Clinical and hormonal response to general anaesthesia in patients affected by different degrees of mental retardation.

BACKGROUND: Poor or no clinical signs of psychological distress are usually observed in patients affected by severe or profound mental retardation (MR). The aim of this study was to use clinical and hormonal parameters in order to compare the amount of stress in patients affected by different degrees of MR undergoing general anaesthesia for dental care. METHODS: Nine patients affected by mild or moderate MR (group A: intellective quotient > 35) and 12 affected by severe or profound MR (group B: intellective quotient < 36), undergoing general anaesthesia for dental care, were studied. The reaction to venipuncture before anaesthesia induction and the recovery from anaesthesia were assessed by clinical scores. Cardiac rate and arterial pressure were recorded before and after venipuncture and after removing the endotracheal tube. Cortisol and prolactin, two hormones affected by stress, were determined in plasma the day prior to surgery (1), after anaesthesia induction (2), and after removing the endotracheal tube (3). RESULTS: During venipuncture, group B was significantly less reactive than group A (p < 0.05) and showed no arterial pressure increase while group A presented a significant increase of systolic pressure; conversely, the groups shared the same hormonal pattern at time 2 (cortisol did not change, prolactin increased significantly). Postoperatively A and B groups did not differ regarding cardiac rate and arterial pressure, while a wider range of postoperative scores was observed in group A in which a further prolactin increase was registered at time 3. CONCLUSIONS: Clinical evaluation can underestimate the stress to which patients affected by severe or profound MR are subjected during anaesthesia induction.

[Evaluation of psychological characteristics in the preoperative phase of patients with mental retardation]. / Valutazione delle caratteristiche psicologiche nella fase prechirurgica dei pazienti con Ritardo Mentale.

OBJECTIVES: To make easier the psychological evaluation of patients affected by Mental Retardation and scheduled for general anesthesia. DESIGN: A form prepared by the Psychological Service of the IRCCS "Oasi Maria SS" in collaboration with the Service of Anesthesia was evaluated prospectively. The form is addressed to relatives and psychologists; the interviewed person is firstly asked for a short description of patient's personality and for any suggestion useful to patient's management; secondly it is requested to assign a score from 1 (never present) to 5 (always present) to 44 items concerning adaptive capacities (divided into body scheme, spoken language, receptive language, mimic-gestural language, temporal orientation, memory), emotional and social fields (including sensibility, dependence, and social behaviors), problematic and stereotyped behaviors. SETTING: A medical and psychological Institute aimed at the study of Mental Retardation. PATIENTS: Twenty-five patients affected by Mental Retardation and scheduled for general and dental surgery. RESULTS: The form supplied a great deal of information and suggestions for patient management. Subjects that did not cooperative with the anesthetist presented lower scores about the ability to use mimic-gestural language (p < 0.05) and higher scores about the presence of anxiety or fear caused by the sight of syringes or blood (p < 0.05) and about behaviors of flight, isolation, or aggression caused by fear (p < 0.05). By contrast, patients presenting mild or moderate degrees of Mental Retardation differentiated from patients with serious degree of Mental Retardation about body scheme (p < 0.001), spoken language (p < 0.05), receptive language (p < 0.001), temporal orientation (p < 0.001) and memory (p < 0.001). CONCLUSIONS: The form was very useful to plan patient management. These data suggest that the lack of cooperation by some patients affected by Mental Retardation is related to fear, anxiety, and incapacity to communicate rather than to the degree of Mental Retardation.

[General anesthesia in Prader-Willi syndrome]. / Anestesia generale nella sindrome di Prader-Willi.

The case of a woman of 27 affected by the Prader-Willi syndrome who underwent general anaesthesia for dental surgery is reported. The patient presented severe mental retardation, small stature, moderate muscular hypotonia, hyperphagia, obesity, and diabetes mellitus. Premedication consisted of diazepam and atropine; anaesthesia was induced with propofol and maintained with propofol, fentanyl and N2O; muscle paralysis was obtained with atracurium. A small glottis was observed at laryngoscopy so that a 6 mm cuffed tube was inserted. Surgery lasted 75 minutes; the patient recovered promptly a few minutes following the end of propofol infusion; no postoperative complication was recorded. As hypoglycemia can occur during and after surgery in the Prader-Willi syndrome, plasma samples for glucose, NEFA, insulin, cortisol, and growth hormone (GH) were collected prior to the induction of anaesthesia (A), 20 minutes after starting surgery (B), at the end of surgery (C), and 3 hours later (D). In spite of the infusion of glucose, hyperglycemia was observed just in C and D samples (A:77; B:88; C:245; D:279 mg/dl). Stable NEFA values, within the normal range, were observed (A:77; B:88; C:245; D:279 mg/dl) suggesting poor or absent lipolysis. Insulin decreased progressively during surgery (A:10.5; B:8.8; C:5.4; D:7.0 mU/L). Cortisol peaked in B (A:9.5; B:20.9; C:13.4; D:4.8 micrograms/dl), suggesting normal hypothalamic reactivity to the surgical stimulus. Finally very low GH levels were observed (A:0.04; B:0.07; C:0.06; D:0.09 ng/ml) suggesting GH deficiency, which had possibly affected the size of patient's glottis. Our data support the hypothesis that hypoglycemia in the Prader-Willi syndrome originates from inadequate lipolysis during starvation.

[General anesthesia in Cohen syndrome. Report of a clinical case]. / Anestesia generale nella sindrome di Cohen. Descrizione di un caso clinico.

The case of a 23 years old woman, affected by the Cohen syndrome, who underwent general anesthesia for extensive dental surgery, is reported. The Cohen syndrome is an autosomal recessive syndrome that causes mental retardation, obesity, short stature as well as oral, ocular, and limb anomalies. The problems the anesthesiologist could deal with include the capacity of the patient to cooperate; difficult intubation because of maxillary hypoplasia, micrognathia, narrow and high-arched palate, and prominent maxillary central incisors; generalized muscular hypotonia; moderate leukopenia, that could theoretically increase the risk of infection: and, finally, possible associated mitral valve prolapse or hiatus hernia. In the case reported the presence of mitral valve prolapse or hiatus hernia was ruled out echographically. The patient was premedicated with diazepam and atropine i.m.; general anesthesia was carried out by propofol-fentanyl association and myorelaxation was obtained with atracurium. Nasotracheal intubation was performed easily in spite of oral anomalies so that the usefulness of thyromental distance, which was 7 cm long, as a clinical test to evaluate a potentially difficult intubation was confirmed. Noteworthy, the thyromental distance was the only test which was suitable for the uncooperative patient. At the end of surgery muscular tone recovered promptly and the endotracheal tube could be regularly removed. No complication was registered postoperatively.