A successful operation for giant intra-abdominal desmoid tumors associated with familial adenomatous polyposis: A case report.

Desmoid tumors are benign proliferations of spindle cells originating in fibro-aponeurotic tissue. Many patients with familial adenomatous polyposis (FAP) die from desmoid tumors, which can arise spontaneously but often appear to be surgically induced by prophylactic colectomy. Desmoid tumors are the second most common cause of death in patients with FAP, second to colorectal cancer. Many patients can live a long life with desmoid tumors without symptoms, but when symptoms (ranging from bowel or ureteric obstruction to bowel perforation with abscess and fistula) appear or there is a risk of functional impairment, a wide spectrum of therapies (local and systemic) are valuable in improving the symptoms and controlling the disease. A half-Japanese, half-Caucasian male, who had been diagnosed with intra-abdominal desmoid tumors associated with FAP at age 13, was treated using abdominal wall incision for decompression and chemotherapy from the age of 38. The therapeutic outcome was progressive disease, based on the modified response evaluation criteria in solid tumors (mRECIST), and when he visited our hospital at age 41 the desmoid tumor had invaded the small bowel with a fistula to the abdominal wall. We performed a palliative operation to improve his symptoms, which were fever, abdominal pain, vomiting, and difficulty eating. As the tumor was extremely large and had invaded the small intestine, massive resection including the small intestine was required. To prepare for anticipated massive bleeding, a balloon catheter was placed in the superior mesenteric artery just prior to surgery. Although the operation was extremely difficult, following surgery the patient regained his ability to eat and when discharged was ambulatory and without short-bowel syndrome. We report our experience treating one of the largest reported intraperitoneal desmoid tumors. Resection resulted in a good postoperative course, with improved quality of life and prognosis.

17-year Follow-up after Mandibular Distraction Performed in an Infant with Robin Sequence.

Robin sequence is a congenital anomaly, first described by Pierre Robin as a triad of micrognathia, upper airway obstruction and glossoptosis. The most significant airway management technique is the use of distraction osteogenesis to advance the mandible. On the other hand, late complications such as permanent dentition loss and malformation of the damaged teeth have been reported. Therefore, long-term follow-up after mandibular distraction is important. In our case report, we describe 17 years of follow-up after mandibular distraction performed in a 4-month-old male infant with Robin sequence. The patient was a male with a cleft palate. The tracheotomy and tongue-lip adhesion was performed 1 month after birth, because the percutaneous oxygen saturation decreased to 70% during feeding and crying. However, postoperative X-ray examination revealed that he could not obtain sufficient airway after the operation. For the purpose of closing the tracheotomy early, we performed bilateral mandibular distraction at the age of 4 months. The bilateral corticotomy was performed on the mandibular body and an internal traction device was placed. The mandible was bilaterally lengthened by 17.5 mm, and expansion of the airway was confirmed by X-ray. A cleft palate operation was performed at the age of 17 months. At the age of 3 years, the tracheostomy orifice closed and speech/language training and orthodontics were begun. Dental panoramic radiographs showed favorable mandibular growth. No additional operation has been performed. Finally, his orthodontic treatment was completed without any complications at age 17.

Ten-year Follow-up After Treating Extended Burn Scar Contracture with an Autologous Cultured Dermal Substitute.

This is the first case report of long-term follow-up after applying the autologous cultured dermal substitute to establish the wound bed before split skin graft. The results suggest that application of autologous cultured cultured dermal substitute contributes to establish the high-quality wound bed for skin graft. Split-thickness skin grafts (STSGs) are the gold standard for the treatment of burn scar contracture. Young patients in particular may require additional skin grafts as they grow, and donor site for skin grafts may be limited. We applied autologous cultured dermal substitutes (CDSs) that are expected to establish a high-quality wound bed to allow thin STSGs. This is the first report of follow-up after application of autologous CDS combined with thin STSG. A male neonate suffered third-degree burns (20% of the total body surface area) on the back. After 2 years, scar contracture of the gluteal regions were released and autologous CDS were applied. Five days after the treatment, a super thin (4-6/1,000 per inch) skin grafting was performed. After 3 years, scar contracture of the back was released and autologous CDS was applied for 2 weeks. Then a split-thick graft was harvested from the same donor site. Ten years after the last operation, the width of the skin graft on his back has extended from 5-8 cm. The contour of the grafted skin is soft, smooth, and can be pinched. This long-term result shows the autologous CDS can be expected to establish the high-quality wound bed that allows thin STSG.

Isolation of small-sized human epidermal progenitor/stem cells by Gravity Assisted Cell Sorting (GACS).

BACKGROUND: Small diameter characterizes epidermal progenitor/stem cells. We have developed Gravity Assisted Cell Sorting (GACS) to simply enrich small-sized epidermal progenitor/stem cells. OBJECTIVE: The cells sorted by GACS were characterized by fluorescence-activated cell sorting analysis, and cultured for up to 7 weeks. The cultured cells were then used for reconstruction of skin equivalent. METHODS: GACS was performed on primary cultures (primary cell) and passage 6-7 cultures (cultured cell) of keratinocytes. A keratinocyte suspension was sized into two groups: cells trapped by a 20 microm filter (trapped cells), and cells flowing through both a 20 and 11 microm filter (non-trapped cells). RESULTS: In the primary cell groups, viability of the trapped cells was 62.5+/-7.2% compared to 77.0+/-3.7% for the non-trapped cells. In the cultured cell groups, viability of the trapped cells was 64.3+/-14.9%, compared to the non-trapped cells (93.1+/-2.0%). Flow cytometric analysis showed better discrimination by cell size between trapped and non-trapped cells in culture than in the primary cell suspension. Non-trapped cells contained a larger number of cells with high levels of alpha6 integrin and low levels of CD71 (alpha6 integrin(bri)CD71(dim)), indicating an enriched progenitor/stem cell population. The difference in these markers between the non-trapped and trapped cells was seen in both the primary and cultured cell groups although this difference was more distinct in cultured cells. Culture of both groups showed that cultures originating from the trapped cells senesced after approximately 15 days while the non-trapped keratinocytes grew for up to 40 days. Manufacture of an epidermis/dermal device (artificial skin) showed that non-trapped cells formed a significantly thicker epithelial layer than the trapped cells, demonstrating the enhanced regenerative capability of the smaller diameter, alpha6 integrin(bri)CD71(dim) cells separated by GACS. CONCLUSION: These results indicate that GACS is simple and useful technique to enrich for epidermal progenitor/stem cell populations, and is more efficient when used on cells in culture.

Skin regeneration for children with burn scar contracture using autologous cultured dermal substitutes and superthin auto-skin grafts: preliminary clinical study.

We have evaluated a novel treatment of burn scar contracture in children. This method involves the application of an autologous cultured dermal substitute (CDS), followed by a graft of superthin split-thickness skin. In the first operation, the autologous CDS was applied to the skin defect that had occurred after releasing the scar contracture. In the second operation, a superthin thickness skin graft (4 approximately 6/1000 inches) was applied 5 approximately 12 days after the first operation. The autologous CDS was applied to 10 sites of 5 children. On 8 sites, the skin grafts were contracted to some extent at an early stage. However, these skin grafts were stretched gradually to a range from 60% to 100% of an original size. At 2 sites, the skin grafts had stretched from 110% to 130% of the original size. This strategy may be useful for the treatment of burn scar contracture in children.

Additional distraction osteogenesis after conventional fronto-orbital advancement.

In the conventional fronto-orbital advancement method, there is a limit to advancement because of scalp skin tension and an absence of a supraorbital bar fixating point. In a case of insufficient advancement after the primary operation, a secondary re-advancement must be performed. In such a condition, additional fronto-orbital advancement by distraction osteogenesis has proved to be very useful. The authors used additional distraction osteogenesis in three infant cases: two of nonsyndromic craniosynostosis and one of Apert's syndrome. They were able to perform these operations safely using their original internal devices. Distraction was started 3 days after the operation. The rate of advancement was 0.5 to 1.0 mm per day. The distraction distances ranged from 16 to 22 mm. They were able to gain enough advancement in all three cases. A reoperation of a fronto-orbital advancement is more difficult than the primary operation because of possible infection, much loss of blood, low blood supply to advanced bones, a tendency of advanced bones to relapse, increased scalp skin tension, and the existence of bone defects. In these poor conditions, distraction osteogenesis has many advantages: good vascularization, no relapsing, a low infection rate, and no need for bony fixating points in the bone defects. Although it is necessary to have a secondary operation to remove the devices and prolonged hospitalization is required, the disadvantages are far outweighed by the many advantages when performing additional fronto-orbital advancement.