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J Anesth (2015) 29:647
DOI 10.1007/s00540-014-1967-7
LETTER TO THE EDITOR
Perioperative concerns in a beta‑ketothiolase‑deficient child
Ravinder Pandey · P. M. Singh · Rakesh Garg ·
V. Darlong · J. Punj
Received: 1 October 2014 / Accepted: 19 December 2014 / Published online: 7 January 2015
© Japanese Society of Anesthesiologists 2015
Presentation includes ketoacidosis, vomiting (hemateme-
sis), bloody diarrhea, hyperpnea, hypotonus, cardiomyopa-
thy, prolonged QT, lethargy, and coma [1–4]. Other symp-
toms are neutropenia, thrombocytopenia, and renal failure.
Acute decompensation occurs with infection, starvation,
and surgery [1–3]. A history of vomiting, rapid breathing,
altered sensorium, and decreased urine output indicates
impending ketosis. The anesthetic goal is to avoid factors
and drugs that trigger ketosis: minimize preoperative fast-
ing, administer glucose-based infusions, and avoid propo-
fol and etomidate (inhibit mitochondrial enzymes) [4]. Thi-
opental, ketamine, volatile agents, and opioids appear safe.
Adequate suction of surgical blood needs to be ensured
because aspirated blood on digestion can be ketotic.
References
1. Kayani R, Botros S, Moore P. Beta-ketothiolase deficiency and
pregnancy. Int J Obstet Anesth. 2013;22:260–1.
2. Arica V, Arica SG, Dag H, Onur H, Obut O, Gulbayazar S. Beta-
ketothiolase deficiency brought with lethargy: case report. Hum
Exp Toxicol. 2011;30:1724–7.
3. Sabetta G, Bachmann C, Giardinin O, Castro M, Gambarara M,
Vici CD, Barlett K, Middleton B. Beta-ketothiolase deficiency
with favorable evolution. J Inherit Metab Dis. 1987;10:405–6.
4. Ellinas H, Frost EAM. Mitochondrial disorders: a review
of anesthetic considerations. Middle East J Anesthesiol.
2011;21:235–42.
Keywords Beta-ketothiolase · Deficiency · Anesthesia ·
Child · Surgery
To the Editor:
Beta-ketothiolase deficiency is an autosomal recessive
disorder of the metabolism of amino acids caused by lack
of acetyl CoA-acetyl transferase-1 [1, 2].
A child aged 2.5 years, a case of beta-ketothiolase defi-
ciency, was scheduled for surgery for laryngeal web. The
medical history revealed recurrent ketosis. Presently,
the child was asymptomatic. His laboratory results were
within normal limits. Venous access was secured after
EMLA cream application. Following overnight infusion of
5 % dextrose in saline, his urinary ketones were negative.
Anesthesia was induced with sevoflurane (2–8 %) in oxy-
gen via a tracheotomy tube. Fentanyl (1 µg/kg) was given;
the tracheostomy tube was replaced with an endotracheal
tube and atracurium (0.5 mg/kg) was given. An oral pack
was placed. Anesthesia was maintained with oxygen, air
(50:50), and sevoflurane (MAC 1–1.5). Ringer’s lactate and
5 % dextrose were used as maintenance fluids with blood
sugar monitoring. After surgery, the flexo-metallic tube was
replaced with a tracheostomy tube, and the residual neuro-
muscular block was reversed. The patient was moved to the
recovery room with maintenance fluid. Venous blood gas
showed normal pH and normal anion gap.
Anesthetic management of such cases is challenging
because of multisystem physiological alterations [1–3].
R. Pandey · P. M. Singh · R. Garg (*) · V. Darlong · J. Punj
Department of Anesthesiology and Intensive Care, All India
Institute of Medical Sciences, Ansari Nagar, New Delhi 110029,
India
e-mail: drrgarg@hotmail.com
