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Polydactyly is perhaps one of the most common congenital hand and foot anomalies. Tetrapolydactyly (polydactyly 24) is a very rare form of hand and foot anomalies. Postaxial ray polydactyly usually occurs in male blacks without associated congenital abnormalities. We report a case of postaxial ray tetrapolydactyly in a female neonate which occurred...
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Citations
... Polydactyly present as unilateral or bilateral and can affect any limb [9,10]. Polydactyly can be categorized as postaxial, preaxial, or central based on where the duplicate digits are located radioulnarly. ...
... Polydactyly can be categorized as postaxial, preaxial, or central based on where the duplicate digits are located radioulnarly. In the Middle East, the incidence of unilateral polydactyly is much more common as opposed to bilateral polydactyly, with a slight prevalence among women [9]. Moreover, while type A supernumerary digit, which is a welldeveloped extra digit, occurs in the Middle Eastern population, type B, which is a rudimentary and pedunculated extra digit, occurs more frequently among Africans and Caucasians [11]. ...
The term "delta phalanx" is proposed to characterize an uncommon deformity that typically affects the middle phalanx of a finger. It has the appearance of the Greek capital letter delta, meaning it is shaped like a triangle. Because the faulty epiphysis occurs proximally to distally instead of along its usual horizontal course, the bone has a semilunar shape. Functional impairment or significant finger shortening are indications for surgery. A variety of congenital hand anomalies are linked to the delta phalanx. Few cases of middle delta phalanx in the ulnar polydactyly finger have been documented. This case study investigates an extremely uncommon occurrence of metacarpal delta phalanx in an ulnar polydactyly finger.
... It can be transmitted through autosomal recessive or dominant inheritance. Having four excess fingers and toes, which is called tetrapolydactyly, is considered very rare [15]. There are syndromes such as Ellis van Creveld that combine congenital heart defects and polydactyly in addition to other manifestations. ...
Introduction
Caroli disease is multifocal segmental dilatation of the large intrahepatic bile ducts that connect to the main duct. It is considered a rare disease with an incidence rate of 1 in 1,000,000 births. There are two types of Caroli: the first type is the simple type, Caroli disease, which includes only cystic dilatation of the intrahepatic bile ducts. The second is called Caroli syndrome, which consists of Caroli disease and congenital hepatic fibrosis and might lead to portal hypertension leading to esophageal varices and splenomegaly. Atrial septal defect is one of the most common congenital heart diseases, occurring when the connection between the left and the right atriums fails to close. Polydactyly is one of the most common congenital malformations of the hands and feet. It manifests in excess fingers on the hands or toes.
Case presentation
A 6-year-old Arab girl presented to the hospital with abdominal pain for the last month with abdominal enlargement. The patient was already diagnosed with Caroli disease and polydactyly (six fingers on each limb) when she was born. Investigations including complete blood count, blood smear, bone marrow biopsy, esophagoscopy, abdominal ultrasound, and computed tomography scan showed splenomegaly associated with hypersplenism, fourth-grade non-bleeding varices, intrahepatic cystic formations in the left and right lobes, and an atrial septal defect with a left-to-right shunt. The patient was scheduled for a splenectomy after she was vaccinated with the appropriate vaccines. After follow-up for a week in the hospital, complete blood count showed an improvement. A month after that, the patient had liver abscesses and biliary fistula that were treated appropriately and her symptoms resolved.
Conclusion
The association of liver diseases, polydactyly, and congenital heart diseases is extremely rare and was only documented few times in the literature. However, to our knowledge, atrial septal defect has never been part of this combination before. The family history also makes this case unique and strongly suggests genetic etiology.
... It is invariably associated with phenotypes (syndromic polydactyly) or may present as a separate entity (non-syndromic polydactyly) [1,2]. It can present in isolation or as a part of a syndromic disease [3,4]. When it occurs in isolation, it is typically inherited in an autosomal dominant fashion [5]. ...
... On the other hand, it usually tends to be autosomal recessive when it is associated with other diseases and syndromes [6]. Some of the associated syndromes include Holt-Oram syndrome, Down's syndrome, polycythemia, Meckel syndrome, Laurence-Moon-Bardet-Biedl syndrome, Patau's syndrome, and Klippel-Trenaunay syndrome [3]. ...
Heptadactyly and hexadactyly are rare congenital disorders from the polydactyly family. This type of polydactyly is usually classified into three major groups: preaxial (medial ray), postaxial (lateral ray), and central polydactyly. The most common presentation is both preaxial and postaxial polydactyly. The occurrence of heptadactyly and hexadactyly has been reported but the presence of both in the same infant has not been reported yet. We report the presence of both these abnormalities in the same infant.
... Polydactyly is a common congenital abnormality of the hands and feet characterized by more than five fingers and toes [1][2][3][4][5][6][7] . It was first described by Theodor Kerchring in the 17 th century [3] . ...
... It was first described by Theodor Kerchring in the 17 th century [3] . Polydactyly is amongst the commonest inherited skeletal conditions seen at birth [1,2,6] occurring in 1 in 500 live birth [7,8] . Polydactyly may be an isolated congenital abnormality or syndromic associated with other congenital anomalies [2,3,5,7,9] . ...
... Polydactyly is amongst the commonest inherited skeletal conditions seen at birth [1,2,6] occurring in 1 in 500 live birth [7,8] . Polydactyly may be an isolated congenital abnormality or syndromic associated with other congenital anomalies [2,3,5,7,9] . Polydactyly may also be described as familial when there is a family history or sporadic when there is no family history [10] . ...
Background: Polydactyly is a common congenital abnormality of the hands and feet characterized by more than five fingers and toes. Polydactyly is amongst the commonest inherited skeletal conditions. Polydactyly can be familial or sporadic. Methods: This article presents a case of familial postaxial polydactyly in a male neonate involving all four limbs seen in our hospital. The mother has history of postaxial polydactyly in both upper limbs consisting only of soft tissue unlike the index patient who has well developed bone on the extra digit. Conclusion: Polydactyly is easily diagnosed at birth however earlier diagnosis during the early antenatal period provides an opportunity for counselling, investigation for syndromic associations or familial gene transmission and planning treatment.
... 1 There are various predisposing factors to this condition, such as genetics, race, ethnic background, and syndromes such as Down syndrome. 2 Postaxial polydactyly is the most common type of polydactyly in African descendants, 1 whereas preaxial polydactyly is the most common among Japanese patients. 3 This case is unique because it does not fit any of the current classification systems mentioned in the literature. ...
... Polydactyly can involve either upper or lower limbs, or both, and can present as unilateral or bilateral. 2,5 In the Middle East, women are more commonly affected than men, and usually present with unilateral and not bilateral polydactyly. 2 African and Caucasian variants usually appear as pedunculated nonfunctioning digits, but the Middle Eastern variety is more likely to present as a soft tissue nubbin. ...
... 2,5 In the Middle East, women are more commonly affected than men, and usually present with unilateral and not bilateral polydactyly. 2 African and Caucasian variants usually appear as pedunculated nonfunctioning digits, but the Middle Eastern variety is more likely to present as a soft tissue nubbin. 4 The diagnosis of polydactyly is usually clinical; however, the exact classification may be determined using AP and lateral view radiographs. ...
Polydactyly is defined as an extra finger or toe that is present at birth. It is a congenital anomaly that can manifest as a single disorder or as a component of a syndrome. Treatment can be conservative or operative depending on the anticipated function of the extra digit. In this report, we describe a case of a bifid 5th digit on the right hand. The aim of this report was to report a new variant of type IIIB described by Duran (2015). This will possibly lead to the modification of the current classifications, which will improve the diagnosis and treatment of patients.
... [10] In some cases there is an extra digit present on both hands and feet (Polydactyly 24). [11] Temtamy and McKusick classification is the most widely used classification system employed to characterize polydactyly and it is broadly classified into Preaxial Polydactyly PPD, postaxial polydactyly PAP, and complex polydactyly all with various sub classifications. [4] Patients most times present in the neonatal period or in the 1 st few months after birth. ...
Polydactyly is a duplication of the digits of the hands or feet. It is a congenital anomaly and can manifest as a single disorder or as a component of a syndrome. It is typically an autosomal dominant inherited condition with variable expression and incomplete penetrance. It is genetically heterogenic being caused by mutations in different genes. It manifests in different forms, based on the position of occurrence of the extra digit. It can occur as a single disorder as a component of a syndrome. Its presentation ranges from just a skin tag to a complete digit with the accompanying metatarsal or metacarpal bone. It can result in functional impairment or it may just be a cosmetically unacceptable digit with no functional impairment. Treatment depends on the nature of the extra digit as well as any functional impairment it may be causing. Surgical excision is employed and various reconstruction procedures are also employed in treatment, and must be well planned out to avoid a worsening of function. We present two cases; a 3-month-old baby girl presenting with a hereditary preaxial polydactyly (PPD) (Wassel type V) of both feet and a 29-year-old man with isolated postaxial polydactyly of the right foot with which was symptomatic.
