There is a deep cleft seen on the affected side of the chin. There is excessive exposure of teeth on the affected side during smiling. Muscle bulk is comparatively less on the affected side. On clenching, the masseter and temporalis muscles appear prominent. There is scarring in the infraorbital region extending till hair line in front of the ear.
Skin is pigmented more on the right zygomatic region, ramus region and the corner of the mouth. Due to complete loss of soft tissue bulk in the cheek and the mandibular region on the affected side, there is stretching of skin on the entire aspect of the right side, causing depression of ala of nose, retraction of upper and lower lip, prominent exposure of infraorbital rim and zygoma, angle of mandible. There is also loss of hair in the lower eye lid. The eye looks sunken on the affected side due to loss of orbital fat 14. There is loss of soft tissue bulk on upper and lower eye lid. On palpation, the supraorbital, infraorbital, zygoma, maxilla and mandible appear almost symmetrical on both the sides. Atrophy of soft tissue is seen in the infraorbital, zygomatic and mandibular region. Zygoma, maxilla and mandible appear asymmetrical on the affected side.
Nasal bones appear normal with depression seen on the affected side of nose close to the tip. Supra orbital margin appears normal with a prominent infraorbital margin. Enopthalmus of the right eye due to atrophy of orbital fat occurs. Shape of the forehead is asymmetrical with a depression on midline of forehead close to glabella.
Depression or furrow is present at anterior fontanelle region and at the mid line of fore head close to hair line. Flattening is seen in the cranial vault at midline extending till hair line.
The head is flat in the anterior region along the midline. The shape and symmetry of head is abnormal with a cleft on the midline. The presence of antinuclear antibodies in the serum suggested that the Parry-Romberg syndrome may be a form of localized scleroderma. Occasionally, there may be some neurological complications, such as trigeminal neuralgia, facial paresthesia, severe headache and contra lateral epilepsy. The extension of the atrophy is frequently limited to one side of the face 16, and cranium, it may occasionally spread to the neck and one side of the body and it is accompanied usually by ocular involvement, the most frequent manifestation is enophthalmy, deviation of mouth and nose to the affected side, and unilateral exposure of teeth (when the lips are involved). 10 It is uncommon and unilateral with a higher incidence rate in females 18. Onset of Disease occurs at first decade of life and progresses slowly over years frequenly for 2 to 10 years and reaches a stable burn out phase. This was first described by Parry and Romberg in the year 1846. Hemifacial atropy is a uncommon degenerative slowly progressing unilateral atrophy of the facial tissues including the skin, subcutaneous fat, muscle, cartilage and bone. Parry Romberg Syndrome-A Review of Treatment Options. Sargunar B, Ebenezer V, Balakrishnan R, Priya S. Hemifacial atrophy Parry-Romberg syndrome physiopathology treatment optionsÄownload this article as: Copy the following to cite this article:
These have included free fat grafts, dermis fat grafts, fascia,muscle grafts, cartilage and bone augmentation ,Orthognathic surgery,microvascular reconstruction and stem cell regeneration of bone and soft tissues. A review of literature with variety of treatment options have been discussed in an attempt to treat the atrophic deformties from mild to severe cases. The objective of this work is, to accomplish a literature review concerning general characteristics, etiology, physiopathology, differential diagnosis and treatment of progressive hemifacial atrophy. Other possible factors that are involved in the pathogenesis include trauma,viral infections, heredity, endocrine disturbances and auto-immunity. A cerebral disturbance of fat metabolism, atrophic malformation of Cervical Sympathetic Nervous System has been proposed as the primary cause. Parry-Romberg Syndrome, also known as Progressive Hemifacial Atrophy, is an uncommon degenerative condition, characterized by a slow and progressive atrophy affecting one side of the face. Balakrishnan and Swarna PriyaÄepartment of Oral and Maxillofacial Surgery, Sree Balaji Dental College and Hospital, Bharath University, Chennai, 600100, India.