BACKGROUND: Macrodactyly is a rare congenital malformation characterized by an increase in the size of all structures of a digit, accounting for less than 1% of all congenital upper extremity conditions.
METHODS: We report a case involving a 49-year-old woman who presented for the first time with untreated, radial-sided hand macrodactyly. We performed soft tissue debulking, amputation, median nerve neurotomy and coaptation, and carpal tunnel release. At the 6-year follow-up, no significant growth was observed in the bone or soft tissue of the affected area.
CONCLUSIONS: Tissue overgrowth in patients with progressive macrodactyly can continue and progress excessively with age. Median nerve neurotomy and coaptation play a crucial role in preventing recurrence of the deformity.

Macrodactyly is a rare congenital limb difference manifesting as an overgrowth of one or more fingers or toes. The pathological process affects all tissues of the ray in the hand or foot. The enlargement can significantly alter the limb\'s appearance and impair its function. The role of a pediatrician is to distinguish isolated macrodactyly from syndromic conditions (including PIK3CA-Related Overgrowth Spectrum) or mimicking conditions to enable early interdisciplinary consultation and treatment planning. The psychological stigma associated with this often disfiguring condition necessitates support for patients and their family. We present a practical guide for physicians who might be the first to raise suspicion of macrodactyly and initiate further diagnostics to achieve adequate treatment and support for children and caregivers.

UNASSIGNED: Megadactyly of the foot is uncommon non hereditary congenital anomalies of the extremities and poses a dilemma on treatment however multiple treatment modalities were developed but is not uniform to all patients with megadactyly. The goal of the surgical treatment is to achieve painless and function of the foot.
METHODS: We report a 14 years old male presented with complaints of progressive enlargement 2nd, 3rd, 4th and 5th toes of the right foot since birth, associated with inability to wear shoes. One month prior to admission he experienced gradual onset painful forefoot and toes that was increasing in severity with time associated with inability to walk normally. He is the first born in a family of four children and his other siblings are healthy with no anomalies. On clinical evaluation, he was health with stable vitals, with enlarged 2nd, 3rd, 4th and 5th toes of the right foot with no tenderness with intact neurovascular status. On clinical and radiological evaluation he was diagnosed with congenital megadactyly of the right foot, a multidisciplinary team including orthopedic surgeons and prosthetics team agreed to do trans-metatarsal, then partial foot prosthesis fabrication. He underwent trans-metatarsal amputation of the affected foot and tissue sample was taken for histopathology. The histological findings revealed bone enlargement with increased proliferation of subcutaneous adipose tissues with increased fibrous septae together with thinning of the epidermis, features suggestive of lipomatosis. The wound site healed very well after 14 days stitches were removed and the child was scheduled for follow-up after six weeks, 12 weeks and 6 month post-surgery. On the last visit he was free from pain on his right forefoot and toes, able to wear fabricated partial foot prosthesis and shoes normally, walk with no incapacitation.
CONCLUSIONS: Our case report is unique due to the involvement of the multiple toes of the right foot with syndactyly at third and fourth toes and its management is challenging because there is no uniformity in its surgical treatment, in our case trans-metatarsal amputation was done and the patient progressed well after six months of follow up.
CONCLUSIONS: Foot megadactyly is uncommon congenital malformation, most common on the right foot. Regardless of the dilemma on treatment, the trans-metatarsal amputation and a fabricated prosthesis to our patient fulfilled the goals of painless right foot and able to wear shoes and walk normally with no impairment.

BACKGROUND: Neural fibrolipoma, also known as fibrolipomatous hamartoma (FLH), is a rare benign tumor that usually affects the upper limb and tends to develop near the n. median. FLH is a rare birth defect defined by the noncancerous growth of fibroadipose tissue around nerve bundles. These conditions are associated with abnormal bone growth, resulting in macrodactyly in about one-third of cases. The illness is medically referred to as macrodystrophia lipomatosa (MDL).
METHODS: A-4 years old girl presents with the index finger and thumb larger than the surrounding fingers and has been present since birth. Over time, the index finger and thumb continue to enlarge. This enlargement is accompanied by pain, a tingling sensation, and occasional bluish discoloration, especially at night. The patient is the third child out of four siblings, with a history of normal birth and no abnormalities in other parts of the body. The patient can grip objects in daily activities, but there is noticeable stiffness in the right hand.
CONCLUSIONS: FLH with macrodactyly is an uncommon abnormality that can manifest as either a minor lesion or a big mass affecting the entire extremity. Clinically, it is challenging to challenging to diagnose FLH in patient with macrodactyly.
CONCLUSIONS: It is crucial to possess a thorough understanding of the distinctive histology and radiological findings in order to accurately diagnose and treat the condition.

Macrodactyly, a often congenital anomaly, entails abnormal enlargement of digits, predominantly affecting hands or feet, either in isolation or as part of a syndromic condition. The authors present a case of Macrodystrophia Lipomatosa (ML), a form of macrodactyly, in a 62-year-old patient, emphasizing macrodactyly manifestations through clinical and radiological assessments. Additionally, the authors explore anatomical aspects related to nerve distribution in affected digits, providing a comprehensive understanding of ML. Teaching Point: Explore macrodactyly, emphasizing digits nerve plexus anatomy, which can reveal crucial clues for diagnosing a specific form of this anomaly.

Klippel-Trenaunay syndrome (KTS) is a rare genetic syndrome comprising an abnormal development of soft tissues and the lymphovascular system with bony overgrowth, venous malformation, and port wine stains. We present an interesting case of a three-year-old child brought to our hospital with a swollen limb and raised skin lesions associated with bleeding from minor trauma. Most of the clinical characteristics of KTS were seen in our patient, including arteriovenous, soft tissue, capillary, and lymphatic abnormalities. The diagnosis of KTS is based on clinical examinations and imaging investigations. He had gross hypertrophy of the left lower limb with measurable lengthening compared to the opposite limb. Ultrasonography of the left limb revealed soft tissue hypertrophy with abnormal venous communication. The management of KTS is mainly symptomatic and should be approached conservatively if the patient has functional limbs without edema, bleeding, ulceration, or pain.

Macrodactyly is a rare congenital anomaly characterized by disproportionate hypertrophy of one or more digits or the forefoot, involving some or all tissue types. It is nonhereditary and can present alone or alongside other deformities. Usually, macrodactyly is treated with amputation of the affected toe or finger to reduce the chance of recurrence. In this paper, we present the case of a child with macrodactyly who was treated successfully without amputation and instead with a reconstruction of the toe shape to resemble a near-natural-looking toe with intact functions. The patient was a one-year-old female who presented with macrodactyly of her right great toe, right second toe, and forefoot. She had no history of other congenital deformities or systemic diseases. A reconstruction surgery was performed, which involved debulking the right great toe, right second toe, and forefoot. Also, it included the creation of the first web space and the restoration of the nailbed of the second toe. Postoperative follow-up revealed minimal complications. Thus, a second reconstructive surgery was performed, which included debulking and further reconstruction of the foot to improve the result. Several techniques exist for the reduction of macrodactyly that can achieve optimal results. The choice of technique depends on the specifics of the case and the experience of the surgeon. We therefore hope our technique will be beneficial for the management of future cases of macrodactyly. One year of follow-up after the second operation revealed maintained function and no regrowth recurrence.

PIK3CA-related overgrowth spectrum (PROS) is a heterogeneous group of diseases, with varied clinical presentations ranging from isolated segmental overgrowths to megalencephaly and vascular malformations, all resulting from post-zygotic activating mutations in PIK3CA. Isolated macrodactyly of upper limb is extremely rare, accounting only for 0.9%-1% of all congenital anomalies of the upper limb. This report describes a case of congenital, isolated, nonprogressive macrodactyly of the right index finger and thumb, in an adult patient that was treated with debulking surgery. The microscopic features were compatible with lipomatosis of nerve. Due to the prompt and pertinent molecular testing, which identified a somatic PIK3CA variant, c.3140A > G, p.H1047R., the case was classified as a PROS. The availability of mTOR inhibitors offers additional treatment possibilities in cases with progressive disease. This case report highlights the importance of molecular testing to identify PROS, to further the knowledge of this continually expanding entity.

Macrodactyly is an uncommon, not inherited congenital malformation of the digit with unknown prevalence and path of pathogenesis. The condition was described in 1940 and since then 107 cases were reported. Manifestations may mislead the diagnosis of hemangiomas and lymphangiomatosis. There are different options for treatment without a clear consensus. The authors are presenting a macrodactyly case that improved the quality of his life after he underwent surgical amputation of the toes.
UNASSIGNED: The authors had a case of a 2-year and 4-month-old male child presented with progressive growth of the left foot toes; which started since birth in the 4th toe and then involved 3rd and 5th toe later; resulting in deformity and difficulty in wearing shoes. Physical examination; showed left foot enlargements of the 3rd-5th toes. X-ray of the left foot was done he was diagnosed to have macrodactyly. Under general anesthesia metatarsophalangeal joint of the 4th-5th toe and distal interphalangeal joint of the 3rd toe, disarticulation was done. The patient is doing okay on follow-up for the last year.
UNASSIGNED: Consistently with other case reports from Korea, Tanzania, and Congo our patient presented with a primary type of left foot macrodactyly in his early life, and he was successfully managed with amputation of the affected digits.
UNASSIGNED: This is one of the rare cases which needs a high index of suspicion to diagnose and treat early to improve quality of life. Amputation is the most important management in resources limited areas.

Macrodystrophia lipomatosa is a rare form of nonhereditary congenital localized gigantism involving upper and lower limbs and is characterized by overgrowth of all the mesenchymal elements predominantly fibro-adipose component, in the distribution of a particular nerve, usually median nerve. It usually presents with progressive painless overgrowth of the involved limb, toe, or digit and is associated with macrodactyly. It might cause limitation of the movement of the involved part. Imaging has an important role in diagnosing this condition and differentiating it from malignant mimics. Imaging findings include hypertrophy of the mesenchymal elements of the involved digits and/or limbs predominantly fibro adipose component with associated overgrowth of the phalanges. In this case report, we present a case of unilateral involvement of index finger and thumb with associated macrodactyly.