Intercarpal Degenerative Arthritis in Adulthood as a Late Consequence of Unilateral Congenital Aplasia of the Scaphoid: A Case Report (2022)

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The Journal of Hand Surgery

Volume 33, Issue 2,

February 2008

, Pages 213-216

We report a case of unilateral congenital aplasia of the carpal scaphoid bone discovered in adulthood, which probably caused a severe painful intercarpal degenerative arthritis. This case was not associated with hypoplasia or aplasia of thenar and forearm muscles, abnormality of the skeleton of the thumb ray, absence of the sesamoid bones, hypoplasia of the forearm bones, as in cases reported in the literature; the only additional anatomic abnormality was a mild hypoplasia of the radial styloid. A surgical treatment (4-bone arthrodesis) has been performed to treat the advanced carpal collapse and the arthritis.

Section snippets

Case Report

A 45-year-old, right-handed manual worker presented with 6 months of pain, swelling, and stiffness of both wrists, much more severe on the left, without any history of injury or other joint complaints. There was no forearm or thenar hypoplasia, and thumb size and creases were normal bilaterally. There was mild swelling and tenderness over the dorsum of the left wrist. Wrist range of motion was normal bilaterally except for diminished radial deviation on the left. There were no neurologic

Discussion

Scaphoid anomalies (hypoplasia or aplasia) are frequently observed in association with radial deficiency or thumb absence or hypoplasia6, 7, 8, 9, 10, 11 and in congenital syndromes such as VACTERL association (vertebral defects, anal atresia, cardiac anomalies, tracheo-esophageal fistula, renal and radial anomalies, and lung anomalies),12, 13 Holt-Oram syndrome (cardiac defects and upper limb abnormalities),14 or TAR syndrome (thrombocytopenia-absent radius).15 Isolated congenital anomalies of

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Cited by (3)

  • Chronic wrist pain. Hypoplasia of the carpal scaphoid bone

    2021, Revista Espanola de Cirugia Ortopedica y Traumatologia

    La ausencia o hipoplasia del escafoides carpiano es una entidad rara e infrecuente, y aún más si se da de forma aislada, pues suele aparecer asociada a otros síndromes congénitos. Suele ser debida a una alteración en el desarrollo fetal antes de la semana 11 del embarazo. Presentamos el caso de una mujer de 53 años, con dolor crónico de muñeca, intervenida de síndrome del túnel del carpo izquierdo, con persistencia de dolor tras la intervención. Las radiografías y la TC permitieron realizar el diagnóstico definitivo, evidenciando la existencia de una hipoplasia escafoidea con inestabilidad carpiana. Tras el procedimiento quirúrgico y la realización de tratamiento rehabilitador posterior la paciente quedó asintomática.

    The absence or hypoplasia of the carpal scaphoid bone is rare and unfrequent, especially if isolated, as it is usually associated with other congenital disorders. Such anomaly is usually the result of fetal development alterations before week 11 of pregnancy. We introduce the case of a 53-year-old woman with chronic wrist pain and persistent pain after surgical intervention of the carpal tunnel syndrome in the left hand. X-rays and CT confirmed the patient's hypoplasia of the scaphoid bone with carpal instability diagnosis. The patient became asymptomatic after surgical intervention and post-surgical rehabilitation.

  • Unilateral congenital absence of the carpal scaphoid associated with dysplasia of the capitate. Presentation of a case

    2012, Revista Espanola de Cirugia Ortopedica y Traumatologia

    Presentamos un caso de aplasia o ausencia congénita unilateral del escafoides carpiano asociado a displasia del hueso grande. La ausencia congénita del escafoides carpiano es una entidad rara e infrecuente, pero bien documentada. Es el séptimo caso presentado en la literatura. A diferencia de la mayoría de los casos publicados de aplasia/hipoplasia de escafoides carpiano, en nuestro caso no se ha detectado asociación de otras anomalías congénitas tipo hipoplasia o aplasia de musculatura tenar y antebrazo, malformaciones del esqueleto del pulgar, ausencia de huesos sesamoideos o alteraciones del desarrollo de los huesos del antebrazo. El estudio radiológico revela la ausencia congénita del escafoides asociado a una displasia del hueso grande e hipoplasia de la estiloides radial. Se completa el estudio con resonancia magnética nuclear.

    We report on a case of aplasia or unilateral congenital absence of the carpal scaphoid associated with dysplasia of the capitate. Congenital absence of the carpal scaphoid is a rare but well-documented condition. As far as we know, the present case is the seventh one reported in the medical literature. Imaging studies (X-ray and MRI) confirmed the absence of the carpal scaphoid associated with a dysplasia of the capitate and malformation of the radial styloid process. Congenital absence of the scaphoid when other congenital abnormalities (such as hypoplasia or aplasia of either forearm bones or thenar eminence, malformations of the skeletal elements of the thumb, absence of sesamoid bones or abnormal development of the forearm bones) do not exist is probably the main feature of the present case report.

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Copyright © 2008 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

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