Paraparesis and congenital severe hyperkyphosis in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: A rare deformity management during the Sars-Cov-2 pandemic
Introduction: Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) syndrome can be classified into two types: type I (isolated) without extragenital abnormalities; type II (associated) with the presence of extragenital dimorphisms. Skeletal abnormalities are the second most frequent extragenital manifesta...
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| Format: | Article |
| Language: | English |
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Elsevier
2023-01-01
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| Series: | Brain and Spine |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2772529423000413 |
| _version_ | 1827591495523237888 |
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| author | Giovanni Andrea La Maida Andrea Della Valle Enrico Gallazzi Marcello Ferraro Davide Cecconi Alfonso Cicatelli Daniele Priano Luca La Verde Bernardo Misaggi |
| author_facet | Giovanni Andrea La Maida Andrea Della Valle Enrico Gallazzi Marcello Ferraro Davide Cecconi Alfonso Cicatelli Daniele Priano Luca La Verde Bernardo Misaggi |
| author_sort | Giovanni Andrea La Maida |
| collection | DOAJ |
| description | Introduction: Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) syndrome can be classified into two types: type I (isolated) without extragenital abnormalities; type II (associated) with the presence of extragenital dimorphisms. Skeletal abnormalities are the second most frequent extragenital manifestations. Research question: Association between MRKH and congenital scoliosis has been described; on the contrary, hyperkyphosis is very rare and sparsely described in the medical literature. Here we report our experience in the management of thoracolumbar hyperkyphosis in 16-year-old patient diagnosed with MRKH syndrome with an acute neurological impairment due to T11-T12 disc herniation. Material and methods: Clinical and radiological images of the case were retrieved from the medical notes, operative records and imaging system. Results: Posterior surgical correction was proposed to treat the severe spinal deformity; however, surgery was delayed because of SARS-CoV2 pandemic outbreak. During the pandemic, the patient had a major clinical and radiological deterioration with development of paraparesis. Complete clinical resolution of the paraparesis and restoration of balance was achieved with a two stage surgical approach, with a first anterior stage followed by a delayed posterior approach aimed at deformity correction. Discussion: Congenital kyphosis are rare deformities that can progress rapidly leading to severe neurological deficits and worsening of the deformity. When patient has neurological deficit the surgical strategy to address the neurological problem first and plan the more complex and demanding corrective surgery remains a valid strategy that must be consider. Conclusion: This is the first reported case of hyperkyphosis in Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) syndrome surgically treated. |
| first_indexed | 2024-03-09T01:33:30Z |
| format | Article |
| id | doaj.art-b9e3cae7486b4d7b9bf7a4c12ac401bb |
| institution | Directory Open Access Journal |
| issn | 2772-5294 |
| language | English |
| last_indexed | 2024-03-09T01:33:30Z |
| publishDate | 2023-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Brain and Spine |
| spelling | doaj.art-b9e3cae7486b4d7b9bf7a4c12ac401bb2023-12-09T06:09:00ZengElsevierBrain and Spine2772-52942023-01-013101753Paraparesis and congenital severe hyperkyphosis in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: A rare deformity management during the Sars-Cov-2 pandemicGiovanni Andrea La Maida0Andrea Della Valle1Enrico Gallazzi2Marcello Ferraro3Davide Cecconi4Alfonso Cicatelli5Daniele Priano6Luca La Verde7Bernardo Misaggi8U.O. Ortopedia e Traumatologia per le Patologie Della Colonna Vertebrale, ASST G. Pini - CTO, Milano, ItalyU.O. Ortopedia e Traumatologia per le Patologie Della Colonna Vertebrale, ASST G. Pini - CTO, Milano, ItalyU.O. Ortopedia e Traumatologia per le Patologie Della Colonna Vertebrale, ASST G. Pini - CTO, Milano, ItalyU.O. Ortopedia e Traumatologia per le Patologie Della Colonna Vertebrale, ASST G. Pini - CTO, Milano, ItalyU.O. Ortopedia e Traumatologia per le Patologie Della Colonna Vertebrale, ASST G. Pini - CTO, Milano, ItalyU.O. Ortopedia e Traumatologia per le Patologie Della Colonna Vertebrale, ASST G. Pini - CTO, Milano, ItalyU.O. Ortopedia e Traumatologia per le Patologie Della Colonna Vertebrale, ASST G. Pini - CTO, Milano, ItalyDipartimento di Scienze Biomediche per La Salute, Università Degli Studi di Milano, Milano, Italy; Corresponding author.U.O. Ortopedia e Traumatologia per le Patologie Della Colonna Vertebrale, ASST G. Pini - CTO, Milano, ItalyIntroduction: Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) syndrome can be classified into two types: type I (isolated) without extragenital abnormalities; type II (associated) with the presence of extragenital dimorphisms. Skeletal abnormalities are the second most frequent extragenital manifestations. Research question: Association between MRKH and congenital scoliosis has been described; on the contrary, hyperkyphosis is very rare and sparsely described in the medical literature. Here we report our experience in the management of thoracolumbar hyperkyphosis in 16-year-old patient diagnosed with MRKH syndrome with an acute neurological impairment due to T11-T12 disc herniation. Material and methods: Clinical and radiological images of the case were retrieved from the medical notes, operative records and imaging system. Results: Posterior surgical correction was proposed to treat the severe spinal deformity; however, surgery was delayed because of SARS-CoV2 pandemic outbreak. During the pandemic, the patient had a major clinical and radiological deterioration with development of paraparesis. Complete clinical resolution of the paraparesis and restoration of balance was achieved with a two stage surgical approach, with a first anterior stage followed by a delayed posterior approach aimed at deformity correction. Discussion: Congenital kyphosis are rare deformities that can progress rapidly leading to severe neurological deficits and worsening of the deformity. When patient has neurological deficit the surgical strategy to address the neurological problem first and plan the more complex and demanding corrective surgery remains a valid strategy that must be consider. Conclusion: This is the first reported case of hyperkyphosis in Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) syndrome surgically treated.http://www.sciencedirect.com/science/article/pii/S2772529423000413Rokitansky syndromeCongenital hyperkyphosisMRKHSars-Cov2 |
| spellingShingle | Giovanni Andrea La Maida Andrea Della Valle Enrico Gallazzi Marcello Ferraro Davide Cecconi Alfonso Cicatelli Daniele Priano Luca La Verde Bernardo Misaggi Paraparesis and congenital severe hyperkyphosis in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: A rare deformity management during the Sars-Cov-2 pandemic Brain and Spine Rokitansky syndrome Congenital hyperkyphosis MRKH Sars-Cov2 |
| title | Paraparesis and congenital severe hyperkyphosis in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: A rare deformity management during the Sars-Cov-2 pandemic |
| title_full | Paraparesis and congenital severe hyperkyphosis in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: A rare deformity management during the Sars-Cov-2 pandemic |
| title_fullStr | Paraparesis and congenital severe hyperkyphosis in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: A rare deformity management during the Sars-Cov-2 pandemic |
| title_full_unstemmed | Paraparesis and congenital severe hyperkyphosis in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: A rare deformity management during the Sars-Cov-2 pandemic |
| title_short | Paraparesis and congenital severe hyperkyphosis in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: A rare deformity management during the Sars-Cov-2 pandemic |
| title_sort | paraparesis and congenital severe hyperkyphosis in mayer rokitansky kuster hauser mrkh syndrome a rare deformity management during the sars cov 2 pandemic |
| topic | Rokitansky syndrome Congenital hyperkyphosis MRKH Sars-Cov2 |
| url | http://www.sciencedirect.com/science/article/pii/S2772529423000413 |
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