Dermal substitution with Matriderm in burns on the dorsum of the hand

Background

Dermal substitutes are used increasingly in deep partial and full-thickness burn wounds in order to enhance elasticity and pliability. In particular, the dorsum of the hand is an area requiring extraordinary mobility for full range of motion. The aim of this comparative study was to evaluate intra-individual outcomes among patients with full-thickness burns of the dorsum of both hands. One hand was treated with split-thickness skin grafts (STSG) alone, and the other with the dermal substitute Matriderm^® and split-thickness skin grafts.

Material and Methods

In this study 36 burn wounds of the complete dorsum of both hands in 18 patients with severe burns (age 45.1 ± 17.4 years, 43.8 ± 11.8% TBSA) were treated with the simultaneous application of Matriderm^®, a bovine based collagen I, III, V and elastin-hydrolysate based dermal substitute, and split-thickness skin grafting (STSG) in the form of sheets on one hand, and STSG in the form of sheets alone on the other hand. The study was designed as a prospective comparative study. Using both objective and subjective assessments, data were collected at one week and 6 months after surgery. The following parameters were included: After one week all wounds were assessed for autograft survival. Skin quality was measured 6 months postoperatively using the Vancouver Burn Skin Score (VBSS). Range of motion was measured by Finger-Tip-Palmar-Crease-Distance (FPD) and Finger-Nail-Table-Distance (FNTD).

Results

Autograft survival was not altered by simultaneous application of the dermal matrix (p > 0.05). The VBSS demonstrated a significant increase in skin quality in the group with dermal substitutes (p = 0.02) compared to the control group with non-substituted wounds. Range of motion was significantly improved in the group treated with the dermal substitute (p = 0.04).

Conclusion

From our results it can be concluded that simultaneous use of Matriderm^® and STSG is safe and feasible, leading to significantly better results in respect to skin quality of the dorsum of the hand and range of motion of the fingers. Skin elasticity was significantly improved by the collagen/elastin dermal substitute in combination with sheet-autografts.     

Prospective study on burns treated with Integra,a cellulose sponge and split thickness skin graft: Comparative clinical and histological study—Randomized controlled trial

Background

The aim of this study was to compare three different methods to cover excised burn wounds in a randomized controlled trial.

Methods

Fascially excised burn wounds, measuring 10 cm × 5 cm, were covered with Integra^®, split thickness skin graft (STSG), and a viscose cellulose sponge Cellonex™ in each of ten adult patients. Integra^® and Cellonex™ treated areas were covered with thin STSG on day 14. Biopsies were taken 3, 7, 14, and 21 days, 3 months, and 12 months after surgery, and samples were subjected to a range of immunohistochemical stains, in addition to hematoxylin and eosin (HE). Scar assessment was performed 3 and 12 months post-operatively with the Vancouver Scar Scale (VSS).

Results

Inflammation was not substantial in any of the study areas, but Cellonex™ had the most neutrophils, histiocytes, and lymphocytes with significant differences on days 7 and 14. Complete vascularization of Integra^® seemed to occur later compared to the other materials. STSG had the most myofibroblasts on day 14 (p = 0.012). In VSS the quality of the scar improved in all materials from 3 to 12 months.

Conclusions

The final results for all treatments after 12 months demonstrate equal clinical appearance, as well as histological and immunohistochemical findings.     

Objective evaluation of the effect of autologous platelet concentrate on post-operative scarring in deep burns

Introduction

The healing of grafted areas after surgical treatment of deep burns frequently generates mutilating scars, and rises the risk of subsequent scar hypertrophy. Scar assessment based on clinical evaluation is inherently subjective, which stimulates search for objective means of evaluation.

Objective

The aim of this study was to objectively evaluate the effect of using autologous platelet concentrate (APC) in combination with split thickness skin grafting (STSG) on scarring processes following surgery of deep burns as compared with application of STSG alone.

Method

Selected viscoelastic properties of 38 scars on 23 patients in total were examined using the Cutometer MPA 580 under controlled conditions for long-term outcomes 1, 3, 6 and 12 months after surgery following deep burns.

Results

The findings of this study suggest that the STSG + APC combination reduces the time of scar viscoelastic properties recovery as compared with application of STSG alone. This was statistically significant for viscoelastic parameters R₂ and Q₁.

Conclusion

APC has been advocated to enhance scarring after surgery of deep dermal and full thickness burns. We objectively demonstrated that the viscoelastic properties of scars treated with STSG + APC combination return more rapidly to the plateau state than areas treated with STSG only.     

Staged management of the open abdomen and enteroatmospheric fistulae using split-thickness skin grafts

Background

Management of the open abdomen with polyglactin 910 mesh followed by split-thickness skin grafts allows safe, early closure of abdominal wounds. This technique can be modified to manage enteroatmospheric fistulae. Staged ventral hernia is performed in a less inflamed surgical field.

Methods

A retrospective review was performed of 59 consecutive patients who underwent abdominal skin grafting for open abdominal wounds from 2001 to 2011.

Results

The median length of follow-up was 215 days. Thirty-one percent of patients presented with preexisting enteroatmospheric fistulae, and 41% required polyglactin 910 mesh placement before skin grafting. Partial or complete skin graft failure occurred in 7 patients. Four patients required repeat skin grafting. All patients ultimately achieved abdominal wound closure, and none developed de novo fistulae.

Conclusions

With proper technique, skin grafting of the open abdomen with a planned ventral hernia repair is a safe and effective alternative to delayed primary closure.     

The use of Suprathel(®) in deep dermal burns: first results of a prospective study

Introduction

While autologous skin grafting has been the standard for coverage of full-thickness areas, several options for deep-partial-thickness defects exist. With regard to economising donor sites, we compared a copolymer based on dl-lactid acid (Suprathel^®) as temporary wound dressing with autologous skin, and analysed time to healing and scar quality in matched areas of deep-partial-thickness burn.

Methods

We recruited 18 patients with a median age of 45 years (range: 25–83 years), for this prospective, non-blinded controlled non-inferiority study, suffering from deep-partial-thickness burns from November 2009 to July 2010. After early tangential excision, matched deep-partial-thickness areas were covered with 1:1.5 meshed autologous skin grafts and the copolymer for direct intra-individual comparison. Scars were evaluated by means of the Vancouver Scar Scale (VSS), the Patient and Observer Scar Assessment Scale (POSAS) and suction cutometry (MPA 580, Courage and Khazaka Electronic GmbH, Cologne, Germany) on days 30 and 90, postoperatively.

Results

Fifteen days after surgery, complete wound closure was present in 44.4% (8/18) of all areas covered with copolymer and 88.9% (16/18) in the split-thickness skin graft (STSG) area (p = 0.008). Evaluation of the total VSS, POSAS and cutometry satisfied the criterion of non-inferiority for Suprathel^® on day 30. Ninety days after surgery, only the Observer Scar Scale showed that Suprathel is non-inferior to STSG, albeit the mean total VSS and Patient Scar Scale were better in Suprathel^® areas.

Conclusion

Suprathel^® represents a solid, reliable epidermal skin substitute with longer healing times in comparison to skin grafts but comparable results concerning early scar formation. Suprathel^® can serve as a tool in treatment portfolio for adult patients suffering from deep dermal burns. Especially in patients with extensive burns, Suprathel^® can be used to cover the deep dermal burn wounds to save STSGs and its donor sites for the coverage of full-thickness burned areas.     

Banking and use of glycerol preserved full-thickness skin allograft harvested from body contouring procedures

Introduction

The use of glycerol preserved skin allograft (GPA) became a main stay in burn treatment. However, harvesting of cadaveric skin is not yet legalized in many countries including Egypt.

Objectives

To estimate the feasibility of using skin harvested from body contouring procedures as a source of GPA and its clinical efficacy.

Patients and methods

Skin harvested from body contouring procedures done in Al-Azhar university hospitals was preserved by glycerolization and used in management of burn and complicated wounds.

Results

In the period between February 2012 and February 2013 skin was harvested from 24 abdomenoplasty cases, 6 bilateral breast reduction cases, and 1 case of thigh lift done in Al-Azhar university hospitals. This yielded about 22,000 cm² of skin preserved by glycerolization. This GPA was used in 15 excised burn wounds, in 9 cases of chronic burn wounds, and in 6 complicated wounds. Partial graft loss occurred in 3 cases and total graft loss occurred in 1 case.

Conclusion

The glycerolized full-thickness skin harvested from body contouring procedures is clinically effective in burn and wound management. In the presence of regional coordination, it can serve as an abundant source for skin banking in where cadaveric skin use is not legalized.     

Topical negative pressure therapy: Does it accelerate neovascularisation within the dermal regeneration template,Integra? A prospective histological in vivo study

Background

The use of topical negative pressure (TNP) dressings with dermal regeneration template (DRT), Integra, has improved outcomes and simplified aftercare. Previous clinical studies have suggested accelerated vascularisation; with a reduction in the duration of the 1st stage after the application of Integra, from 2 to 4 weeks to as little as 4 days, but with no histological evidence. However, histological studies, without TNP, have shown that vascularisation occurs between the second and the fourth week. This study set out to examine histologically the rate of DRT neovascularisation when combined with TNP.

Methods

Eight patients with nine reconstruction sites were enlisted. Unmeshed Integra and fibrin sealant to promote adherence were used. TNP was applied for the duration between the 1st and the 2nd stages. Patients underwent serial biopsies on days 7, 14, 21 and 28 post-application. The biopsies were stained with H&E and endothelial markers CD31 and CD34. Template vascularisation was assessed as a percentage of the template depth in which patent, canalised vascular channels could be demonstrated.

Results

The median percentage of the template depth which demonstrated canalised channels was 0%, 20%, 61% and 80% for days, 7, 14, 21 and 28, respectively.

Conclusion

The application of TNP dressings to dermal templates can reduce shearing forces, restrict seroma and haematoma formation, simplify wound care and improve patient tolerance. However, this study could not demonstrate that TNP accelerates neovascularisation as verified by the presence of histologically patent vascular channels.     

The use of dermal regeneration template (Pelnac®) in acute full-thickness wound closure: A case series

Background

Dermal regeneration template (DRT) has been well implicated in the reconstruction of full-thickness burn injury. This case series specifically presents our experience and our clinical application of Pelnac® to achieve wound closure with complex acute full-thickness defect.

Methods

A retrospective review of patients treated with Pelnac for complex wound defects from 2008 to 2014 at Concord Burns Unit was carried out. Variables such as wound aetiology, wound size and complications were considered.

Results

Five patients (four females and one male with a mean age 54?±?20) all had full-thickness defects (mean defect size 4.3?±?2.0 % TBSA), some with exposed tendon and bone. The wounds were treated with Pelnac®; the silicone layer was removed at postoperative day 14 and a split-thickness skin graft (0.2 to 0.3 mm) was applied. Clinically, the reconstructed areas demonstrated good granulation tissue at 14 days with good take of the skin graft. There were no major acute graft loss, rejection or associated infection. However, there were small areas of graft loss which did not require re-grafting.

Conclusions

DRT provides a safe and efficacious alternative when dealing with acute contaminated full-thickness wounds. Pelnac® seems versatile in obtaining wound coverage in difficult complex wounds, especially in critically ill patients where free or pedicle flap reconstruction would be problematic.Level of Evidence: Level V, therapeutic study.

     

Vattikuti Institute Prostatectomy: Technical Modifications in 2009

Background

Since we last published our technique of robotic prostatectomy, we have introduced three technical refinements: superveil nerve sparing, bladder drainage with a percutaneous suprapubic tube (PST), and limited node dissection of the obturator and internal iliac nodes in preference to the external iliac nodes in selected patients.

Objective

To describe selection criteria, to explain the three techniques, and to evaluate functional and oncologic results.

Design, setting, and participants

Single-institution study of 1151 radical prostatectomies performed from 2006 to 2008 by one surgeon.

Surgical procedure

The superveil nerve-sparing technique spares nerves from the 11-o’clock position to the 1-o’clock position. The bladder is drained with a PST rather than a urethral catheter. For low- or intermediate-risk disease, limited lymphadenectomy concentrates on the internal iliac and obturator nodes, excluding the external iliac lymph nodes.

Measurements

Erectile function and patient comfort were evaluated using questionnaires administered by a third party. Lymph node yield was quantified by a qualified uropathologist.

Results and limitations

At 6–18 months after surgery, 94% of men who attempted sexual intercourse were successful with a median Sexual Health Inventory For Men (SHIM) score of 18 out of 25. PST bladder drainage resulted in less patient discomfort; visual analog scores were 2 at 2 days after prostatectomy and 0 at 6 days after prostatectomy. The modified lymphadenectomy harvested few overall nodes, but it increased the yield of positive nodes >13-fold in patients with low-risk stratification (6.7% compared with 0.5%).

Conclusion

In this single-institution, single-surgeon study, these modifications improved erectile function outcomes, decreased catheter-associated discomfort, and enhanced the detection of positive nodes.     

Pedicled skin flap of foreskin for phalloplasty in the management of completely concealed penis

Objective

To evaluate the effect of pedicled skin flap of foreskin for phalloplasty in the management of completely concealed penis.

Methods

We retrospectively reviewed 97 consecutive patients with completely concealed penis, which had been surgically corrected between November 2004 and January 2012. All patients were repaired using 2 surgical methods: 18 with Shiraki's operation and 79 with a technique of pedicled foreskin skin flap.

Results

With 1 month to 7 years (mean 18 months) follow-up, the 18 cases treated by Shiraki's operation had satisfactory exposed penile shaft but too much incision and obvious edema. The 79 cases treated by pedicled foreskin skin flap uniformly reported satisfaction with the operative results. They found the surgical repair was successful in appearance and improved hygiene, accessibility, and penile exposure. There was no recurrence to the pre-treatment condition, or any chordee, penile distortion, trapped penis, erectile abnormalities, or voiding complications in any case. The pedicled skin flap was without ischemia or necrosis and yielded no post-operative wound infections, wound separation, or unsightly scarring. Slight edema and swelling of the flap were common, but these issues completely resolved by 3 weeks.

Conclusion

Numerous operative procedures have been described and adopted for the management of completely concealed penis, but the pedicled skin flap phalloplasty can achieve maximum utilization of prepuce to assure coverage of the exposed penile shaft. It has fewer complications, achieving marked aesthetics and often functional improvement. This suggests pedicled foreskin skin flap for phalloplasty is a relatively ideal means for treating completely concealed penis.     

The application of glycerol-preserved skin allograft in the treatment of burn injuries: An analysis based on indications

Introduction

Glycerol-preserved skin allograft (GPA) plays a crucial role in the management of burns. Its indications include wound-bed preparation, definitive dressing and sandwich grafting technique.

Objective

We analysed the experience of using GPA and its efficacy in burn treatment in our burn centre.

Methods

All burns managed with GPA in our burn centre from October 2001 to May 2008 were analysed.

Results

Mean total body surface area (TBSA) of 43 consecutive cases was 28.7%. GPA adhered to the wound for an average of 8.4 days before rejection. The length of hospital stay of the survivors was 42.5 days. The autograft take after wound-bed preparation with GPA was 88.4%. For sandwich grafting technique, the autograft take was 74.4%. When GPA was applied for partial-thickness burn as definitive dressing, all patients achieved complete healing within an average of 19 days without further surgical intervention. Despite colonisation of burn wounds after application of skin allograft, the outcomes of autograft take and wound healing were not significantly different.

Conclusion

The selective and strategic use of the GPA in major burn patients ensures optimal benefits in the management of burns. It is versatile in various categories of burn wounds with minimal morbidity.     

A multicentre,prospective, randomised comparison of the sliding hip screw with the Medoff sliding screw and side plate for unstable intertrochanteric hip fractures

Objective

This study aimed to compare the dynamic hip screw (DHS) and Medoff sliding plate (MSP) for unstable intertrochanteric hip fractures.

Design

A randomised, prospective trial design was used.

Setting

The study was undertaken in two level-1 trauma centres and one community hospital.

Patients/participants

A total of 163 patients with unstable intertrochanteric hip fractures (Orthopaedic Trauma Association (OTA) 31-A2) were randomised to DHS or MSP. Inclusion and exclusion criteria were designed to focus on isolated unstable intertrochanteric hip fractures in ambulatory patients.

Intervention

Randomisation was performed intra-operatively, after placement of a 135° guide wire. Follow-up assessments were performed at regular intervals for a minimum of 6 months.

Main outcome measurements

The primary outcome measure was re-operation rate. The secondary outcome was patient function, evaluated using a validated outcome measure, the Hip Fracture Functional Recovery Score. Tertiary outcomes included: mortality, hospital stay, quality of reduction and malunion rate.

Results

A total of 86 patients were randomised to DHS and 77 to MSP. The groups had similar patient demographics, pre-fracture status and in-hospital course. The quality of reduction was the same for each group, but the operative time was longer in the MSP group (61.6 vs. 50.1 min, P = 0.01). The rate of re-operation was low (3/86 in DHS and 2/77 in MSP) with no statistically significant difference. The functional outcomes were the same for both groups, with functional recovery scores at 6 months of 51.0% in the DHS arm and 49.7% in the MSP arm.

Conclusions

The two techniques produced similar results for the clinically important outcomes of the need for further surgery and functional status of the patients at 6 months’ follow-up.     

Short-Term Result of Renal Transplantation Using Extracorporeal Membrane Oxygenation–Supported Brain-Dead Donors

Background

Extracorporeal membrane oxygenation (ECMO) is now widely used to maintain hemodynamic stability after traumatic events among medical centers. It remains unclear whether renal transplantation using ECMO-supported donors carries poorer outcomes.

Methods

From February 2010 to March 2013, we performed 9 renal transplantations (6 females and 3 males) from 5 ECMO-supported donors. Demographic data and clinical outcomes were retrospectively analyzed through medical chart review.

Results

The mean follow-up period was 15 ± 9 months (range: 8–37). Eight of the 9 grafts remained functioning within the follow-up period. One (11.1%) graft loss was noted after repeated acute rejection. Acute rejection occurred in 3/9 (33%) of cases. Delayed graft function was also observed in 3/9 (33%) of cases.

Conclusion

Renal transplantation using ECMO-supported brain-dead donors was not associated with an unacceptably high rate of graft loss in this short-term follow-up. It might be an alternative way to expand donor pools.     

Management of comminuted but continuous mandible defects after gunshot injuries

Introduction

Firearm injuries continue as a major public health problem, contributing significant morbidity, mortality, and expense to our society. There are four main steps in the management of patients with gunshot wounds to the face: securing an airway, controlling haemorrhage, identifying other injuries and definitive repair of the traumatic facial deformities. The objective of this study was to determine late outcome of two treatment options by open reduction and internal fixation versus closed reduction and maxillomandibular fixation (MMF) in the treatment of gunshot injuries of the mandible.

Methods

Sixty patients of gunshot injury were randomly allocated in two groups. In group A, 30 patients were treated by open reduction and internal fixation and in group B, 30 patients were treated by closed reduction and maxillomandibular fixation. Patients were discharged as the treatment completed and recalled for follow up. Up to 3 months after injury, fortnightly complications like infection, malocclusion, malunion of fractured fragments, facial asymmetry, sequestration of bone and exposed plates were evaluated and the differences between two groups were assessed. The follow-up period ranged from 3 months to 10 months.

Results

Patients treated by open reduction tended to have less complications as compared to closed reduction.

Conclusion

Based on this study open reduction and internal fixation is the best available method for the treatment of gunshot mandible fractures without continuity defect.     

Cutaneous microcirculatory assessment of the burn wound is associated with depth of injury and predicts healing time

Rationale

Current trends for the treatment of deep partial thickness and full-thickness burns include early excision and skin grafting. In this study we retrospectively evaluated the ability of Laser Doppler Flowmetry (LDF), taken within 24 h of the burn to predict: (1) burn wound depth and (2) wounds which would heal in less than 21 days.

Method

The Laser Doppler Flowmeter (O2C, LEA Medizintechnik, Germany) was employed to non-invasively measure the cutaneous microcirculation of 173 selected areas on 28 patients who suffered burns.

Results

A distinct association between initial flow (<24 h after burn injury) and the clinical assessment of depth of burn wounds was observed. Wounds demonstrating an initial blood flow of >100 AU were, in 93.1% of cases, correctly (positively) predicted for spontaneous healing within 21 days. A blood flow of <100 AU (negatively) predicted in 88.2%, those wounds which would not go on to heal within 21 days. Sequential measurement analysis (<24 h, 3 days after injury and 6 days after injury) revealed no significant decrease in skin perfusion velocity or flow rate.

Conclusion

LDF can provide immediate results for early determination of burn wound depth and is useful in selecting patients for conservative treatment of their burn wounds.     

Functional outcomes following syndesmotic fixation: A comparison of screws retained in situ versus routine removal – Is it really necessary?

Introduction

Syndesmotic disruption can occur in up to 20% of ankle fractures and is more common in Weber Type C injuries. Syndesmotic repair aims to restore ankle stability. Routine removal of syndesmosis screws is advocated to avoid implant breakage and adverse functional outcome such as pain and stiffness, but conflicting evidence exists to support this. The aim of the current study is to determine whether functional outcome differs in patients who had syndesmosis screws routinely removed, compared to those who did not, and whether a cost benefit exists if removal of screws is not routinely necessary.

Patients and methods

A retrospective review of consecutive syndesmosis repairs was performed from 1 January 2008 to 31 December 2010 in a single regional trauma centre. We identified 91 patients who had undergone open reduction internal fixation of an ankle fracture with placement of a syndesmosis screw at index procedure. As many as 69 patients were eligible for the study as defined by the inclusion criteria and they completed a validated functional outcome questionnaire.The functional outcomes of patients with ‘retained screws’ and ‘removed screws’ were analysed and compared using the Olerud Molander Ankle Score (OMAS).

Results

A total of 63 patients responded with a mean follow-up period of 31 months (range 10–43 months). Of those patients, 43 underwent routine screw removal whilst 20 had screws left in situ. The groups were comparable considering age, gender and follow-up time. The ‘retained’ group scored higher mean OMAS scores, 81.5 ± 19.3 compared to 75 ± 12.9 in the ‘removed’ group (p = 0.107). The retained group achieved higher functional scores in each of the OMAS domains as well as experiencing less pain. When adjusted for gender, the findings were found to be statistically significant (p = 0.046).

Conclusion

Our study has shown that retained-screw fixation does not significantly impair functional capacity, with additional cost-effectiveness. We therefore advocate that syndesmosis screws be left in situ and should only be removed in case of symptomatic implants beyond 6 months postoperatively.     

Size matters: How accurate is clinical estimation of traumatic wound size?

Aims

The presentation of traumatic wounds is commonplace in the accident & emergency department. Often, these wounds need referral to specialist care, e.g. trauma & orthopaedic, plastic or maxillofacial surgeons. Documentation and communication of the size of the wound can influence management, e.g. Gustilo & Anderson classification of open fractures. Several papers acknowledge the variability in measurement of chronic wounds, but there is no data regarding accuracy of traumatic wound assessment.The authors hypothesised that the estimation of wound size and subsequent communication or documentation was often inaccurate, with high inter-observer variability. A study was designed to assess this hypothesis.

Methods

A total of 7 scaled images of wounds related to trauma were obtained from an Internet search engine. The questionnaire asked 3 questions regarding mechanism of injury, relevant anatomy and proposed treatment, to simulate real patient assessment. One further question addressed the estimation of wound size. 50 doctors of varying experience across several specialities were surveyed. The images were analysed after data collection had finished to provide appropriate measurements, and compared to the questionnaire results by a researcher blinded to the demographics of the individual.

Results

Our results show that there is a high inter-observer variability and inaccuracy in the estimation of wound size. This inaccuracy was directional and affected by gender. Male doctors were more likely to overestimate the size of wounds, whilst their female colleagues were more likely to underestimate size.

Conclusions

The estimation of wound size is a common requirement of clinical practice, and inaccurate interpretation of size may influence surgical management. Assessment using estimation was inaccurate, with high inter-observer variability. Assessment of traumatic wounds that require surgical management should be accurately measured, possibly using photography and ruler measurement.     

Surgery for Graves’ disease: a 25-year perspective

Background

Optimal treatment of Graves’ disease (GD) remains controversial. The authors retrospectively reviewed the surgical cases of GD at a single academic tertiary center.

Methods

Demographic, clinical, and surgical data were analyzed for all patients with GD undergoing thyroidectomy over 25 years, in 3 periods: 1985 to 1993 (n = 32), 1994 to 2002 (n = 91), and 2003 to 2010 (n = 177).

Results

There were 300 patients with GD (85.7% women; mean age, 39.3 years; median length of follow-up, 24.6 months). Overall, perioperative morbidity occurred in 36 patients (12.0%), and there was no mortality. Thyroidectomy-specific morbidity was very low, and the incidental malignancy rate was 10.3%.

Conclusions

Surgical treatment of GD has a very high safety profile, with low perioperative and thyroidectomy-specific morbidity, even in patients with overt hyperthyroidism. Incidental malignancy in patients with GD is not uncommon.     

Fludarabine,Low-Dose Cyclophosphamide and Rabbit Antithymocyte Globulin Allowed Stable Engraftment After Allogeneic Peripheral Blood Stem Cell Transplantation for Poly-Transfused Dyskeratosis Congenita Patient: Case Report

Background

Dyskeratosis congenita (DC) is characterized by the clinical triad of reticular skin pigmentation, oral leukoplakia, and nail dystrophy associated with bone marrow failure (BMF) and an high risk to develop cancer and pulmonary complications. The only curative treatment for patients with DC and BMF is stem cell transplantation. Due to the rarity of the disease, the best transplant procedure is not yet known. The use of myeloablative procedures has been associated with high mortality. In the last 2 decades, encouraging results have been obtained with nonmyeloablative procedures. Heavily transfused patients have an additional risk of graft failure.

Case Report

Herein we have reported a 4-year-old boy with DC and severe BMF at the time of transplantation, who had been transfused with nonleucodepleted blood products for 18 months. He experienced a favorable outcome after nonmyeloablative transplant conditioning using low-dose cyclophosphamide (40 mg/kg), fludarabine (180 mg/kg), and rabbit antithymocyte globulin (10 mg/kg). The patient received a peripheral stem cell graft containing 7.52 × 10⁶ CD34/kg from an HLA identical sister. Graft versus host disease (GVHD) prophylaxis consisted of a short-term combination of cyclosporine and methotrexate.

Results

We observed rapid neutrophil engraftment on day +21 and for platelets on day +40. No early or late complications were recorded during 15 months follow-up. The patient developed only grade I skin GVHD. On day +30, chimerism assay showed 100% donor cells.

Conclusion

Long-term follow-up is essential to establish the efficacy and safety of this procedure.