Anesthesiology
doi: 10.25005/2074-0581-2026-28-1-252-258
ANESTHESIA DURING LAPAROSCOPIC SURGERY IN A CHILD WITH GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY AND SITUS INVERSUS TOTALIS: A CLINICAL CASE
1Avicenna Tajik State Medical University, Dushanbe, Republic of Tajikistan
2National Medical Center of Republic of Tajikistan “Shifobakhsh”, Dushanbe, Republic of Tajikistan
This case describes the anesthetic management of a child with a complete mirror-image disposition of the internal organs (thoracic and abdominal cavities), including dextrocardia and a left-sided diaphragmatic hernia. A 4-year-old girl was admitted for a splenectomy. CT imaging revealed the spleen on the right, the liver on the left, the stomach located in the left thoracic cavity, and the heart rotated to the right.
Anesthetic challenges arose from the combination of situs inversus totalis and glucose-6-phosphate dehydrogenase (G6PD) deficiency. The plan to perform multiple surgical procedures simultaneously increased the risk of adverse anesthetic events, elevating the patient's status from ASA II to ASA III.
Upon induction and initiation of mechanical ventilation, the following modes were used: Volume Control (VC) and Assist/Control (A/C). Following the creation of pneumoperitoneum and the reduction of the abdominal organs from the chest, the ventilator was switched to VC with a Synchronized Intermittent Mandatory Ventilation (SIMV) algorithm. Anesthesia was maintained via bolus administration of anesthetics, narcotic analgesics, and muscle relaxants. Hemodynamic parameters remained stable throughout. To maintain homeostasis, 400 ml of crystalloid and colloid solutions were administered; total urine output was 230 ml. The procedure lasted 3 hours and 20 minutes with no anesthesia-related complications.
Keywords: Situs inversus totalis, children, anesthesia, esophagogastrofundoplication, splenectomy, clinical case.
References
-
1. Ibrahim R, Daoud H. Kartagener's syndrome: A case report. Can J Respir Ther. 2021;57:44-8. https://doi.org/10.29390/cjrt-2020-064
2. Wang L, Zhao S, Liang H, Zhang L, Li S, Li D, et al. Novel compound heterozygous DNAH5 mutations identified in a pediatric patient with Kartagener syndrome: A report clinical case report and literature review. J BMC Pulm Med. 2021;21(1):263. https://doi.org/10.1186/s12890-021-01586-4
3. Çiki K, Türer OB, Khyzal M, Tugcu GD, Emiralioglu N, Yalcin E, et al. A rare cause of acute abdominal pain in a patient with primary ciliary dyskinesia and complete inversion of internal organs. Turk J Pediatr. 2020;62(1):156-9. https://doi.org/10.24953/turkjped.2020.01.024
4. Lin H, Cao Z, Zhao S, Ye QJ. Left midlobe resection for bronchiectasis in a patient with Kartagener syndrome: A clinical case. Cardiothorac Surg J. 2016;11:37. https://doi.org/10.1186/s13019-016-0426
5. Butt SRR, Shakoor H, Khan TJ, Almaalouli B, Ekhator C, Ansari S, et al. A Rare case of Kartagener syndrome presenting with sinusitis, situs inversus, and bronchiectasis: Emphasizing early diagnosis and management strategies. Cureus. 2023;15(7):e41890. https://doi.org/10.7759/cureus.41890
6. Kendigelen P, Tyutuncu ACh, Erbabakan SE, Kaya G, Altindash F, Terk J. Anesthesiological management of a patient with synchronous Kartagener syndrome and biliary atresia: A clinical case. Anaesthesiol Reanim J. 2015;43(3):205-8. https://doi.org/10.5152/TJAR.2015.94546
7. Cheng L, Dong Y, Liu S. Anesthetic management of patients with Kartagener's syndrome: A systematic review of 99 cases. J Cardiothorac Vasc Anesth. 2023;37(6):1021-5. https://doi.org/10.1053/j.jvca.2023.01.033
8. Sharipov AM, Mukhiddinov ND, Rakhmatova RA, Mazabshoev SA. Transpozitsiya vnutrennikh organov v sochetanii s gryzhey pishchevodnogo otverstiya diafragmy na fone nasledstvennoy gemoliticheskoy anemii u rebenka 4 let: klinicheskoe nablyudenie [Transposition of internal organs in combination with a hernia of the esophageal opening of the diaphragm against the background of hereditary gemolytic anemia in a 4-year-old child: A clinical observation]. Rossiyskiy vestnik detskoy khirurgii, anesteziologii i reanimatologii. 2022;12(3):383-90. https://doi.org/10.17816/psaic1037
9. Botwe BO, Sarkodie BD, Mensah YB, Antwi WK. Congenital transpositions of stomach and spleens with partial pyloric stenosis. BJR Case Rep. 2016;3(2):20150196. https://doi.org/10.1259/bjrcr.20150196
10. Akrimi S, Simiyu V. Anesthetic treatment of children with sickle cell anemia. BJA Educ. 2018;18(11):331-6. https://doi.org/10.1016/j.bjae.2018.08.003
11. Chaithanya K, Reddy PN, Gandra S, Srikanth A. Anaesthetic management of a case of hereditary spherocytosis for splenectomy and cholecystectomy. Indian J Anaesth. 2014;58(3):343-5. https://doi.org/10.4103/0019-5049.135082
12. Sahajananda H, Sanjay OP, Johnny T, Bobby D. General anesthesia for lobectomy in an 8-year-old child with Kartagener syndrome: A clinical case. Pediatr Anesth J. 2003;13(8):714-7. https://doi.org/10.1046/j.1460-9592.2003.01039
13. Uludag Yanaral T, Karaaslan P, Uzunoglu E, Atalay YO, Tobias JD. Liver transplantation in a child with Kartagener syndrome: A case report. Int Med Case Rep J. 2021;14:295-9. https://doi.org/10.2147/IMCRJ.S311843
14. Mathur P, Khare A, Jain N, Verma P, Mathur V. Anesthetic considerations in a child with unrepaired D-transposition of great arteries undergoing noncardiac surgery. Anesth Essays Res. 2015;9(3):440-2. https://doi.org/10.4103/0259-1162.158511
Authors' information:
Mazabshoev Salomatsho Aslishoevich,
Candidate of Medical Sciences, Associate Professor of the Department of Pediatric Surgery, Avicenna Tajik State Medical University
ORCID ID: 0000-0002-9346-0764
SPIN: 6678-2908
E-mail: samikdoctor@mail.ru
Sharipov Aslamkhon Makhmudovich,
Doctor of Medical Sciences, Head of the Department of Pediatric Surgery, Avicenna Tajik State Medical University
ORCID ID: 0000-0002-3534-9208
SPIN: 3072-5037
E-mail: aslam72@list.ru
Shernazarov Ibragim Bekmirzaevich,
Candidate of Medical Sciences, Associate Professor of the Department of Pediatric Surgery, Avicenna Tajik State Medical University
ORCID ID: 0009-0006-1573-6979
E-mail: dr_shernazarov@mail.ru
Safedov Fakhriddin Kholniyozovich,
Doctor of Medical Sciences, Associate Professor of the Department of Pediatric Surgery, Avicenna Tajik State Medical University
ORCID ID: 0000-0001-6300-6061
SPIN: 1364-3679
E-mail: fsafedov @mail.ru
Rakhmonov Bobokhon Yatimovich,
Head of the Operating Unit, National Medical Center of Republic of Tajikistan “Shifobakhsh”
ORCID ID: 0009-0009-2732-6151
E-mail: bobokhanrahman@gmail.com
Information about support in the form of grants, equipment, medications
The authors did not receive financial support from manufacturers of medicines and medical equipment.
Conflicts of interest: No conflict
Address for correspondence:
Mazabshoev Salomatsho Aslishoevich
Candidate of Medical Sciences, Associate Professor of the Department of Pedi- atric Surgery, Avicenna Tajik State Medical University
734026, Republic of Tajikistan, Dushanbe, Sino str., 29-31
Tel.: +992 (935) 125071
E-mail: samikdoctor@mail.ru
This work is licensed under a Creative Commons Attribution 4.0 International License.
