Congenital anomalies of the hand present a wide array of clinical challenges due to
their diverse nature. Each case can significantly differ in terms of anatomical involvement,
severity, functional impairment, and associated conditions. As a result, treatment
plans must be highly individualized, balancing surgical interventions, physical therapy,
and sometimes prosthetics or adaptive devices. Some cases may require multiple surgeries
over time, especially as a child grows, while others might benefit from noninvasive
management strategies. This diversity necessitates a multidisciplinary approach and
careful long-term planning to optimize functional outcomes and patient quality of
life.
Defining outcomes early in the management of congenital anomalies is essential. Early
outcome definition guides the treatment strategy, setting realistic expectations for
both health care providers and patients. It enables tailored interventions suited
to the specific anomaly or deformity, considering functional and aesthetic goals.
This proactive approach can also help in monitoring progress effectively, adjusting
treatments as needed, and ensuring that all interventions align closely with the desired
outcomes, ultimately leading to improved patient satisfaction and functional results.
Effective communication with patients or caregivers is necessary in these conditions,
which often require long-term, complex treatment plans, including surgeries and rehabilitation.
Clear communication ensures that patients and their caregivers fully understand the
diagnosis, treatment options, and potential outcomes. It helps in setting realistic
expectations, which is vital for emotional and psychological preparedness. Additionally,
effective dialogue fosters a stronger patient–clinician relationship, enhances adherence
to treatment plans, and ultimately leads to better overall patient satisfaction and
outcomes.[1]
Congenital hand anomalies have been classified in many ways, for example, the Swanson
classification, the Oberg–Manske–Tonkin (OMT) classification, while hand functional
outcomes are scored using the Disabilities of the Arm, Shoulder, and Hand (DASH) score,
QuickDASH score, etc.[2]
[3]
[4]
[5] However, conveying the severity of the anomalies or deformity to a patient is always
a challenge for the caregiver.
Recognizing the challenges in consistently evaluating and communicating the complexities
of hand anomalies and deformities, we are proposing a novel grading system ([Table 1]) to bridge this gap.
Table 1
The Hand Anomalies and Deformity (HAnD) grading system
Grade
|
Severity
|
Characteristics
|
Treatment outcome
|
Examples
|
I
|
Mild anomalies
|
Minimal functional impairment; anomalies primarily cosmetic
|
Excellent functional and cosmetic outcomes expected with or without surgical intervention
|
Slight syndactyly without bone fusion, minor polydactyly, small accessory digits
|
II
|
Moderate anomalies
|
Some functional impairment; may have significant cosmetic concerns
|
Good functional outcomes expected with surgical intervention; cosmetic results may
vary
|
Complete simple syndactyly, moderate polydactyly, mild camptodactyly
|
III
|
A
|
Severe anomalies with compromised function
|
Functional improvement possible with surgical intervention, but perfect functional
or cosmetic outcomes may not be achievable
|
Significant functional impairment and cosmetic issues; may affect multiple digits
or the whole hand
|
Complex syndactyly (with bone fusion), severe polydactyly, severe camptodactyly, congenital
constriction band syndrome with limb involvement
|
B
|
Severe anomalies with associated syndromes
|
Functional outcomes vary widely; multiple surgeries often required; multidisciplinary
approach critical for management
|
Major hand deformities often associated with systemic syndromes; significant functional
and cosmetic impairment
|
Radial club hand, severe cases of arthrogryposis affecting the hand, Apert's syndrome
with complex syndactyly
|
IV
|
Profound anomalies with poor functional outcome
|
Extreme deformities with very limited potential for normal function
|
Focus on maximizing residual function and prosthetic adaptation; limited surgical
benefit for functionality
|
Major aplasia/hypoplasia of the hand, severe phocomelia
|
Note: This grading system aims to assist health care providers in categorizing congenital
hand anomalies, aiding in communication, planning treatment strategies, and setting
realistic expectations for outcomes, and would be beneficial in both clinical and educational settings.
Our aim was to empower health care providers (particularly hand surgeons) with a simple
tool that goes beyond anatomical descriptions or complex scoring to offer a framework
for informing clinical decision-making, streamlining communication, and setting realistic
expectations for patients.
The Hand Anomalies and Deformity (HAnD) grading system is based on two key factors:
characteristics and treatment outcome. It grades anomalies on a scale from I (mild)
to IV (profound), with grade III (severe) further divided into A and B subcategories
for nuances in complexity and associated syndromes ([Fig. 1]).
Fig. 1 Selective examples of hand anomalies representing different grades. Grade I: (A) ulnar polydactyly. Grade II: (A) trigger thumb (older child); (B) macrodactyly; (C) simple complete syndactyly. Grade IIIA: (A) brachydactyly; (B) severe constriction ring syndrome; (C) severe clasp thumb deformity. Grade IIIB: (A) bilateral ulnar deficiency; (B) Apert's hand deformity; (C) synpolydactyly in Saethre–Chotzen syndrome. Grade IV: (A) unilateral adactyly of digits 2 to 5 with thumb hypoplasia (transverse limb deficiency).
These deformities will often need additional physical therapy to achieve optimum function.
The system outlines the expected functional and cosmetic outcomes for each grade.
This system thus helps health care providers accurately classify and communicate the
nature of the hand condition to the patient or the caregiver.
Some additional features of the HAnD grading system include the following:
-
Emphasis on function: The system prioritizes the impact of the deformity on hand function, aiding in treatment
planning, and setting realistic expectations for patients.
-
Consideration of associated syndromes: Recognizing the presence of associated syndromes like Apert's syndrome allows for
a more holistic approach to management.
-
Focus on treatment feasibility: The system acknowledges the limitations of surgical intervention for severe anomalies,
guiding treatment decisions toward maximizing the remaining function.