Maine (Hemimelia)

Hemimelia is a congenital absence or thickening and shortening (aplasia) of the peripheral parts of the limbs. Sometimes only one of the two paired bones of the limbs is affected (the ulna and radius of the arm or the tibia and fibula of the leg).

Hemimelia is a congenital developmental anomaly and is most often diagnosed in the prenatal period during ultrasound examination. The reasons for its development are not fully understood; presumably, it may be due to gene mutations, nutritional deficiencies, and exposure to harmful factors on the fetus.

The severity of hemimelia can vary - from slight shortening of the bones to the complete absence of a limb segment. The lower extremities are most often affected. Treatment is mainly surgical and involves lengthening and realigning the bones. The prognosis depends on the extent of the lesion. With severe forms of hemimelia, disability may develop.

Hemimelaya (Latin for “half-limb”) is a congenital developmental disorder manifested in underdevelopment of a limb on one side. There are the following types of hemimelia:

- Atrophic hemimelia – manifests itself in atrophy of limb tissue and malnutrition;

- Contraction hemimelia (from the Latin contractio - “compression”) – develops as a result of joint contractures. In this case, tension in the tendons or muscles of the limb increases, which leads to shortening of the limb;

Hemimelia: Congenital Developmental Disorder of the Limbs

Hemimelia, also known as hemimelic aplasia, is a rare congenital disorder characterized by absent or thickened and shortened peripheral limbs. This condition can affect one of two paired limb bones, such as the ulna and radius in the arm or the tibia and fibula in the leg. Hemimelia belongs to a family of disorders known as congenital limb abnormalities.

Definition and causes of hemimelia:

Hemimelia occurs as a result of incomplete or abnormal formation of a limb during embryonic development. In many cases, the exact causes of this disorder remain unknown, although some research points to genetic and environmental factors that may influence its occurrence.

Symptoms and classification of hemimelia:

Symptoms of hemimelia vary depending on the extent and location of the lesion. However, the most common signs are the absence or underdevelopment of bones, muscles, joints and other tissues in the affected areas of the limbs. In children suffering from hemimelia, one limb may be significantly shorter and less functional than its paired limb.

Depending on the nature of the lesion, hemimelia can be classified as follows:

1. Hemimelia degree I: In this case, only the distal part of the limb (located closer to the end of the limb) is missing or underdeveloped.

2. Hemimelia II degree: Here, both the proximal (located closer to the body) and distal parts of the limb are absent or underdeveloped.

3. Hemimelia III degree: In this case, only the proximal part of the limb is absent or underdeveloped.

Treatment and management of hemimelia:

Treatment of hemimelia often requires a multidisciplinary approach and may include surgical, orthopedic, and rehabilitation measures.

Surgical interventions are aimed at restoring the anatomical structure and functionality of the affected limb. This may include surgery to increase bone length, bone grafts, or tissue grafts.

Orthopedic treatment involves the use of orthoses, prostheses, and other devices to support and improve mobility of the affected limb.

Rehabilitation plays an important role in teaching children with hemimelia various skills and helping them adapt to everyday life. Physical therapy, occupational therapy, and other rehabilitation modalities can be used to strengthen muscles, increase mobility, improve coordination, and develop self-care skills.

Forecast and support:

The prognosis for patients with hemimelia depends on the extent of the lesion, the presence of associated abnormalities, and the availability of appropriate treatment. With early diagnosis and a comprehensive approach to treatment, many children with hemimelia can achieve significant improvements in quality of life and some degree of independence.

However, it is important to note that hemimelia is a chronic condition that requires long-term care and support. Regular consultations with doctors, physical therapists and other specialists will help ensure optimal patient health and development.

Conclusion:

Hemimelia is a rare and complex congenital limb development disorder that can significantly impact a patient's quality of life. A fair understanding of this condition, its classification and treatment approaches allows professionals to provide the necessary medical care and support to patients with hemimelia and their families. Continued research and educational efforts are needed to improve diagnosis, treatment, and quality of life for people suffering from hemimelia.