow-up of 8.6 years showed that 65 remained disease-free, 5 had disease, 1 was dead of disease, and 10 were unknown. The results of our pediatric thyroid carcinoma review are compared with previously published figures.

Warthin's tumor and cigarette smoking.

Warthin's tumor is a relatively uncommon salivary gland neoplasm, traditionally considered a disease of men. Recent reports have brought this assumption into question by identifying a substantial percentage of patients who are women. Furthermore, several reports have attempted to correlate cigarette smoking with development of Warthin's tumor. We retrospectively studied all cases of parotid tumor entered in the Brooke Army Medical Center Tumor Registry from 1973 to 1993. Relative percentages of benign and malignant disease, as well as frequency of individual tumor types, in general mirrored those in earlier reports. A high proportion of Warthin's tumor-23% of all tumors-was identified. More than one third of patients with Warthin's tumor were women. Also, 94% of patients with Warthin's tumor had a history of tobacco use; for all other tumor types combined, only 60% of patients had a history of tobacco use. This statistical difference supports the correlation between cigarette smoking and Warthin's tumor.

Eagle's syndrome (elongated styloid process)

Eagle's syndrome occurs when an elongated styloid process or calcified stylohyoid ligament causes recurrent throat pain or foreign body sensation, dysphagia, or facial pain. Additional symptoms may include neck or throat pain with radiation to the ipsilateral ear. In adults, the styloid process is approximately 2.5 cm long, and its tip is located between the external and internal carotid arteries, just lateral to the tonsillar fossa. It may develop inflammatory changes or impinge on the adjacent arteries or sensory nerve endings, leading to the symptoms described. Diagnosis can usually be made on physical examination by digital palpation of the styloid process in the tonsillar fossa, which exacerbates the pain. In addition, relief of symptoms with injection of an anesthetic solution into the tonsillar fossa is highly suggestive of this diagnosis. Radiographic workup should include anterior-posterior and lateral skull films. The treatment of Eagle's syndrome is primarily surgical. The styloid process can be shortened through an intraoral or external approach. We present two cases and review the literature.

Inferior sagittal osteotomy with hyoid bone suspension for obese patients with sleep apnea.

BACKGROUND: An inferior sagittal osteotomy with a sliding mandibular osteotomy and hyoid bone suspension, combined with a uvulopalatopharyngoplasty, is advocated in patients with obstructive sleep apnea who fail to tolerate nasal continuous positive airway pressure. Some investigators have suggested that this surgical procedure may be less effective in obese patients. METHODS: Preoperative and postoperative nocturnal polysomnograms and lateral cephalometric radiographs were obtained on 12 obese patients with obstructive sleep apnea who underwent these procedures. RESULTS: The mean +/- SD respiratory disturbance index during sleep decreased 53% +/- 11% after surgical procedures were performed (49 +/- 17 vs 23 +/- 11 events per hour, P < .001). Surgical outcome did not correlate with the body mass index, and 5 (71%) of 7 of the morbidly obese patients (body mass index, > or = 33) had greater than a 50% reduction in the respiratory disturbance index after the surgical procedures were done. CONCLUSION: An inferior sagittal osteotomy with a sliding mandibular osteotomy and hyoid bone suspension, combined with a uvulopalatopharyngoplasty, tends to improve obstructive sleep apnea in obese patients.

Laryngoceles and saccular cysts.

Laryngoceles and saccular cysts are related, benign abnormalities of the larynx. The pathophysiology of both lesions can involve congenital as well as acquired factors, and the appearance of both may range from incidental findings on laryngoscopy to symptoms such as hoarseness, dysphagia, dyspnea, and laryngeal discomfort. The surgical techniques used for treatment of laryngoceles and saccular cysts are controversial. We present two cases for comparison and discuss the history, diagnosis, and surgical treatment of laryngoceles and saccular cysts. Emphasis is on the surgical approach, which in both cases transected the thyrohyoid membrane externally and provided excellent visualization and exposure without significant morbidity.

Comparison of cephalometric analysis with ethnicity in obstructive sleep apnea syndrome.

Many studies have documented significant craniomandibular abnormalities in obstructive sleep apnea syndrome (OSAS) patients. Recent literature clearly describes the cephalometric abnormalities commonly associated with OSAS. Studies have not evaluated specific cephalometric abnormalities that may contribute to OSAS by various ethnic groups. Data were collected on 48 patients (20 Caucasian, 15 Black and 13 Hispanic) with completed cephalometric analysis and polysomnography. Cephalometric landmarks, angles and measurements [angle measured from sella to nasion to subspinale point (SNA), angle measured from sella to nasion to supramentale point (SNB), difference between SNA and SNB (ANB), perpendicular distance from gonion to gnathion to hyoid (MP-H), distance from posterior nasal spine to tip of soft palate (PNS-P) and posterior airway space (PAS)] commonly used in the evaluation of OSAS patients were recorded. Measurements were normalized by dividing the observed value by the mean value for the ethnic group. Statistically significant differences in normalized SNA and SNB appeared in the Black and Hispanic groups when compared to the Caucasian group. For both SNA and SNB, Blacks averaged approximately 3.5% above their ethnic mean, whereas Hispanics averaged 1.8-2.8% below their ethnic mean. There was a statistically significant correlation between respiratory distress index (RDI) and MP-H. These baseline cephalometric differences in the ethnic groups studied suggest that surgical intervention might be approached differently in various ethnic groups. Further studies that evaluate the surgical success achieved by various procedures among different ethnic groups may help define surgical protocol in various ethnic groups for OSAS.

The influence of reserpine on the responsiveness to norepinephrine and electrolyte contents of rabbit atria.

This study was designed to investigate the relationships between reserpine-induced supersensitivity and electrolyte levels in isolated rabbit atria. Atria from reserpine-treated (1, 3 or 4 mg/kg 24 hr before) and untreated rabbits were placed in an isolated organ bath for determination of dose-response relationships for norepinephrine, or were used for electrolyte assay. Reserpine-treated atria had greater resting and maximum rates of beat than did untreated atria. However, the ED50 concentrations of norepinephrine producing a rate response were similar. Atria pretreated with 1 or 3 mg/kg reserpine were supersensitive to the inotropic effect of norepinephrine. 4 mg/kg reserpine did not induce inotropic supersensitivity. Atrial sodium contents were significantly increased by 3 mg/kg reserpine. Potassium contents were increased by 1 and 4 mg/kg reserpine. Calcium contents were significantly decreased by 4 mg/kg reserpine, while magnesium contents were increased by 1 and 4 mg/kg. Reserpine induced supersensitivity to the inotropic effects of norepinephrine but did not alter the sensitivity to the chronotropic effect of the drug. This supersensitivity may be linked to alterations in the tissue's homeostatic mechanisms for calcium.