Viena soļa apakšstilba rekonstrukcija sešus mēnešus vecam zēnam ar vaskularizētu funkcionālu Vastus Lateralis muskuļu lēveri, ārstējot embrionālu rabdomiosarkomu
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Date
2021
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Rīgas Stradiņa universitāte
Rīga Stradiņš University
Rīga Stradiņš University
Abstract
Ievads. Rabdomiosarkoma (RMS) ir bieža mīksto audu sarkoma bērniem, kaut gan ta ir ļoti reta neonatālajā periodā. (0.4–2% gadījumu). Ķirurģija paliek standarta ārstēšana, bet pilnīga izārstēšana nevar tikt sasniegta bez adjuvantas ārstēšanas. Pediatriskai populācijai, kas jaunāki par diviem gadiem, rekonstrukcijas iespējas ir ierobežotas, jo jaundzimušajiem ir relatīvi mazi, piemēram, asinsvadi.
Klīniskā gadījuma izklāsts. Šajā gadījuma tiek apskatīts zēns, kuram tika atklāta RMS divu nedēļu vecumā, bet oficiāli tā tika diagnosticēta trīs mēnešu vecumā. Magnētiskā rezonanse un scintigrāfija pierādīja mīksto audu audzēju m.soleus, tikmēr biopsija apstiprināja embrionālu RMS ar augstu mitotisko aktivitāti (Ki67 (monoklonālas antivielas) ~ 80%). Tika nozīmēts CWS (Cooperative Weichteilsarkom Studiengruppe)-2012 kopā ar I2VA (ifosfamīds, vincristīns, aktinomicīns) ķīmijterapijas režīmu atbilstoši protokolam. Ķirurģiskā ārstēšana tika veikta 6 mēnešu un 18 dienu vecumā. Operācija sastāvēja no radikālas audzēja rezekcijas un totālas triceps surae ar daļēju fibula rezekciju. Tika veikta tūlītēja triceps muskuļa rekonstrukcija, izmantojot vaskularizētu funkcionālu musculocutaneous vastus lateralis lēveri.
Funkcionālais iznākums tika vērtēts, izmantojot Lower Extremity Functional Scale (LEFS) un the Foot and Ankle Outcome Score (FAOS), iegūstot sekojoši 92,5% un 99%.
Secinājumi. Embrionāla rabdomiosarkoma ir rets iedzimts audzējs ar labu prognotisko iznākumu. Veiksmīga ārstēšana tiek nodrošināta ar maksimālu funkcionālu restaurāciju, izmantojot mikrovaskulāras audu transplantēšanas tehnikas jaundzimušo periodā. Pediatriskiem pacientiem, kuri jaunāki par diviem gadiem, tiek rekomendēta audzēja radikāla rezekcija un funkcionāla rekonstrukcija, izmantojot mikrovaskulāru leveri.
Kopsavilkums. Šis klīniskais gadījums demonstrē sarežģītu ārstēšanu embrionālai RMS, viena soļa ķirurģisku rekonstrukciju ar funkcionālu vastus lateralis myocutaneous mikrovaskulāru lēveri, jaunākajam pacientam Latvijā. Maza izmēra asinsvadi ir sarežģīti, lai veidotu mikrovaskulāras anastomozes jaundzimušo vecumā, bet attiecīgas ķirurģiskās prasmes un lielisks palielinājums var rezultēties ar veiksmīgu iznākumu.
Introduction. Rhabdomyosarcoma (RMS) is a common soft tissue sarcoma in childhood, however, it is very rare in the neonatal period (0.4–2% of cases). Surgery remains the standard of treatment, yet the curative aim cannot be achieved without adjuvant treatment. Reconstructive possibilities are limited in the pediatric population younger than two years of age due to relatively small structures such as blood vessels. Case report description. This case depicts a boy, who presented with RMS at two weeks of age but officially diagnosed at the age of three months. MRI and scintigraphy determined a soft tissue tumor in the soleus muscle, while biopsy confirmed embryonal RMS with high mitotic activity (Ki67 (monoclonal antibodies) ~80%). CWS (Cooperative Weichteilsarkom Studiengruppe)-2012 with I2VA (ifosfamide, vincristine, actinomycin) chemotherapy regimen was administered per protocol. Surgical treatment was performed at age of 6 months and 18 days. The operation consisted of radical tumor resection and total triceps surae with partial fibula resection. Immediate reconstruction of triceps muscle was accomplished using a vascularized functional musculocutaneous vastus lateralis flap. Functional outcome was measured using the Lower Extremity Functional Scale (LEFS) and the Foot and Ankle Outcome Score (FAOS) with the results of 92.5% and 99% respectively. Conclusion. Embryonal rhabdomyosarcoma is a rare congenital tumor with a good prognostic outcome. Successful treatment is provided with maximal functional restoration using microvascular tissue transplantation techniques in the infant period. Radical resection of the tumor is recommended and functional reconstruction using microvascular flaps for pediatric patients younger than one year of age. Summary. This case demonstrates complex treatment of the embryonal RMS with one-step surgical reconstruction with functional vastus lateralis myocutaneous microvascular flap for the youngest patient in Latvia. Small vessel size is challenging for microvascular anastomosis at the infant age but refined surgical skills and excellent magnification can lead to successful surgical outcomes.
Introduction. Rhabdomyosarcoma (RMS) is a common soft tissue sarcoma in childhood, however, it is very rare in the neonatal period (0.4–2% of cases). Surgery remains the standard of treatment, yet the curative aim cannot be achieved without adjuvant treatment. Reconstructive possibilities are limited in the pediatric population younger than two years of age due to relatively small structures such as blood vessels. Case report description. This case depicts a boy, who presented with RMS at two weeks of age but officially diagnosed at the age of three months. MRI and scintigraphy determined a soft tissue tumor in the soleus muscle, while biopsy confirmed embryonal RMS with high mitotic activity (Ki67 (monoclonal antibodies) ~80%). CWS (Cooperative Weichteilsarkom Studiengruppe)-2012 with I2VA (ifosfamide, vincristine, actinomycin) chemotherapy regimen was administered per protocol. Surgical treatment was performed at age of 6 months and 18 days. The operation consisted of radical tumor resection and total triceps surae with partial fibula resection. Immediate reconstruction of triceps muscle was accomplished using a vascularized functional musculocutaneous vastus lateralis flap. Functional outcome was measured using the Lower Extremity Functional Scale (LEFS) and the Foot and Ankle Outcome Score (FAOS) with the results of 92.5% and 99% respectively. Conclusion. Embryonal rhabdomyosarcoma is a rare congenital tumor with a good prognostic outcome. Successful treatment is provided with maximal functional restoration using microvascular tissue transplantation techniques in the infant period. Radical resection of the tumor is recommended and functional reconstruction using microvascular flaps for pediatric patients younger than one year of age. Summary. This case demonstrates complex treatment of the embryonal RMS with one-step surgical reconstruction with functional vastus lateralis myocutaneous microvascular flap for the youngest patient in Latvia. Small vessel size is challenging for microvascular anastomosis at the infant age but refined surgical skills and excellent magnification can lead to successful surgical outcomes.
Description
Pediatrija
Pediatrics
Veselības aprūpe
Health Care
Pediatrics
Veselības aprūpe
Health Care
Keywords
Embrionāla rabdomiosarkoma; funkcionāla vasatus transplantācija; jaundzimušo mikroķirurģija., Embryonal rhabdomyosarcoma; functional vastus transplantation; infant microsurgery.