Edit concept Question Editor Create issue ticket

Congenital Phlebectasia

Bockenheimer Syndrome


Presentation

  • […] if the condition was present at the time of inpatient admission.[icd.codes]
  • .- ) Other congenital malformations of peripheral vascular system Present On Admission POA Help "Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter,[icd10data.com]
  • We present a case of a 15-year-old male with no antecedent history of trauma and an enlarging right neck mass. Pertinent literature and relevant diagnostic and therapeutic modalities are reviewed.[unboundmedicine.com]
  • It is present at birth (congenital) and is progressive during life. The venous dilatation can be seen beneath the skin on the arms and legs. It is more common on a single arm and hand but may occur on both sides.[malacards.org]
Renal Artery Stenosis
  • artery stenosis Q27.2 - Other congenital malformations of renal artery Q27.3 - Peripheral arteriovenous malformation Q27.4 - Congenital phlebectasia Q27.8 - Other specified congenital malformations of peripheral vascular system Q27.9 - Congenital malformation[icd-code.org]
  • artery stenosis Q27.2 - Other congenital malformations of renal artery Q27.3 - Arteriovenous malformation (peripheral) Q27.8 - Other specified congenital malformations of peripheral vascular system Q27.9 - Congenital malformation of peripheral vascular[emedcodes.com]
  • Similar Codes ICD-10 Code ICD-10 Description Q27 Other congenital malformations of peripheral vascular system Q27.0 Congenital absence and hypoplasia of umbilical artery Q27.1 Congenital renal artery stenosis Q27.2 Other congenital malformations of renal[healthprovidersdata.com]
  • artery stenosis Q27.2 Other congenital malformations of renal artery Q27.3 Arteriovenous malformation (peripheral) Q27.30 Arteriovenous malformation, site unspecified Q27.31 Arteriovenous malformation of vessel of upper limb Q27.32 Arteriovenous malformation[icd10data.com]
Disability
  • Martino V, Ferrarese A, Alessandro B, Bullano A, Marola S, Surace A, Gentile V, Bindi M, Solej M, Enrico S Homozygous FIBP nonsense variant responsible of syndromic overgrowth, with overgrowth, macrocephaly, retinal coloboma and learning disabilities.[medicbind.com]
  • These abnormalities have been associated with mental retardation with abnormal EEG findings, speech disability, defective growth, cleft palate, presence of simian lines and an increase in the atd angles of both palms, diffuse demineralization of bony[karger.com]
Failure to Thrive
  • Fred Ferri’s popular "5 books in 1" format provides quick guidance on menorrhagia, Failure to Thrive (FTT), Cogan’s syndrome, and much more.[books.google.com]
  • […] to thrive (FTT). [10] Remarkable reports include atypical CMTC with retinoblastoma. [11] Interestingly, association with tumor syndromes such as meningioma or leukemia have also been reported.[e-ijd.org]
Venous Insufficiency
  • insufficiency (Medical Encyclopedia) [ Read More ][icdlist.com]
Pachyonychia
  • 137 Necrobiosis lipoidica diabeticorum NLD 137 139 Aplasia cutis congenita 139 140 Alopecia areata 140 141 Tinea capitis and kerion 141 142 Traction alopecia and hair pulling 142 143 Ectodermal dysplasias 142 145 Telogen and anagen effluvium 144 146 Pachyonychia[books.google.com]
  • Stay Healthy MD It can be pachyonychia congenita . Another possibility is twenty nail dystrophy. please meet a dermatologist.[healthcaremagic.com]
  • .: nail patella syndrome (Q87.2) Q84.4 Congenital leukonychia Q84.5 Enlarged and hypertrophic nails Congenital onychauxis Pachyonychia Q84.6 Other congenital malformations of nails Congenital: clubnail koilonychia malformation of nail NOS Q84.8 Other[embryology.med.unsw.edu.au]
Piebaldism
  • Epidermal cysts 96 97 Dermoid cysts 97 98 Pilomatricomas and trichoepitheliomas 98 99 Granuloma annulare 98 100 Neurofibroma and lipomas 100 102 Sweat gland tumors 102 104 Postinflammatory hypopigmentation and pityriasis alba 104 105 Vitiligo 105 106 Piebaldism[books.google.com]
Hyperpigmentation
  • Piebaldism 105 108 Tinea versicolor 107 109 Lichen sclerosis 109 111 Morphea 111 112 Nevus depigmentosus 112 114 Hypomelanosis of Ito 114 116 Mongolian spots 116 118 Caféaulait macules CALMs 118 120 Junctional nevi 120 122 Lentigines 122 124 Postinflammatory hyperpigmentation[books.google.com]

Workup

  • This case is presented in view of rarity and suggested that during preoperative workup the nearby structures like carotid sheath should be evaluated by magnetic resonance imaging to avoid such per-operative surprises.[ijoonline.com]

Treatment

  • Sclerotherapy: Treatment of Varicose and Telangiectatic Leg Veins, by Drs. Mitchel P. Goldman, Jean-Jerome Guex, and Robert A Weiss, equips you to implement the latest cosmetic procedures for the treatment of varicose and telangiectatic leg veins.[books.google.com]
  • Surgical treatment of jugular vein phlebectasia in children. Amer J Surg 2006;192:286–90[entcase.net]
  • Babies with birth defects often need special care and treatments. The treatments may include surgery, medicines, assistive devices, and therapies.[icdlist.com]
  • Treatment may include surgery, embolization, and elastic stockings. MalaCards based summary : Genuine Diffuse Phlebectasia, is also known as bockenheimer syndrome. Affiliated tissues include skin.[malacards.org]
  • The authors describe the clinical features, treatment and outcome of this anomaly. A six-year-old child with no particular past medical history presented with a two-year history of progressively enlarging mass on the right side of the neck.[em-consulte.com]

Prognosis

  • It is characterized histologically by diffuse endothelial cell hyperplasia within the reticular dermis rather than the intravascular proliferation typical of reactive angiomatosis Prognosis: The prognosis for cutis marmorata telangiectatica congenita[dailyrounds.org]
  • Prognosis, in uncomplicated cases, is good. Downloads month by month Downloads Download data is not yet available. Article Details 2008-2019 • PAGEPress is a registered trademark property of PAGEPress srl, Italy. • VAT: IT02125780185 • Privacy[pediatrmedchir.org]
  • The diagnosis is mainly clinical, and prognosis is generally good, with cutaneous lesions improving during infancy. There is no specific treatment, and long term follow up is indicated with associated abnormalities.[cags.org.ae]
  • CMTC has a favorable prognosis with partial or complete resolution during adolescence and therefore needs no specific therapy. N.K. Anand, M.S. Pannu, Palwinder Singh, Department of Pediatrics, Government Medical College, Amritsar, Punjab, India.[indianpediatrics.net]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • Abstract Fusiform dilation of the jugular vein, or jugular venous phlebectasia, is a rare clinical entity, with an etiology of cervical swelling.[unboundmedicine.com]
  • , and congenital causes are the most commonly proposed etiologies [4–7].[entcase.net]
  • Case Discussion Phlebectasia is an uncommon condition of the venous system, possibly of a congenital etiology. Clinically, phlebectasia usually presents as a neck mass that enlarges with Valsalva maneuver.[radiopaedia.org]
  • Teratogens and autosomal dominant mode of inheritance with incomplete penetrance have been considered as etiological factors. Prognosis, in uncomplicated cases, is good. Downloads month by month Downloads Download data is not yet available.[pediatrmedchir.org]

Epidemiology

  • IJP is a rare condition and its epidemiologic features have not been well documented. In recent years, its incidence has increased.[e-aps.org]
  • Epidemiology [ edit ] Usually observed at birth or shortly thereafter in 94% of patients, [9] in other reports, patients did not develop skin lesions until 3 months or even 2 years after birth. [13] [14] Females are typically affected more often than[en.wikipedia.org]
  • Epidemiology; Epidemic or AIDS-associated. 2. Clinical Lesions: A multi-centric systemic vascular tumor characterized by violaceous nodules and by edema secondary to lymphatic obstruction. 3. Clinical Variants of KS a. Classic KS b.[web.sapmed.ac.jp]
  • Epidemiology Frequency United States The frequency of this disorder is not known. It may be more common than reported, because it is usually a benign disorder, and most cases that are reported have an associated malformation.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Features may overlap. [1] , [2] , [3] Further the variability in presentation with overlap, which is seen with both these disorders may have a common pathophysiology related to the Happle gene hypothesis.[ijpd.in]
  • Pathophysiology: The pathogenesis of cutis marmorata telangiectatica congenita (CMTC) remains unclear. Its cause may be multifactorial. Most cases occur sporadically, although rare cases occur in families.[dailyrounds.org]
  • Although the left IJV valves were clearly visible on the DUS scan (Figure 2), a functional undetectable valvular deterioration or incompetence of the left IJV valves might be the reason for possible pathophysiology.[tgkdc.dergisi.org]
  • Petrozzi reported the first case of CMTC in the United States in 1970. [8] CMTC is believed to be more common than suspected, as studies have shown that milder forms of the disease are not being recognized as CMTC. [6] The pathophysiology is still unclear[en.wikipedia.org]
  • Pathophysiology Doppler studies have shown a decreased flow rate of red blood cells, suggesting an abnormality in the vascular innervation of affected areas.[dermatologyadvisor.com]

Prevention

  • - 0% Emergent - ED Care Needed - Preventable/Avoidable - 0% Emergent - ED Care Needed - Not Preventable/Avoidable - 0% Primary diagnosis of injury 0% Primary diagnosis of mental health problems 0% Primary diagnosis of substance abuse 0% Primary diagnosis[codelay.com]
  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.com]
  • Centers for Disease Control and Prevention Intersex (Medical Encyclopedia) [ Read More ] Varicose Veins Varicose veins are swollen, twisted veins that you can see just under the skin.[icdlist.com]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!