|LETTER TO EDITOR
|Year : 2022 | Volume
| Issue : 1 | Page : 61-62
Anaesthesia in a patient with seckel syndrome - Plan for contingencies
Sujata Shivlal Rawlani, M Dave Nandini
NH-SRCC Children's Hospital, (Managed by Narayana Health), Mumbai, Maharashtra, India
|Date of Submission||12-Jan-2022|
|Date of Acceptance||09-Feb-2022|
|Date of Web Publication||11-Mar-2022|
Dr. Sujata Shivlal Rawlani
Flat No. 8, Hind Mansion, Sitladevi Temple Road, Opposite Kolhapur Dairy, Mahim, Mumbai - 400 016, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Rawlani SS, Nandini M D. Anaesthesia in a patient with seckel syndrome - Plan for contingencies. Airway 2022;5:61-2
Primordial dwarfism is a rare autosomal recessive disorder first described in 1960 with an incidence of 1 in 10,000 live births. The condition was then called Seckel syndrome. However, due to its heterogeneous expression and distinct radiological findings, a subclassification of microcephalic osteodysplastic primordial dwarfism (MOPD) was introduced. These children are commonly known as 'bird-headed' dwarfs due to their peculiar facial features. Only a few cases of anaesthesia for MOPD patients have been described in literature. We report the anaesthetic management of a 6.5-year-old girl diagnosed with MOPD type 2 for multiple extractions and restoration of teeth.
The child was born to non-consanguineous parents at 35 weeks of gestation with a birth weight of 1 kg. She had a history of multiple episodes of supraventricular tachycardia (SVT) during infancy, treated with tablet amiodarone and tablet metoprolol. These medications had been discontinued on medical advice for the past 5 years. Although her developmental milestones were slightly delayed, her intelligence was near normal. On examination, the child weighed 5.5 kg (<1st percentile for age) and had a height of 108 cm (2nd percentile for age). Her facial features included microcephaly, retrognathia, bird-like face and small low-set ears. Mouth opening was adequate with a modified Mallampati class II. Routine blood investigations and echocardiography were normal. Chest radiograph showed mild scoliosis in the lower dorsal spine and essentially normal lung fields. Magnetic resonance imaging of the brain was suggestive of caudal herniation of cerebellar tonsil in the cervical cord. Although the parents of the child had allowed clinical details to be revealed, they did not give permission to publish any clinical photographs or radiographs.
Preoperative heart rate was 120/min and blood pressure was 118/50 mm Hg. Oral midazolam premedication (0.5 mg/kg) was administered in the preoperative holding area. A difficult airway cart was kept ready. A multiparameter monitor was employed to monitor heart rate and rhythm, noninvasive blood pressure, oxygen saturation, capnograph and inhalational agent concentration. In view of an anticipated difficult airway, inhalational induction was performed with sevoflurane in oxygen and spontaneous respiration was preserved. Intravenous (IV) access was established with a 24 SWG cannula after achieving adequate depth of anaesthesia. Following the stimulus of IV cannulation, there was an episode of abrupt onset supraventricular tachycardia (SVT) (narrow complex tachycardia at 220/min with absent P waves). The arrhythmia reverted to sinus rhythm in about 30 s without any intervention.
Injection fentanyl 15 ug was administered and after ascertaining chest rise with bag-mask ventilation, injection atracurium 3 mg was given to facilitate nasal intubation. A 4.0 mm ID uncuffed nasal tube was inserted and a throat pack was placed. Anaesthesia was maintained with sevoflurane in an air:oxygen mixture (3:2) and dexmedetomidine infusion at 0.5 ug/kg/h. Injection paracetamol 60 mg and injection diclofenac 6 mg were administered for perioperative analgesia. Injection Xylocard® 10 mg was administered before antagonising residual neuromuscular blockade at the end of surgery. Following extubation, the child had another episode of SVT which was treated with injection metoprolol 0.1 mg/kg. The postoperative course was uneventful with no further episodes of arrhythmia.
Anaesthetic management in Seckel syndrome can be challenging. A difficult airway and difficult venous access remain prime concerns. A dysmorphic face with a receding chin and a relatively big nose could also render mask ventilation difficult. Age and weight may fail to predict endotracheal tube size. We were prepared to handle the anticipated difficulty of improper mask fit with an appropriate-sized laryngeal mask airway. Smaller size tracheal tubes were also kept ready.
Seckel syndrome is known to be associated with cardiovascular abnormalities such as patent ductus arteriosus and complex cardiac defects., While the majority of reports highlight structural cardiac abnormalities in Seckel syndrome, there are no reports of rhythm abnormalities or aberrant conduction pathways which make these patients prone to arrhythmias.
In our patient, airway management and venous access proved easy, but we encountered two episodes of SVT. Considering the past history of arrhythmia, we were prepared with medications as well as a cardioverter-defibrillator. Patients with Seckel syndrome should be assessed not only for difficult airway and structural cardiac abnormalities but should also be carefully investigated for rhythm disturbances which might turn into an intraoperative nightmare for the unsuspecting anaesthesiologist.
Declaration of patient consent
The authors certify that the parents have given consent for clinical information to be reported in the journal without revealing the patient's identity. The parents did not give consent for clinical photographs and radiographs to be published even if the identity was adequately concealed. The authors have therefore ensured that the name and initials of the child have been carefully concealed. The authors regret their inability to submit clinical photographs.
I would like to extend my gratitude to Dr. Nandini Dave for her support to succesfully report this study.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Parent P, Moulin S, Munck MR, de Parscau L, Alix D. Bird headed dwarfism in Seckel syndrome. Nosologic difficulties. Arch Pediatr 1996;3:55-62.
Mona A, Ingo K, Friederike FS, Katja K, Dominique S, Konrad R, et al
. Skeletal and neurological features of Seckel syndrome and microcephalic osteodysplastic primodrial dwarfism – A review of the literature. Am J Pediatr 2020;6:373-80.
Srinivasan D, Jegath Reddy RC, Louis CJ, Dakshinamurthi S. Dental management of Seckel syndrome in a child. J Indian Acad Dent Spec Res 2018;5:29-31. [Full text]
Grewal A, Sood D, Bhatia N, Garg R, Shah S, Kaur H. Palatoplasty in a patient with Seckel syndrome: An anesthetic challenge. Braz J Anesthesiol 2014;64:216-8.
Rajamani A, Kamat V, Murthy J, Hussain SA. Anesthesia for cleft lip surgery in a child with Seckel syndrome – A case report. Paediatr Anaesth 2005;15:338-41.
Arora S, Ghai B, Rattan V. Anesthetic management of a child with Seckel syndrome for multiple extractions and restoration of teeth. J Anaesthesiol Clin Pharmacol 2012;28:398-9.
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