Anestesia en paciente pediátrico con síndrome de Rohhad / Anesthesia in a pediatric patient with ROHADD syndrome

El síndrome obesidad de rápida progresión, hipoventilación alveolar, disfunción hipotalámica y disregulación autonómica (ROHHAD) es una entidad infrecuente caracterizada por un comienzo en niños sanos a los 2-4 años. Se trata de un síndrome complejo caracterizado por una rápida ganancia de peso con hiperfagia, disfunción hipotalámica, hipoventilación central y disregulación autonómica, entre otros síntomas. Presentamos el caso de un niño de 10 años con diagnóstico de síndrome de ROHHAD a quien se colocó un porth-a-cath bajo anestesia general y que desarrolló complicaciones durante el procedimiento anestésico relacionadas con su enfermedad. El manejo perioperatorio de estos pacientes supone todo un reto para el anestesista dada la afectación de múltiples sistemas y las frecuentes comorbilidades respiratorias que asocian. Se resumen algunas de las implicaciones y consideraciones anestésicas que hay que tener en cuenta en el manejo de estos pacientes

Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome is a rare entity that is characterised by its onset in healthy children at 2-4 years of age. It is a complex syndrome that includes, among other symptoms, rapid weight gain with hyperphagia, hypothalamic dysfunction, central hypoventilation, and autonomic dysregulation. The case is presented of a 10-year-old boy with a diagnosis of ROHHAD syndrome undergoing insertion of a port-a-cath under general anaesthesia, who developed complications during the anaesthetic procedure related to his illness. The peri-operative management of these patients represents a challenge for the anaesthetist, given the involvement of multiple systems and the frequent respiratory comorbidities associated with them. A summary is presented of some of the implications and anaesthetic considerations that must be taken into account in the management of these patients

Postoperative Complications in Patients With Unrecognized Obesity Hypoventilation Syndrome Undergoing Elective Noncardiac Surgery.

BACKGROUND: Among patients with OSA, a higher number of medical morbidities are known to be associated with those who have obesity hypoventilation syndrome (OHS) compared with OSA alone. OHS can pose a higher risk of postoperative complications after elective noncardiac surgery (NCS) and often is unrecognized at the time of surgery. The objective of this study was to retrospectively identify patients with OHS and compare their postoperative outcomes with those of patients with OSA alone. METHODS: Patients meeting criteria for OHS were identified within a large cohort with OSA who underwent elective NCS at a major tertiary care center. We identified postoperative outcomes associated with OSA and OHS as well as the clinical determinants of OHS (BMI, apnea-hypopnea index [AHI]). Multivariable logistic and linear regression models were used for dichotomous and continuous outcomes, respectively. RESULTS: Patients with hypercapnia from definite or possible OHS and overlap syndrome are more likely to experience postoperative respiratory failure (OR, 10.9; 95% CI, 3.7-32.3; P < .0001), postoperative heart failure (OR, 5.4; 95% CI, 1.9-15.7; P = .002), prolonged intubation (OR, 3.1; 95% CI, 0.6-15.3; P = .2), postoperative ICU transfer (OR, 10.9; 95% CI, 3.7-32.3; P < .0001), and longer ICU (?-coefficient, 0.86; SE, 0.32; P = .009) and hospital (?-coefficient, 2.94; SE, 0.87; P = .0008) lengths of stay compared with patients with OSA. Among the clinical determinants of OHS, neither BMI nor AHI showed associations with any postoperative outcomes in univariable or multivariable regression. CONCLUSIONS: Better emphasis is needed on preoperative recognition of hypercapnia among patients with OSA or overlap syndrome undergoing elective NCS.

A case of obesity hypoventilation syndrome with respiratory failure that improved with abdominoplasty.

We report on a 70-year-old man with severe respiratory failure caused by obesity hypoventilation syndrome due to abdominal adiposis. Obesity hypoventilation syndrome is a severe condition that is diagnosed when all of the following criteria are satisfied: body-mass index >30 kg/m(2); apnea hypopnea index >30; PaCO2 >45 mm Hg (in the daytime); and marked daytime somnolence. Abdominoplasty, which is generally used for abdominal laxness, striae, and rectus muscle diastases and for women in the postpartum period, was performed for this patient to facilitate ventilator weaning and produced a satisfactory result.

Emerging risk factors and prevention of perioperative pulmonary complications.

Modern surgery is faced with the emergence of newer "risk factors" and the challenges associated with identifying and managing these risks in the perioperative period. Obstructive sleep apnea and obesity hypoventilation syndrome pose unique challenges in the perioperative setting. Recent studies have identified some of the specific risks arising from caring for such patients in the surgical setting. While all possible postoperative complications are not yet fully established or understood, the prevention and management of these complications pose even greater challenges. Pulmonary hypertension with its changing epidemiology and novel management strategies is another new disease for the surgeon and the anesthesiologist in the noncardiac surgical setting. Traditionally most such patients were not considered surgical candidates for any required elective surgery. Our review discusses these disease entities which are often undiagnosed before elective noncardiac surgery.

Hypoxemia and hypoventilation syndrome improvement after laparoscopic bariatric surgery in patients with morbid obesity.

BACKGROUND: The objective of this study was to evaluate the relationship between oxygen partial pressure (pO(2)), awake oxymetric saturation (SpO(2)), body mass index (BMI), and percentage of excess weight loss (EWL) in extremely severe obesity (BMI >50 kg m(-2)) and hypoxemia, before and after laparoscopic Roux-en-Y gastric bypass. PATIENTS AND METHODS: A group of 11 obese patients aged 41.2 + or - 10.2 years (4 men, 7 women, median BMI=52.3 kg/m(2), range 50.2-57.1) were prospectively enrolled in the study. BMI, arterial blood gas measurements, and spirometry were obtained before and after (6 and 12 months) surgery. RESULTS: The main preoperative parameters were SpO(2)=88.3 + or - 3.9%, predicted forced vital capacity (FVC)=84.5 + or - 8.3%, predicted forced expiratory volume exhaled in one second (FEV1)=79.9+/-10.1%. No relationship (p>0.01) was found between BMI, SpO(2), and FEV1. A significant correlation between SpO(2) and both paO(2) (R=0.74, p=0.009) and EWL (R=-0.75, p=0.008) was found. Three, 6, and 12 months after surgery EWL was 18.9%, 26.4%, and 39.6% (p<0.001), respectively. At one-year follow-up SpO(2), FVC, and FEV1 were 96.2 + or - 3.2% (p<0.001), 112.3 + or - 9.9% (p<0.001), and 101.6 + or - 18.8% (p=0.003), respectively. CONCLUSION: In patients with extremely severe obesity, bariatric surgery may improve significantly both SpO(2) and spirometric parameters, and EWL represents the factor that impacted the results.

Early mortality rate of morbidly obese patients after tracheotomy.

OBJECTIVES: To 1) determine the early mortality rate (within 30 days) of morbidly obese patients after tracheotomy; 2) determine the difference between the mortality rate after tracheotomy of morbidly obese patients and patients who are not morbidly obese; and 3) determine the difference between the mortality rate after tracheotomy adjusted for case mix index (CMI) of morbidly obese patients and patients who are not morbidly obese. STUDY DESIGN: Retrospective cohort study of 278 patients who had undergone a tracheotomy by the otolaryngology head and neck surgery department from 2004 to 2006. The patients were subdivided into two groups: 1) body mass index (BMI) <35 (n = 229) and 2) BMI > or =35 (morbidly obese) (n = 49). METHODS: Charts reviewed for age, sex, weight, height, BMI, indication for tracheotomy, date of tracheotomy, type of tracheotomy, date of discharge, date of death, length of hospital stay, and CMI. RESULTS: There is a trend toward significance (P = .09) between the mortality rate after tracheotomy of morbidly obese patients (29%) and patients who are not morbidly obese (18%). There is less significance between the adjusted mortality rate based on CMI after tracheotomy when the patient population is divided into morbidly obese patients and patients who are not morbidly obese (P = .12). CONCLUSION: The mortality rate after tracheotomy of morbidly obese patients is greater than patients who are not morbidly obese.

[Many persons with snoring problems and apnea are untreated. A review of therapeutic methods]. / Många snarkare och apnoiker obehandlade. Genomgång av terapimetoderna.

As sleep apnoea and snoring are very disabling conditions both for patients and their families, and hazardous for drivers and others in traffic, there is good reason to treat snoring problems. Treatment should be individualised, always beginning conservatively--i.e., positional training, weight reduction if necessary, more sleep if sleep deficiency is present, and a review of any muscle-relaxant or mucolytic medication. Sleep registration will demonstrate the extent of any sleep apnoea syndrome, which is of decisive importance for further choice of treatment. Mild apnoics and social snorers may initially be offered an occlusal splint if their dental status allows. Otherwise, in such cases surgery is a form of treatment yielding immediate results, though the patient must be forewarned of the discomfort which can occur in isolated cases. For patients with sleep apnoea syndrome of marked or intermediate severity, continuous positive airway pressure (CPAP) treatment should be available. If the patient can not tolerate CPAP treatment, the occlusal splint alternative can be tried. For patients who can not have CPAP or occlusal splint treatment, tracheostomy is a possibility. This treatment may be lifelong, but if weight reduction is achieved postoperatively, it may be possible to remove the tracheostomy.

[Late results of the treatment of patients with the extreme degree of alimentary-constitutional obesity by the method of formation of a small stomach]. / Otdalennye rezul'taty lecheniia bol'nykh s krainymi stepeniami alimentarno-konstitutsional'nogo ozhireniia metodom formirovaniia malogo zheludka.

The work analyses the results of treatment of 311 patients with extreme degrees of alimentary-constitutional obesity by formation of a small stomach. Fatal outcomes (1.9%) were encountered in the period of operative technique mastering. The late-term results were studied in 167 patients in follow-up periods of up to 3 years. The patients' average body weight was 149.4 kg, average height 166.2 cm, average body weight excess as compared to the ideal weight was 125.6%. Study of the late-term results of the operation showed that the postoperative weight loss depends on the initial weight excess and the diameter of the anastomosis formed between the proximal and distal parts of the stomach. The more the initial excess of weight as compared to the ideal value, the more the loss of body weight is. The diameter of the formed anastomosis should be no larger than 15 mm. Besides loss of weight, the activity of vital organs and systems is normalized after the operation, and arterial hypertension, diabetes mellitus, the Pickwickian syndrome, and metabolic polyarthritis take a milder course. The operation for formation of a small stomach made it possible for the patients to resume their customary occupation, freed them of the threat of invalidation, and reduced the duration of the disability period by 4.3 times. After surgical treatment the nature of the patients' life significantly improved; 95.8% of patients appraised the effect of the treatment as excellent and good.

Airway management in obesity hypoventilation syndrome.

The role of tracheostomy is limited in the obesity hypoventilation syndrome unless severe upper airway obstruction exists. If it is performed, special techniques must be applied to overcome the problems associated with tracheostomy in the morbidly obese patient. If attention is paid to these details, however, tracheostomy provides clinically important benefits in this difficult clinical situation.

Therapeutic effects of tracheostomy in two cases of hypersomnia with respiratory disturbance during sleep.

Recently the association of hypersomnia and respiratory insufficiency without lesion in the respiratory organ has attracted attention of many investigators. Obese patients with such a condition have been called the Pickwickian syndrome. In this report, two non-obese patients with a similar condition were presented, one with micrognathia and frequent apneic episodes during sleep, and the other with laryngeal stenosis due to paralysis of the bilateral laryngeal nerves and chronic laryngitis. Tracheostomy had a prompt and long-lasting therapeutic effect to make their sleep stable and also to relieve their excessive daytime sleepiness. These findings suggest that the obstruction or stenosis of the upper airway during sleep disturbed their nocturnal sleep, and that their excessive daytime sleepiness was a phenomenon compensating for their disturbed nocturnal sleep.

Complications of obesity-hypoventilation syndrome in childhood.

The obesity-hypoventilation syndrome is rare in children, but it leads to serious complications and is associated with a high mortality. We report a child with this syndrome whose condition improved after intestinal bypass surgery. Review of the literature indicates that vigorous treatment of this disorder is necessary to prevent fatalities.

The Pickwickian syndrome with hypertrophy of tonsils: a re-appraisal.

Pronounced tonsilar hypertrophy was found in two obese patients suffering from hypersomnolence, periodic attacks of apnea and disturbing snoring at night. Both patients underwent tonsillectomy. Immediately after the operation the hypersomnolence disappeared, the breathing became normal, and the disturbing snoring at night ceased. Follow-up over a period of three years did not reveal any recurrence of these symptoms, even though the patients had not lost any weight during this period.