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Syndactyly, Type 4

Syndactyly Type IV


Presentation

  • Case Report We present a healthy 2-year-old boy ( Figure 1 ) of first gestation and full-term pregnancy from healthy young parents in a non-consanguineous marriage.[omicsonline.org]
  • Diagnosis/patient presentation Syndactyly can be present in a large variety of forms as it is only a descriptive term.[plasticsurgerykey.com]
  • Hypoplastic thumb can present by itself or with other radial deficiencies. In 60 percent of cases, both thumbs are involved.[surgery.wisc.edu]
  • Spring-Summer 2011 collections are presented at the fas[kurier.at]
  • The thumb is usually present, but it may be short. In severe symbrachydactyly, the baby has either a partial thumb or no thumb and no fingers.[nyulangone.org]
Pain
  • Utilize the very latest approaches in hip surgery including hip resurfacing, hip preservation surgery, and treatment of hip pain in the young adult; and get the latest information on metal-on-metal hips so you can better manage patients with these devices[books.google.com]
  • The child will have had pain relief in theatre but when this wears off, pain relief will be given in the form of medicines to be swallowed. Their hand or foot will be covered in a large bandage to protect the skin grafts while they heal.[gosh.nhs.uk]
  • He'll be in a lot of pain and I'll have to give him pain medications. I don't know for how long, it depends on the baby. This all freaks me out. I crave someone to relate to on this.[mybabywithsyndactyly.blogspot.com]
  • When to call the doctor Following surgery, parents should call the doctor if any of these symptoms occur: Unexpected bleeding from the wound An oral temperature of over 101 degrees Pain that is nor relieved by pain medication A persistent dry cough Nausea[healthofchildren.com]
  • Your child should feel no pain nor have any memory of the surgery. This surgery is usually performed on children between the ages of 1 and 2. This is when the risks of anesthesia are lower.[healthline.com]
Disability
  • The soft-tissue syndactyly in the feet of this patient caused neither disability nor functional loss and did not impair the ability to perform any activity, including walking or running.[omicsonline.org]
  • Hand deformities can be particularly disabling as the child learns to interact with the environment through the use of his or her hands.[hopkinsmedicine.org]
  • Treatment is recommended in patients with a fixed pronation of more than 60 and complaints of disability in daily life.[plasticsurgerykey.com]
Down Syndrome
  • More common causes include Down syndrome and hereditary syndactyly, for some children, syndactyly is only one feature of a more complex genetic condition or other syndrome like Apert, Carpenter, Pfeifeer or Smith-Lemli-Opitz syndrome.[omicsonline.org]
  • syndrome 10 neurofibromatosis type 1 11 Non syndromic isolated polydactyly, a.k.a. polysyndactyly isolated brachydactyly, a.k.a. brachysyndactyly isolated ectrodactyly Distribution As a whole the 2 nd and 3 rd digits tend to be most frequently associated[radiopaedia.org]
Nail Abnormality
  • When more fingers are distally fused they can be flat to very cupped with anomalous nails; abnormal bones with different lengths and abnormal located insufficient developed joints.[plasticsurgerykey.com]
Fracture
  • See how to proceed better than ever before with 45 surgical videos demonstrating hip revision, patellar tendon allograft preparation, open reduction internal fixation clavicle fracture, total shoulder arthroplasty, total elbow arthroplasty, and more -[books.google.com]
Hip Pain
  • Utilize the very latest approaches in hip surgery including hip resurfacing, hip preservation surgery, and treatment of hip pain in the young adult; and get the latest information on metal-on-metal hips so you can better manage patients with these devices[books.google.com]
Small Hand
  • The clinical manifestations of the homozygous phenotype include very small hands and feet with short digits, complete soft tissue syndactyly involving all four limbs, complex polydactyly, distortion of the tubular bones in the hands and feet, and abnormal[rrnursingschool.biz]
Genu Valgum
  • The knees demonstrate mild to moderate genu valgum. The feet have simple and complete syndactyly. No acrosyndactyly of feet was encountered in contrast to the hand. If present, medial polydactyly is mostly at the base of the first metatarsal.[plasticsurgerykey.com]
Suggestibility
  • Winter RM, Tickle C (1993) Syndactylies and polydactylies: embryological overview and suggested classification. Eur J Hum Genet 1: 96-104. Malik S (2012) Syndactyly: phenotypes, genetics and current classification. Eur J Hum Genet 20: 817-824.[omicsonline.org]
  • The diagnosis may be suggest if the digits appear to contantly move together 7. The fingers may appear constantly deformed with complex syndactyly 6.[radiopaedia.org]
  • There is no evidence to suggest that having fully or partially webbed toes affects swimming ability. Syndactyly occurs on its own as an isolated condition or as a feature of several other conditions, including Apert syndrome and Holt-Oram syndrome.[brighthub.com]
  • Holt-Oram syndrome Fanconi's anemia Blackfan-Diamond anemia imperforate anus cleft palate tibial defects Associated conditions pollex abductus abnormal connection between EPL and FPL tendons, seen in approximately 20% of hypoplastic and duplicated thumbs suggested[orthobullets.com]
  • Some theories suggest that amniotic banding may lead to ring constrictions around a finger or limb. In a few cases, the finger may need to be amputated. Other generalized problems with the skeletal system.[hopkinsmedicine.org]
Spastic Paraplegia
  • The feet were also affected coexistence of syndactyly type III and spastic paraplegia in the same family over multiple generations has raised the possibility that these two genes are linked (Opjordsmoen et al. 1980).[rrnursingschool.biz]
Abnormal Gait
  • The foot deformity causes shoe fitting problems and abnormal gait, and possible pain due to callouses over the prominent fifth and third metatarsal heads.[plasticsurgerykey.com]

Workup

  • […] technique a segmental distal transfer (on-top plasty) Postaxial Polydactyly (Small Finger Duplication) Epidemiology demographics Genetics inherited as autosomal dominant (AD) in African Americans more complex genetics in caucasians and a thorough genetic workup[orthobullets.com]
  • Propanolol therapy should be preceded by a full cardiovascular workup, and be managed carefully by a pediatrician and plastic surgeon due to an increased risk of hypoglycemia, hyperkalemia, and bradycardic hypotension.[surgery.wisc.edu]

Treatment

  • Make informed clinical choices for each patient, from diagnosis and treatment selection through post-treatment strategies and management of complications, with new evidence-based criteria throughout.[books.google.com]
  • They can be lightened significantly with a series of six to 10 pulse-dye laser treatments; additional “maintenance” treatments may be needed every few years after that.[surgery.wisc.edu]
  • Evaluation of the operative treatment of syndactyly. Syndactyly: can web space creep be avoided? New modified method for the surgical treatment of syndactyly. Evaluation of uniform operative technique to treat syndactyly. Burn syndactyly.[wheelessonline.com]
  • Camptodactyly as a spectrum of congenital deficiencies: a treatment algorithm based on clinical examination. Plast Reconstr Surg. 2006;117(6):1897–1905. 3. Hori M, Nakamura R, Inoue G, et al. Nonoperative treatment of camptodactyly.[posna.org]
  • Surgical treatment is not always required to correct these deformities.[hopkinsmedicine.org]

Prognosis

  • Treatment and prognosis The overall prognosis can be extremely variable dependent on the presence of other associated anomalies.[radiopaedia.org]
  • If present, proximal metacarpal synostosis should also be separated early to improve mobility of the fifth ray Outcomes: Prognosis Hand function and prognosis is best with the combination of thumb and little finger separation.[posna.org]
  • Prognosis For children with isolated polydactyly and syndactyly, the prognosis is excellent. After surgery most children will have full use of their fingers and toes.[healthofchildren.com]
  • In young children bone or cartilaginous fusions can be separated by knife or osteotome. 11 Outcomes, prognosis, and complications Outcome can be related to web creep, scarring, graft take, rotation, deviation and loss of function.[plasticsurgerykey.com]

Etiology

  • Campylo arched and Dactylos finger Etiology A consequence of anatomical imbalance between the extrinsics and anomalous insertion of intrinsics to the affected finger Primary etiology: Anomalous lumbrical insertion to the superficial flexor Secondary etiology[posna.org]
  • Hand Malformations Hand and finger malformations represent a challenging area of clinical genetics from the standpoint of understanding the classification as well as in understanding the genetic etiologies, which are often due to single gene mutations[peds.ufl.edu]
  • Etiology The locus associated with SD5 maps to 2q31-q32. Mutations in the HOXD13 gene may be causative. Genetic counseling The condition is inherited as an autosomal dominant trait.[rarediseases.info.nih.gov]
  • Constriction band syndrome (streeter’s dysplasia)  Quite common  Etiology- constricting amniotic band & intrinsic causes have been proposed  Patterson clasificationa. simple constrictions (partial/circumferential) b.[slideshare.net]

Epidemiology

  • Introduction A congential malformation of the hand Three forms exist preaxial polydactlyly thumb duplication postaxial polydactlyly small finger duplication central polydactlyly Preaxial Polydactyly (Thumb Duplication) Epidemiology incidence 1 per 1,000[orthobullets.com]
  • Jordan D, Hindocha S, Dhital M, Saleh M, Khan W (2012) The epidemiology, genetics and future management of syndactyly. Open Orthop J 6: 14-27.[omicsonline.org]
  • Definition Congenital persistence of webbing between digits Epidemiology Most common Congenital Anomaly of hand and foot Autosomal Dominant inheritance Pathophysiology Develops before 6 weeks post-conception Signs Digits attached by persistent webbing[fpnotebook.com]
  • Epidemiology So far, less than ten reports have been described in the literature. Clinical description Soft tissue syndactyly (involving the 3rd and 4th fingers and the 2nd and 3rd toes) may be present.[rarediseases.info.nih.gov]
  • Epidemiology The Danish registry of congenital malformations showed an incidence of malformations of the upper extremities for the period 1984 – 93 of 14.6 per 10 000 live births ( 3 ). The incidence of syndactyly was 2.8 per 10 000.[tidsskriftet.no]
Sex distribution
Age distribution

Pathophysiology

  • Definition Congenital persistence of webbing between digits Epidemiology Most common Congenital Anomaly of hand and foot Autosomal Dominant inheritance Pathophysiology Develops before 6 weeks post-conception Signs Digits attached by persistent webbing[fpnotebook.com]
  • Pathophysiology and treatment. Yale J Biol Med. 1993 May-Jun; 66(3): 143–155. 2. Patterson, TJ. Congenital ring-constrictions. BR J Plast Surg. 1961;14:1-31 3. Miura T. Congenital constriction band syndrome. J Hand Surg Am. 1984;9:82–88. 4.[posna.org]

Prevention

  • Our radiologists have special expertise using ultrasound to look for bone and joint changes so we can work with your child to help prevent future problems.[seattlechildrens.org]
  • Cases of syndactyly usually do not require repair; the aim of web reconstruction for syndactyly of the foot is purely cosmetic [ 10 ], to prevent the associated significant psychological morbidity and allow the patient to gain social acceptance [ 11 ][omicsonline.org]
  • Prevention There is no known prevention for polydactyly and syndactyly. Parental concerns Parents may be concerned about their child's ability to use his hands and feet.[healthofchildren.com]
  • Syndactylism: Results of zig-zag incision to prevent postoperative contracture. Technical modification in repair of syndactylism. Combined double Z plasty and Y-V advancement for thumb web contracture.[wheelessonline.com]
  • Timing Syndactyly of the border digits (thumb/ index finger or ring/ small fingers) is treated at early age to prevent the larger digit from curving towards the smaller digit with growth.[en.academic.ru]

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