Monoblock Craniofacial Internal Distraction in a Child with Pfeiffer Syndrome: A Case Report

A 7-yr-old boy visited our surgical center with Pfeiffer syndrome type 1, presenting with macrocrania, broad big toe and thumb, exophthalmos, tongue protrusion, malocclusion with midfacial retrusion, mild respiratory difficulty due to minor upper airway obstruction, and developmental delay. He also exhibited anthrophobia with a passive character. The patient was treated with internal monoblock distraction osteogenesis to increase the intracranial and intraorbital volumes, and the nasal and pharyngeal airway spaces using two modular mid-facial internal distractors. For distraction, the latency period was 1 week, the daily activation of 1.0 mm was 20 days (total advancement 20 mm at the midline), and the consolidation period was 3 months. The follow-up computed tomography 12 months after surgery showed expansion of the brain and proper ossification in the distracted area. The patient also showed aesthetically good cranial contours, improved tongue and eyeball protrusion, no respiratory difficulty, and improved learning. We suggest that the internal distraction may last longer than an external type, resulting in a better bone fusion rate and successful expansion of craniofacial bones.

A Case of Pfeiffer Syndrome

Pfeiffer Syndrome is as rare as Apert syndrome in the Western population. This condition is very rare in the Asian population and has not been previously reported in Korea. The authors report with a review of literature the case of a newborn baby with Pfeiffer syndrome, manifested by bicoronal craniosynostosis, broad thumbs, and big toes. The infant also had bilateral syndactyly of the fingers and toes, mild proptosis, choanal hypoplasia and maxillary hypoplasia.

Treatment of Penile Vaselinoma Using Thick Split Thickness Skin Graft

The problems of foreign body injection such as vaseline, paraffine, mineral oil for penile augmentation done by illegal medical practitioners are tissue inflammation, necrosis, granuloma formation, deformity and erectile dysfunction. The treatment of these complication are composed of foreign body removal and resurfacing of denuded penis. Many resurfacing procedures are introduced such as split thickness skin graft, flap-to-graft conversion method, scrotal flap, groin flap and free flap. Skin graft is the simplest method with minimal donor site morbidity, but there are some complications like scar contracture, hypertrophic scar and difficulty of erection. Thick split thickness skin graft can prevent these complications. We measured the length, circumference and surveyed erectile function with International Index of Erectile Function(IIEF) after long-term follow up. Increase in mean length and circumference at erection are 43% and 36%, respectively, compared to resting, and the survey with IIEF had a better result than that of normal control group. We found preserved erectile function without scar contracture and hypertrophic scar. Thick split thickness skin graft is the good method for treatment of penile vaselinoma with simplicity, minor donor site morbidity and preservation of erectile function.

The Efficacy of Colostomy for the Sacral or Ischial Pressure Sore in Spinal Cord Injury Patients

Pressure sore is a necrotic ulceration of the skin and underlying tissue, and usually occurs over the bony prominences of the body after prolonged or repeated pressure. The purpose of this study is to evaluate the effectiveness of colostomy performed as an adjunct procedure to heal pressure ulcers on the sacral or ischial area in patients with spinal cord injury. We reviewed the data from 19 spinal cord injury patients who needed surgery for treatment of the sacral or ischial pressure sore. Of these 19 patients, 7 underwent colostomy before the surgery for the pressure sore. The range of organisms isolated in the group that underwent colostomy was compared with that of the other group. The patients who underwent colostomy before the surgery were asked about bowel care, dietary management of the fecal stream and their satisfaction with colostomy. This study showed improvement of the infection control in the postoperative wound after colostomy. All 7 patients expressed their satisfaction with the procedure and didn't undergo colostomy closure. Our conclusion is that colostomy may be an appropriate ancillary treatment for some spinal cord injury patients with the sacral or ischial pressure sore.

Malignant Melanoma on Congenital Melanocytic Nevus

Congenital melanocytic nevi are considered to be precursors of malignant melanoma. Although the risk of malignant melanoma with medium and small congenital melanocytic nevi is uncertain, it is important to notice the possibility of malignant transformation in those lesions. We describe a 62-year-old woman who had had a brown soft verrucous tumor on her right lower back since birth. She first noticed a black nodule in the center of the tumor 5 years before which had ulcerated 3 months prior to presentation without healing. A biopsy specimen revealed malignant melanoma arising from a congenital melanocytic nevus.