Spinal anesthesia in a child with Brachmann-de Lange (Cornelia de Lange) syndrome

Kachko, Ludmyla; Sanko, Elena; Freud, Enrique; Katz, Jacob

doi:10.1007/s00540-010-1026-y

Cited by 4 publications

(4 citation statements)

References 19 publications

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“…Prior limited case reports/small series describe successful use of awake SA for NCS in pediatric patients with CHD (13)(14)(15)(16)(17). The major concern in the use of SA in patients with CHD is the potential for adverse effects on hemodynamic function.…”

Section: Discussionmentioning

confidence: 99%

Spinal anesthesia for noncardiac surgery in infants with congenital heart diseases

Kachko

Birk

Simhi

et al. 2011

Pediatric Anesthesia

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Spinal anesthesia (SA) in pediatrics began to be used in the late nineteenth century in multiple procedures, with priority for high-risk and former preterm infants, for its suggested protective role compared to the development of postoperative apnea with general anesthesia (GA). In children, higher doses of local anesthetics are required with a shorter duration of action and a greater hemodynamic stability compared to adults. The puncture must be performed in the L4-L5 or L5-S1 spaces to prevent spinal injuries. The practice of SA in pediatric patients requires skill and experience; failure rates of up to 28% have been reported. The drugs most commonly used for SA are tetracaine and bupivacaine alone or with adjuvants. SA complications are rare and often without consequences, except for postdural puncture headaches and backaches. Although SA is today considered safe and effective for pediatric patients, it remains relatively underutilized compared to GA.

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Section: Discussionmentioning

confidence: 99%

Spinal anesthesia for noncardiac surgery in infants with congenital heart diseases

Kachko

Birk

Simhi

et al. 2011

Pediatric Anesthesia

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“…Previously, several single case reports [Takeshita et al, ; Tsukazaki et al, ; August and Sorhabi, ; Kachko et al, ; Washington and Kaye, ] and a case series [Moschini et al, ] documented the anesthetic management of CdLS patients. Most of these reports dealt with pediatric patients.…”

Section: Discussionmentioning

confidence: 99%

“…We do not have knowledge of previous works documenting the use of sedation and procedural anesthesia in CdLS patients. Indeed, previous works documented the use of general anesthesia, while a recent case report showed the use of regional anesthesia for a CdLS patient [Kachko et al, ]. In our institution, we chose sedation rather than general anesthesia for several reasons: (i) frequently just a brief period of immobility is necessary to perform the required surgical procedure; (ii) most of the procedures are performed in a day‐surgery regimen and patients are dismissed just a few hours after the interventions; (iii) the patients’ airways are particularly prone to bronchospasm following manipulation [Takeshita et al, ].…”

Section: Discussionmentioning

confidence: 99%

Sedation and general anesthesia for patients with Cornelia De Lange syndrome: A case series

Moretto¹,

Scaravilli

Ciceri

et al. 2016

American J of Med Genetics Pt C

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Cornelia De Lange syndrome (CdLS) is a rare congenital disease characterized by typical facial dysmorphism, developmental disability, and limb deficiency defects. Various congenital malformations and medical complications have been described with gastroesophageal reflux as the major one. CdLS patients often require multiple high-risk anesthetic procedures. At San Gerardo Hospital (Monza, Italy) the management of CdLS patients is routinely organized through a standard protocol and a dedicated pediatric anesthesia team has been implemented. We report on a retrospective descriptive analysis of the anesthetic records of the CdLS patients admitted to San Gerardo Hospital from January 2010 to December 2015. We retrieved: demographics, genetic profiles, type of procedures, anesthetic approaches, anesthetics usage and complications. Data are reported as median (interquartile range) values. Twenty-seven patients (11 female), with age 12 (7-15) years old, weight 24 (14-35) kg, and severity score of 25 (18-32) were included. NIBPL mutations were the most frequently represented. We analyzed 58 procedures (30 esophagogastroduodenoscopies, 8 evoked auditory potential tests, 5 radiodiagnostics, 5 catheters positioning, 4 bronchoscopies) managed by sedation (36) and general anesthesia (6). Each patient underwent one (1-2) anesthetic procedure. Propofol (59%), sevoflurane (31%), fentanyl (24%), and ketamine (10%) were used. Three out of six endotracheal intubations were difficult. The only documented intraoperative complications were three episodes of desaturation (oxygen saturation <90%) occurring during sedations and were managed without the need for an invasive control of the airways. Implementation of a specific management protocol and a dedicated allowed to provide anesthesia to CdLS patients without the occurrence of major complications. © 2016 Wiley Periodicals, Inc.

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“…It is generally sporadic, but can also be dominant and recessive (1). Problems such as difficult intubation, gastroesophageal reflux, aspiration, and hyperthermia make the airway safety more difficult in general anesthesia practices (2). We aimed to present our anesthesia management experience with a patient having CdLS who was operated because of undescended testis and circumcision after taking the consent of his family.…”

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confidence: 99%

Anesthetic Management of Cornelia de Lange Syndrome: A Case Report

Şen¹,

Calapoglu²,

Sen³

2015

Erciyes Med J

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Dear Editor, Cornelia de Lange syndrome (CdLS) is described as a congenital disease characterized by delayed growth, mental-motor retardation, typical face appearance, thick eyebrows meeting at the midline, micrognathia, thin lips, micromelia, dental problems, and malformations in the systems such as cardiac, gastrointestinal, genitourinary, and musculoskeletal systems. It is rarely seen, with a reported incidence of 1 in 10,000 to 40,000. It is generally sporadic, but can also be dominant and recessive (1). Problems such as difficult intubation, gastroesophageal reflux, aspiration, and hyperthermia make the airway safety more difficult in general anesthesia practices (2). We aimed to present our anesthesia management experience with a patient having CdLS who was operated because of undescended testis and circumcision after taking the consent of his family.The patient with a diagnosis of CdLS was 4 years of age, 12 kg in weight, and 85 cm in height. The operation was planned for undescended testis and circumcision. The preoperative examination revealed a history of retarded development, speech impediment, atrial septal defect (ASD), frequent upper respiratory tract infection, conjunctivitis, and food allergy. He had the findings of small face size, restricted mouth opening, short nose, and micrognathia, which could make intubation difficult in general anesthesia. No finding was available in the cardiovascular system, except ASD. The history of infections recurring frequently in the respiratory system constituted a perioperative risk for the patient. The patient who would be operated for circumcision and undescended testis was decided to undergo caudal anesthesia and sedation. However, alternative instruments were kept available in order to maintain airway safety in case of an unsuccessful block. After administering endocarditis prophylaxis, he was taken to the operation room, 1 mg of intravenous midazolam was injected, and he was monitored in the standard manner. Then, sedation was deepened using 10 µg of fentanyl and 20 mg of propofol, and the patient was given lateral decubitus position. After providing aseptic conditions, 12 cc of 2% levobupivacaine was given to the caudal space. The operation was initiated when no pain was observed with the pinprick test after waiting for about 30 min. Anesthetic gases were not used in order to avoid upper airway irritation. The patient was monitored for a while after the 30-min operation and transferred to the clinic because no complication developed.In CdLS, typical facial appearance is characteristic. The findings, including thick eyebrows that often meet at the midline, microcephalia, micrognathia, long eyelashes, small face, high-arched palate, and short nose, are also indicators of difficult intubation (1).In patients with CdLS, the risk for gastroesophageal reflux and associated aspiration pneumonia is high (3). In our case, the existence of food allergy and recurring upper respiratory tract infections were signs of an irritable airway. We avoided the application of g...

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Spinal anesthesia in a child with Brachmann-de Lange (Cornelia de Lange) syndrome

Cited by 4 publications

References 19 publications

Spinal anesthesia for noncardiac surgery in infants with congenital heart diseases

Spinal anesthesia for noncardiac surgery in infants with congenital heart diseases

Sedation and general anesthesia for patients with Cornelia De Lange syndrome: A case series

Anesthetic Management of Cornelia de Lange Syndrome: A Case Report

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