An Unusual Variant of Hallermann – Streiff Syndrome

نویسندگان

  • H.S. Narayanan
  • K.S. Mohan
  • K.R. Manjunatha
  • S.M. Channabasavanna
چکیده

The cranial sutures have delayed ossification and fontanelles remain open for a long time and as a result brachycephaly occurs with frontal and parietal bossing and thin calvarium, facial bones are hypoplastic, small beaked nose with receeding, hypoplastic mouth and chin. Low-set ears, thin and light hair, hypotrichosis of scalp, eyebrows and eye lashes, micro-opthalmia with congenital cataract (total or incomplete in 90 % of cases), microstomia, high arched palate, hypoplasia and/or malimplantation of teeth and partial anodontia, micrognathia with hypoplasia of the rami and displacement of the tempero mandibular joint can occur. Nystagmus and squint are common with stiff joints, thin skin and many visible bloodvessels, stunted growth is common.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Mid-diaphyseal Endosteal Thickening With Subsequent Medullary Narrowing in a Patient With Hallermann-Streiff Syndrome

UNLABELLED We report on a 5-year-old girl who presented with the full clinical criteria of Hallermann-Streiff syndrome (HSS). Classically, overtubulation (thin and gracile) bones are the characteristic and constant features in HSS. Interestingly, our present patient manifested unusual mid-diaphyseal endosteal thickening with subsequent medullary narrowing (defective endosteal resorption). To th...

متن کامل

Hallermann Streiff Syndrome-The Oral Manifestations in a Child

Hallermann-Streiff syndrome (HSS) is a rare genetic disorder that is primarily characterized by distinctive malformations of the skull and facial region, sparse hair, eye abnormalities, dental defects, atrophic skin changes and a proportionate short stature. Here we discuss a case of 9 years-old female child who presented with abnormal facial features, dental problems and associated cardiac pro...

متن کامل

A familial study of Hallermann–Streiff–François syndrome

Hallermann-Streiff-François syndrome is a rare sporadic genetic pathology characterized by a phenotype consisting of growth retardation, ocular abnormalities, and a "bird-like head". We hereby report a case of this syndrome found in three generations of the same family - father, daughter, and grand-daughter - who presented with a short stature and facial dysmorphic features, nystagmus, cataract...

متن کامل

Hallermann-Streiff Syndrome: a case report from Turkey.

Hallermann-Streiff Syndrome (HSS) is a rare disorder characterized primarily by head and face abnormalities, with dental abnormalities also present in 50-80 percent of cases. The first description seems to have been made by Aubry in 1893. HSS was first described completely in 1948 by Hallermann, and then in 1950 by Streiff. We report a 4-year-old girl with HSS, presenting oro-dental characteris...

متن کامل

A case of Hallermann-Streiff-François syndrome: an ophthalmological perspective

Malta Medical Journal Volume 29 Issue 02 2017 Abstract Hallermann-Streiff-François syndrome is a rare condition which offers multidisciplinary diagnostic and therapeutic challenges. The difficulty in dealing with these cases is compounded by the presentation at a very young age. The ophthalmologist has an important role in helping to establish a diagnosis and to recognize the need for early tre...

متن کامل

Obstructive sleep apnoea in a puerperal patient with Hallermann-Streiff syndrome.

A 26 yr old puerperal female with Hallermann-Streiff syndrome developed serious obstructive sleep apnoea syndrome during pregnancy. She underwent an elective Caesarean section delivery, but ending the pregnancy did not improve her clinical symptoms. By treating her with nasal continuous positive airway pressure, a worsening of her headaches and glaucoma was prevented. The administration of acet...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره 27  شماره 

صفحات  -

تاریخ انتشار 1985