Soldado Francisco, Nguyen Trong-Quynh, Rojas-Neira Juliana, Rodriguez-Ostuni Daniel, Cherqaoui Abdelmounim, Díaz-Gallardo Paula
Pediatric Hand Surgery and Microsurgery, Barcelona Children's Hospital, HM Nens, HM Hospitales.
Pediatric Orthopedic Unit, Saint Paul Hospital, Hanoi, Vietnam.
J Pediatr Orthop. 2024 Oct 1;44(9):567-571. doi: 10.1097/BPO.0000000000002764. Epub 2024 Jul 11.
The congenital insufficiency of the extensor tendon central slip of the fingers is a relatively rare condition, with only a few reported cases in pediatric patients, as described in 2 clinical series. In this study, we aimed to present the natural history of a significant number of untreated patients with this deformity.
This study has received institutional review board approval, and parents provided informed consent following the Declaration of Helsinki guidelines for biomedical research involving humans. A retrospective analysis of children with this deformity, ranging from June 2008 to July 2021, was collected by 1 surgeon. The inclusion criteria included children with a supple PIP flexion deformity, characterized by MP hyperextension and PIP extension lag, which had been present since birth. Complete passive PIP extension and the absence of volar skin webbing differentiated this condition from camptodactyly.
The mean age of 24 children with 57 involved digits at diagnosis was 7 months (range, 1 to 17) and the mean follow-up was 6 years to 9 months (2 yr to 1 mo to 13 yr). Six patients had an incorrect previous diagnosis of camptodactyly.Active PIP extension recovered progressively. At the final follow-up, complete PIP extension occurred in all except 4 cases in which a residual 10° extension lag. The mean time for a complete active PIP extension was 2 years to 7 months (20 mo to 3 yr to 9 mo). Nineteen cases (79%) showed a mild FDS contracture of the involved digits at the final follow-up.The deformity was bilateral in 15 children (62.5%) and involved only 1 finger (unilaterally or bilaterally) in 15 cases (62.5%), and 2 fingers in 6 (25%). Little and ring fingers were most commonly involved. In 7 cases, there was a family history of finger deformity.
Congenital insufficiency of the extensor tendon central slip typically resolves spontaneously within the first 4 years of life. Literature suggests that splinting can expedite the correction of the deformity and thus, if possible, it can be used. In most cases, a residual, clinically insignificant FDS contracture may be present. This condition is often misdiagnosed as camptodactyly.
IV.
手指伸肌腱中央束先天性发育不全是一种相对罕见的病症，如两个临床系列报道所述，儿科患者中仅有少数病例报告。在本研究中，我们旨在呈现大量未经治疗的该畸形患者的自然病程。
本研究已获得机构审查委员会批准，家长根据涉及人类的生物医学研究的《赫尔辛基宣言》准则提供了知情同意书。由1名外科医生对2008年6月至2021年7月期间患有这种畸形的儿童进行回顾性分析。纳入标准包括自出生以来就存在的以掌指关节过伸和近端指间关节伸展滞后为特征的柔软的近端指间关节屈曲畸形的儿童。近端指间关节完全被动伸展以及掌侧皮肤无蹼可将这种情况与先天性屈曲挛缩畸形相区分。
24例患儿共57个手指在诊断时的平均年龄为7个月（范围1至17个月），平均随访时间为6年9个月（2年1个月至13年）。6例患者先前被误诊为先天性屈曲挛缩畸形。近端指间关节的主动伸展逐渐恢复。在最后一次随访时，除4例残留10°伸展滞后外，所有患者近端指间关节均完全伸展。近端指间关节完全主动伸展的平均时间为2年7个月（20个月至3年9个月）。19例（79%）在最后一次随访时受累手指出现轻度指浅屈肌挛缩。15例患儿（62.5%）畸形为双侧，15例（62.5%）仅累及1根手指（单侧或双侧），6例（25%）累及2根手指。小指和环指最常受累。7例有手指畸形家族史。
伸肌腱中央束先天性发育不全通常在生命的前4年内自发缓解。文献表明，夹板固定可加快畸形矫正，因此，如果可能，可以使用。在大多数情况下，可能存在残留的、临床上无明显意义的指浅屈肌挛缩。这种情况常被误诊为先天性屈曲挛缩畸形。
四级
