Outcome of velopharyngoplasty in patients with velocardiofacial syndrome

Objective: To compare the outcomes of surgical correction of velopharyngeal insufficiency (VPI) in patients with velocardiofacial syndrome (VCFS) and a non-VCFS group. Design: Twenty-five patients with VCFS (16 girls and 9 boys) underwent palatal lengthening for VPI between 1986 and 2001. The mean age at surgery was 6.4 years. Revision was defined as the need for secondary sphincter pharyngoplasty as determined by speech investigation, nasal endoscopy, and acoustic nasometry. A comparison was made to a control group made up of a randomized group of patients without VCFS who underwent palatal lengthening for VPI (32 patients: 10 girls and 22 boys). Setting: Wilhelmina Children's Hospital, a tertiary referral center in Utrecht, the Netheralands. Patients: A total of 57 patients who underwent palatal lengthening for VPI, 25 with VCFS and 32 without VCFS. Interventions: Primary surgery consisted of a palatal lengthening technique. If revision was needed, a sphincter pharyngoplasty was carried out. Main Outcome Measures: Pharyngeal function was assessed using perceptual speech investigation, nasal endoscopy, and acoustic nasometry. Results: In the VCFS group, 16% of the patients required surgical revision (4 of 25). These patients were slightly older at the time of primary surgery than those who did not require surgical revision (mean age, 6 vs 5.5 years). In the control group, no patients required revision. Preoperative speech analysis showed a more pronounced VPI in the VCFS group than in the control group. Outcomes of endoscopy and speech hypernasality improved significantly more in the control group than in the VCFS group. Improvement in the results of acoustic nasometry did not differ significantly between the 2 groups. Conclusions: Treatment of VPI using palatal lengthening in children with VCFS is both safe and effective. The discrepancy in improvement between the speech analysis and the nasal endoscopy results within the VCFS group indicates that mechanical improvement does not necessarily correspond to an improvement in speech and emphasizes the complexity of speech disorders found in VCFS. ©2008 American Medical Association. All rights reserved

Outcome of Velopharyngoplasty in Patients With Velocardiofacial Syndrome

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Factors influencing blood loss and allogeneic blood transfusion practice in craniosynostosis surgery

Objective/Aims: To identify factors influencing perioperative blood loss and transfusion practice in craniosynostotic corrections. Background: Craniosynostotic corrections are associated with large amounts of blood loss and high transfusion rates. Methods: A retrospective analysis was performed of all pediatric craniosynostotic corrections during the period from January 2003 to October 2009. The primary endpoint was the receipt of an allogeneic blood transfusion (ABT) during or after surgery. Pre-, intra-, and postoperative data were acquired using the electronic hospital registration systems and patients' charts. Results: Forty-four patients were operated using open surgical techniques. The mean estimated blood loss during surgery was 55 ml·kg -1. In 42 patients, red blood cells were administered during or after surgery with a mean of 38 ml·kg -1. In 23 patients, fresh frozen plasma was administered with a mean of 28 ml·kg -1. A median of two different donors per recipient was found. Longer duration of surgery and lower bodyweight were associated with significantly more blood loss and red blood cell transfusions. Higher perioperative blood loss and surgery at an early age were correlated with a longer duration of admission. Conclusions: In this study, craniosynostotic corrections were associated with large amounts of blood loss and high ABT rates. The amount of ABT could possibly be reduced by appointing a dedicated team of physicians, by using new less-invasive surgical techniques, and by adjusting anesthetic techniques. © 2011 Blackwell Publishing Ltd

Minimally Invasive Strip Craniectomy Simplifies Anesthesia Practice in Patients With Isolated Sagittal Synostosis

Background: Traditional open corrective surgery for isolated sagittal synostosis entails significant blood loss, transfusion rates, morbidity, and a lengthy hospitalization. Minimally invasive strip craniectomy (MISC) was introduced to avoid the disadvantages of open techniques. Objectives: The aim of the study was, first, to compare the anesthesia practice in MISC and open extended strip craniectomy (OESC), and, second, to evaluate the incidence of perioperative complications in both surgical procedures. Methods: A retrospective analysis was conducted for all consecutive patients receiving either OESC or MISC for nonsyndromic isolated sagittal synostosis between January 2006 and February 2014. The primary endpoints were the volume of blood loss, the volume of infused blood products, the duration of surgery, the anesthesia time, the intubation time, and the length of admission to high care units and the hospital. Results: In MISC, the median duration of surgery (90 versus 178 min.), anesthesia time (178 versus 291 min), and intubation time (153 versus 294 min) were all significantly (P < 0.001) shorter than in OESC. Intraoperative blood loss was less in MISC than in OESC (3.8 versus 29.7 mL/kg, P < 0.001), requiring less crystalloids (33.3 versus 76.9 mL/kg, P < 0.001) as well as less erythrocyte transfusions (0.0 versus 19.7 mL/kg, P < 0.001) in a smaller number of patients (2/20 versus 13/15). The improved hemodynamic stability in MISC allowed for placement of less arterial and central venous catheters. After OESC all 15 patients were admitted to high care units, compared with 9 of 20 in MISC. The overall median hospital stay was shorter in MISC than in OESC (4 versus 6 d, P < 0.001). Although the incidence of technical complications was similar in both techniques, patients in MISC were less affected by perioperative electrolyte and acid-base disturbances and postoperative pyrexia. Conclusions: Minimally invasive strip craniectomy simplifies anesthesia practice relative to OESC with shorter operative times, decreased needs for replacement fluids and blood products, lessened requirements for invasive monitoring, and reduced demands for postoperative high care beds