This article reports a pedigree with two previously deceased neonates. Both neonates did not experience asphyxia but passed away on their 5th and 13th day of life. The chromosomal analysis of the parents' karyotype revealed no abnormalities. Clinical manifestations of the two deceased cases and relevant medical records were recollected. Whole exome sequencing was conducted on the stem blood sample of Neonate 2, revealing a c.729_730insTT homozygous mutation (p.D244Lfs*39) in the methylmalonyl-CoA mutase gene (NM_000255). It was confirmed that Neonate 2 was affected with methylmalonic acidemia. Amniocentesis was performed at 20 +3 weeks in the current pregnancy. Sanger sequencing of amniotic fluid indicated that the fetus carried the same gene mutation as Neonate 2. Consequently, the fetus was expected to be a patient with methylmalonic acidemia and to exhibit the same phenotype as Neonate 2. Termination of pregnancy, therefore, was selected at 24 weeks of gestation.

Objective:To investigate the clinical outcomes of homodigital reversed dorsal digital artery island flap innervated by the dorsal digital nerve to repair degloving injury of distal thumb.Methods:From July 2016 to June 2019, a total of 15 cases (15 thumbs) with degloving injury of distal part were admitted to the Department of Hand Surgery, the Second Hospital of Tangshan. Nine males and six females were involved, with an average age of 49 years (range, 41 to 69 years). There were twist injury in eight cases and crush injury in seven cases, with four cases of distal phalanx fracture. The defect dimensions after debridement ranged from 3.5 cm×1.8 cm to 4.6 cm×2.4 cm, and the dimension of the flaps ranged from 3.8 cm×2.1 cm to 5.0 cm×2.7 cm. All defects were repaired using homodigital reversed dorsal digital artery island flap innervated by the dorsal digital nerve. The survival, appearance and sensory recovery of the flaps and function of the injured fingers were observed at the follow-up after operation.Results:All the flaps survived without wound infection and blood supply disorder. The follow-up times ranged from 9 to 22 months (mean, 16 months). There was satisfactory appearance of the flaps with similar color and texture to the surrounding tissue. Fracture healing ranged from 4 to 6 weeks. At final follow-up, the values of static 2-PD test of the flaps ranged from 5 to 10 mm (mean, 7.8 mm). The results of range of motion of injured thumb joints were excellent in nine cases and good in five cases. There was slight linear scar left at the donor area of dorsal thumb.Conclusions:The innervated reversed dorsal digital artery island flap has a simple procedure and minimal donor-site cost, which is especially suitable for elderly patients who refuse to free toe transfers.

Objective:To investigate the effects of the dorsal branch of digital artery pedicled flap combined with V-Y advancement flap for repair of degloving injury of fingertip and reverse dorsal metacarpal recurrent artery pedicled island flap for relaying repair of the soft tissue defects in the donor sites of the proximal dorsum.Methods:A total of 21 patients with degloving injuries of fingertips at the 2nd to 5th fingers were hospitalized in the Department of Hand Surgery of the Second Hospital of Tangshan from June 2016 to January 2019, including 14 males and 7 females aged 24-60 years. The retrospective clinical follow-up study was conducted. The areas of wounds after debridement ranged from 2.0 cm×1.5 cm to 3.5 cm×2.2 cm. The dorsal branch of digital artery pedicled flaps with dorsal branch of the proper digital nerve and dorsal digital nerve were designed in the proximal dorsum of the affected fingers to repair dorsal wounds in the distal dorsum of the affected fingers, and the sizes of the flaps ranged from 1.6 cm×1.5 cm to 2.6 cm×2.4 cm. The V-Y advancement flaps in the palmar side of the affected fingers were designed to repair palmar wounds in the distal segment of the affected fingers, and the sizes of the flaps ranged from 0.8 cm×0.6 cm to 2.0 cm×1.5 cm. The reverse dorsal metacarpal recurrent artery pedicled island flaps were used to repair the soft tissue defects in the donor sites of proximal dorsum, the sizes of the flaps ranged from 1.8 cm×1.7 cm to 2.8 cm×2.6 cm, and the donor sites of the flaps in back of hand were sutured directly. The survivals after the operation and the blood supply and appearance during follow-up of the three flaps were observed. At the final follow-up, the static two-point discrimination distance of the three flaps was measured, the satisfaction degree of patients for the appearance of hand was evaluated based on Michigan Hand Function Questionnaire, and the total active range of motion of the injured finger joints was assessed by the trial standard for the evaluation of the functions of the upper limbs of the Hand Surgery Society of the Chinese Medical Association.Results:All the flaps survived after operation. Tension blisters appeared on the surface of one dorsal branch of digital artery pedicled flap, and the wound healed after removing the stitch at the pedicle and changing dressings. During follow-up of 6-20 months, with an average of 12 months, the three kinds of flaps had good appearance, soft texture, and similar color with surrounding tissue, and with only linear scars in donor sites of the dorsal hand. At the final follow-up, the static two-point discrimination distances of V-Y advancement flaps, dorsal branch of digital artery pedicled flaps, and reverse dorsal metacarpal recurrent artery pedicled island flaps were 4-7 mm, 5-10 mm, and 8-15 mm, respectively. Sixteen patients were strongly satisfied with the appearance of hand, and the remaining five patients were satisfied with the appearance of hand. The total active range of motion of the injured finger joints was evaluated as excellent in 17 cases, good in 4 cases.Conclusions:The operation is simple and reliable for dorsal branch of digital artery pedicled flap combined with V-Y advancement flap to repair the degloving injury of fingertip, and reverse dorsal metacarpal recurrent artery pedicled island flaps to repair the soft tissue defects in the donor sites of the proximal dorsum, and the appearance and function of the affected fingers recover well, with minimal injury.

Objective:To investigate the clinical effects of ulnar artery perforator chain flaps in repairing wounds on distal forearm or wrist with vascular anastomosis.Methods:The retrospective observational study method was used. From March 2015 to July 2019, a total of 11 serious trauma patients on distal forearm or wrist with vascular injury were admitted to the Second Hospital of Tangshan, including 8 male patients and 3 female patients, aged from 25 to 62 years, with an average age of 45 years. Vascular anastomosis and tendon repair were performed in all patients, and fracture reduction and fixation were conducted in 9 patients, of which 8 patients underwent external fixation of radius. The residual wounds were located in palmar distal forearm in 5 patients, palmar wrist in 4 patients, dorsal distal forearm in 1 patient, and dorsal wrist in 1 patient, with wound sizes ranged from 4.5 cm×3.0 cm to 10.0 cm×6.0 cm after the last debridement. The wounds were repaired with ulnar artery perforator chain flaps, with the flap sizes ranged from 5.2 cm×3.5 cm to 11.0 cm×7.0 cm. The wound in flap donor site of 1 patient was sutured directly, the wounds in flap donor sites of the other 10 patients were repaired with free skin grafts from ipsilateral thigh after being sutured partially, and the sizes of free skin grafts ranged from 4.0 cm×2.0 cm to 8.5 cm×5.0 cm. The survivals of flaps, skin grafts, and injured limbs after operation were observed. The appearances of the flaps and donor sites of flaps were observed during follow-up. At the final follow-up, the static two-point discrimination distances of the flaps were measured, and the satisfaction degrees of patients for the appearances of injured limbs were evaluated based on Michigan Hand Function Questionnaire.Results:All flaps, skin grafts, and injured limbs survived after operation, without wound infection and blister formation. All patients were followed up for 8 to 26 months, the appearances of the flaps were good and not bloated, with similar color, texture, and thickness to the surrounding skin. The donor sites of flaps repaired with skin grafts were smooth, with circle scar at the edges. At the final follow-up, the static two-point discrimination distances of the flaps was 10-15 mm; 7 patients were strongly satisfied with the appearances of the injured limbs, and the remaining 4 patients were satisfied with the appearances of flaps.Conclusions:The ulnar artery perforator chain flap has constant vascular anatomy and reliable blood supply, with simple operation, which provides a good treatment method for repairing wounds on distal forearm or wrist with vascular anastomosis. It is especially suitable for the patients with radius fracture fixed by external fixator.

Objective:To investigate the clinical effects of neurocutaneous vascular flap innervated by terminal branch of lateral antebrachial cutaneous nerve in repairing finger tip or finger pulp wounds of the thumb.Methods:A retrospective observational study was conducted. From February 2016 to April 2019, a total of 21 patients (21 fingers) with finger tip or finger pulp wounds of the thumbs met the inclusion criteria and were admitted to the Second Hospital of Tangshan, including 13 males and 8 females, aged 21 to 65 years, with the defects located at the finger tips of 14 patients and the finger pulps of 7 patients, and all having exposed bones and/or tendons. The wound sizes of patients in this group after debridement ranged from 2.0 cm×1.5 cm to 4.0 cm×2.7 cm. All the wounds were repaired with the neurocutaneous vascular flap innervated by terminal branch of lateral antebrachial cutaneous nerve, with the flap sizes ranging from 2.3 cm×1.8 cm to 4.3 cm×3.0 cm. In surgery, the terminal branch of lateral antebrachial cutaneous nerve carried by the flaps was anastomosed with the end of the proper digital nerve of the wounds. The donor sites of flaps were sutured directly. The survival of flaps and healing of wounds in donor sites after operation were observed. The appearance of flaps and donor sites were observed during follow-up. At the final follow-up, the static two-point discrimination distances of the flaps were measured, and the degree of satisfaction of patients for the appearances of injured hands were evaluated based on Michigan Hand Function Questionnaire. The total action motion (TAM) of the injured and contralateral thumbs and the angle of thumb web of the injured and contralateral hands were measured. Data were statistically analyzed with paired sample t test. Results:All the flaps of the 21 patients survived with good blood supply and no infection. The wounds at the donor sites were all healed. All the patients were followed up, with the time ranging from 8 to 22 months. The appearances of flaps were good with their color and texture similar to the surrounding tissue. There was no pain in the finger tip or finger pulp, nor any ectopic sensation in flaps. There was only some linear scar left at the radial side of thumb. At the final follow-up, the static two-point discrimination distances of the flaps of the patients were 6 to 11 mm; 18 patients were very satisfied and 3 patients were satisfied with the overall appearance of the injured hand. The TAM of injured thumbs and the angle of thumb web of the injured hands of the patients were respectively (140±5)o and (94±9)°, which were similar to (141±5)o of the thumbs and (95±9)° of hands in the contralateral side, respectively ( t=-2.024, -1.142, P>0.05). Conclusions:The neurocutaneous vascular flap innervated by terminal branch of lateral antebrachial cutaneous nerve has constant anatomy and is easy to perform. It can repair the finger tip or finger pulp wounds of the thumb achieving good appearance and function recovery. It provides a good option for repair of finger tip or finger pulp wounds of the thumb and is especially suitable for emergency application.

Objective:To investigate the effects of the dorsal branch of digital artery pedicled flap combined with V-Y advancement flap for repair of degloving injury of fingertip and reverse dorsal metacarpal recurrent artery pedicled island flap for relaying repair of the soft tissue defects in the donor sites of the proximal dorsum.Methods:A total of 21 patients with degloving injury of fingertip at the 2nd to 5th finger were hospitalized in the Department of Hand Surgery of the Second Hospital of Tangshan from June 2016 to January 2019, including 14 males and 7 females with ages ranging from 24 to 60 years. The retrospective clinical follow-up study was conducted. The areas of wounds after debridement ranged from 2.0 cm×1.5 cm to 3.5 cm×2.2 cm. The dorsal branch of digital artery pedicled flaps with dorsal branch of the proper digital nerve and dorsal digital nerve were designed in the proximal dorsum of the affected fingers to repair dorsal wounds in the distal dorsum of the affected fingers, and the sizes of the flaps ranged from 1.6 cm×1.5 cm to 2.6 cm×2.4 cm. The V-Y advancement flaps in the palmar side of the affected fingers were designed to repair palmar wounds in the distal segment of the affected fingers, and the sizes of the flaps ranged from 0.8 cm×0.6 cm to 2.0 cm×1.5 cm. The reverse dorsal metacarpal recurrent artery pedicled island flaps were used to repair the soft tissue defects in the donor sites of proximal dorsum, the sizes of the flaps ranged from 1.8 cm×1.7 cm to 2.8 cm×2.6 cm, and the donor sites of the flaps in back of hand were sutured directly. The survivals after the operation and the blood supply and appearance during follow-up of the three flaps were observed. At the final follow-up, the static two-point discrimination distance of the flaps was measured, the satisfaction degree of patients for the appearance of hand was evaluated based on Michigan Hand Function Questionnaire, and the total active range of motion of the injured fingers joint was assessed by the trial standard for the evaluation of the functions of the upper limbs of the Hand Surgery Society of the Chinese Medical Association.Results:All flaps survived after operation. Tension blisters appeared on surface of one dorsal branch of digital artery pedicled flap, and the wound healed after removing the stitch at the pedicle and changing dressings. During follow-up of 6-20 months, with an average of 12 months, three kinds of flaps had good appearance, soft texture, and similar color with surrounding tissue, and there was only linear scars in donor site of the dorsal hand. At the final follow-up, the static two-point discrimination distances of V-Y advancement flaps, dorsal branch of digital artery pedicled flaps, and reverse dorsal metacarpal recurrent artery pedicled island flaps were 4-7 mm, 5-10 mm, and 8-15 mm, respectively. Sixteen patients were strongly satisfied and the remaining five patients were satisfied with the appearance of hand. The total active range of motion of the injured fingers joint was evaluated as excellent in 17 cases, good in 4 cases.Conclusions:The operation is simple and reliable for dorsal branch of digital artery pedicled flap combined with V-Y advancement flap to repair the degloving injury of fingertip, and reverse dorsal metacarpal recurrent artery pedicled island flaps to repair the soft tissue defects in the donor sites of the proximal dorsum, and the appearance and function of the affected fingers recover well, with minimal subsidiary-injury.

Objective:To investigate the effects of the dorsal branch of digital artery pedicled flap combined with V-Y advancement flap for repair of degloving injury of fingertip and reverse dorsal metacarpal recurrent artery pedicled island flap for relaying repair of the soft tissue defects in the donor sites of the proximal dorsum.Methods:A total of 21 patients with degloving injury of fingertip at the 2nd to 5th finger were hospitalized in the Department of Hand Surgery of the Second Hospital of Tangshan from June 2016 to January 2019, including 14 males and 7 females with ages ranging from 24 to 60 years. The retrospective clinical follow-up study was conducted. The areas of wounds after debridement ranged from 2.0 cm×1.5 cm to 3.5 cm×2.2 cm. The dorsal branch of digital artery pedicled flaps with dorsal branch of the proper digital nerve and dorsal digital nerve were designed in the proximal dorsum of the affected fingers to repair dorsal wounds in the distal dorsum of the affected fingers, and the sizes of the flaps ranged from 1.6 cm×1.5 cm to 2.6 cm×2.4 cm. The V-Y advancement flaps in the palmar side of the affected fingers were designed to repair palmar wounds in the distal segment of the affected fingers, and the sizes of the flaps ranged from 0.8 cm×0.6 cm to 2.0 cm×1.5 cm. The reverse dorsal metacarpal recurrent artery pedicled island flaps were used to repair the soft tissue defects in the donor sites of proximal dorsum, the sizes of the flaps ranged from 1.8 cm×1.7 cm to 2.8 cm×2.6 cm, and the donor sites of the flaps in back of hand were sutured directly. The survivals after the operation and the blood supply and appearance during follow-up of the three flaps were observed. At the final follow-up, the static two-point discrimination distance of the flaps was measured, the satisfaction degree of patients for the appearance of hand was evaluated based on Michigan Hand Function Questionnaire, and the total active range of motion of the injured fingers joint was assessed by the trial standard for the evaluation of the functions of the upper limbs of the Hand Surgery Society of the Chinese Medical Association.Results:All flaps survived after operation. Tension blisters appeared on surface of one dorsal branch of digital artery pedicled flap, and the wound healed after removing the stitch at the pedicle and changing dressings. During follow-up of 6-20 months, with an average of 12 months, three kinds of flaps had good appearance, soft texture, and similar color with surrounding tissue, and there was only linear scars in donor site of the dorsal hand. At the final follow-up, the static two-point discrimination distances of V-Y advancement flaps, dorsal branch of digital artery pedicled flaps, and reverse dorsal metacarpal recurrent artery pedicled island flaps were 4-7 mm, 5-10 mm, and 8-15 mm, respectively. Sixteen patients were strongly satisfied and the remaining five patients were satisfied with the appearance of hand. The total active range of motion of the injured fingers joint was evaluated as excellent in 17 cases, good in 4 cases.Conclusions:The operation is simple and reliable for dorsal branch of digital artery pedicled flap combined with V-Y advancement flap to repair the degloving injury of fingertip, and reverse dorsal metacarpal recurrent artery pedicled island flaps to repair the soft tissue defects in the donor sites of the proximal dorsum, and the appearance and function of the affected fingers recover well, with minimal subsidiary-injury.

Objective:To explore the effect of venous anastomosis of the pedicled digital artery dorsal branch island flap in repairing fingertip or pulp defects of the same finger.Methods:From February 2016 to September 2018, a total of 56 emergency cases (67 fingers) with fingertip or pulp defects in 2-5 fingers were admitted to the Second Hospital of Tangshan (hereinafter referred to as the author′s affiliation), and the prospective research was conducted. The patients were divided into venous anastomosis group of 29 cases (35 fingers) and non-venous anastomosis group of 27 cases (32 fingers) by drawing lots. There were 18 males and 11 females in venous anastomosis group, aged 17 to 62 years, with wound area of 1.6 cm×1.3 cm-3.1 cm×2.4 cm after debridement. There were 17 males and 10 females in non-venous anastomosis group, aged 20 to 59 years, with wound area of 1.7 cm×1.2 cm-3.0 cm×2.4 cm after debridement. According to the location and size of the fingertip or pulp defect of patients in the two groups, the flap was designed on the dorsum of the middle or proximal phalanx of the injured finger. During the operation, the epineurium of dorsal branch of the proper digital nerve or the dorsal digital nerve carried by the flap was anastomosed end-to-end with the stump of proper digital nerve in the wound. One or two superficial vein (s) carried by the flap was/were interrupted end-to-end anastomosed with the superficial veins on the dorsum or palm of the wound surface in venous anastomosis group, and the venous anastomosis was not performed in non-venous anastomosis group. The flap size resected ranged from 1.8 cm×1.5 cm to 3.4 cm×2.6 cm in venous anastomosis group, and that ranged from 1.9 cm×1.4 cm to 3.3 cm×2.6 cm in non-venous anastomosis group. The donor site wounds in the two groups were resurfaced by free full-thickness skin graft harvested from the proximal forearm or the medial side of the upper arm. The blood circulation of the flap of patients in 2 groups was observed after operation. During follow-up after operation, the patients′ satisfaction for the appearance of the flap was evaluated by Michigan Hand Function Questionnaire, the flap color was observed by the chief physician of the Department of Hand Surgery in the author′s affiliation, and the incidence of flap pigmentation was calculated. Data were statistically analyzed with t test and chi-square test. Results:All the flaps of patients survived without tension blister in venous anastomosis group after operation. Tension blisters occurred in 6 cases (6 fingers) in non-venous anastomosis group due to venous reflux obstruction, and the flaps survived after removing some sutures of the pedicle and changing dressing. During follow-up of 8-20 months, with an average of 15 months, the patients′ satisfaction score for flap appearance in venous anastomosis group was (4.6±0.5) points, which was obviously higher than (4.3±0.6) points of non-venous anastomosis group ( t=2.482, P<0.05). The incidence of flap pigmentation in venous anastomosis group was 9% (3/35), which was significantly lower than 31% (10/32) of non-venous anastomosis group ( χ2=5.498, P<0.05). Conclusions:The pedicled digital artery dorsal branch island flap with venous anastomosis repairs the same fingertip or pulp defects, resulting in unobstructed venous reflux, low incidence of flap pigmentation, good appearance and high patient satisfaction.

Objective: To investigate the clinical outcomes of snuffbox perforator flap of radial artery to repair large soft tissue defects of the thenar and dorsal thumb. Methods: From June 2012 to March 2017, a total of 18 cases with large soft tissue defects of the thenar and dorsal thumb were hospitalized in the Second Hospital of Tangshan. There were 13 males and 5 females, with an average age of 39 years (range, 17 to 64 years). The right hand was involved in 14 cases and the left in 4 cases. There were 5 cases of thenar defect, 7 cases of dorsal thumb defect and 6 cases of thenar combined with dorsal thumb defect. The tendon and / or bone were exposed in all 18 cases. The size of the wounds after debridement ranged from 2.5 cm × 3.5 cm to 4.5 cm × 6.0 cm. The snuffbox perforator flap was designed to repair the defect based on the pivot point of snuff box and the axis line from styloid process of radius to capitulum of radius. Four cases with extensor tendon defects of the thumbs were grafted with a section of brachial radial tendon. The dimension of the flaps ranged from 2.8 cm × 3.8 cm to 5.0 cm × 6.8 cm. The observation indexes included blood supply and appearance of the flap, the total activity of flexion and extension (TAM) of the injured thumb, the angle of the first web span and the satisfaction of the patients. Results: All flaps survived uneventfully. The donor area of forearm was directly closed in 16 cases, and the remaining wound in 2 cases was repaired by free skin graft. All the cases were followed up for 8 to 16 months (mean, 12 months). The good appearance of the flaps with soft texture was obtained. The average values of TAM of the injured thumbs and the first webs were 139°and 96°, respectively. They were excellent in 15 cases and good in three cases for TAM, and excellent in all cases for the first webs. 10 patients were very satisfactory with flap appearance and thumb function and the rest was satisfactory. Conclusions The snuffbox perforator flap of radial artery provides a reliable and simple technique with constant blood supply and minimal donor-site cost, which could be an ideal option for repairing large soft tissue defects of the thenar and dorsal thumb with extensor tendon defects of the thumbs.

Objective To investigate the clinical effectiveness of the reversed flaps pedicled with lateral vas-cular chain of dorsal branch of digital artery and perforating branch of the dorsal metacarpal artery for relaying repair of soft tissue defects at middle-distal part of the 2nd ～ 5th finger and donor site. Methods From June, 2014 to May, 2016, a total of 18 cases (11 male and 7 female) with soft tissue defects at middle-distal part of 2nd～5th fin-ger were treated with the reversed flap pedicled with lateral vascular chain of dorsal branch of digital artery. The donor site was repaired with the reversed flap pedicled with the perforating branch of the dorsal metacarpal artery. The aver-age age at the time of reconstruction was 41 years. The right hand was involved in 12 cases and the left in 6 cases. The etiologies of injury were avulsion (n=5) , crush (n=6), and saw-cut injury (n=7). There were 15 cases with soft tissue defect at the distal phalanx and 3 cases with loss of distal and middle phalanx. All these cases were exposed with bone or tendon. The defect size ranged from 1.0 cm × 1.8 cm to 2.0 cm × 3.0 cm. And the dimension of the re-versed flaps pedicled with lateral vascular chain of dorsal branch of digital artery and perforating branch of the dorsal metacarpal artery ranged from 1.2 cm × 2.0 cm to 2.3 cm × 3.3 cm, and 1.4 cm × 2.3 cm to 2.6 cm × 3.6 cm, respec-tively. Postoperative follow-up was done termly. Results All flaps survived uneventfully with primary healing at donor site. The mean of followed-up time was 11 months (ranged from 6 to 15 months). The appearance and texture of the flaps were satisfactory and similar to the surrounding tissue. There was only some linear scar left at the dorsum of the palm. On the basis of the MHQ, 16 patients were strongly satisfied (score 5) with the appearance of the flap, and the remaining 2 patients was satisfied (score 4) with the appearance. The static 2-point discrimination of the flaps at the middle-distal part of 2nd～5th finger was 5-9 mm. Based on TAM assessment, the function of the injured finger was graded as excellent in 15 cases, good in 3 cases. Conclusion The relaying reversed flaps pedicled with lat-eral vascular chain of dorsal branch of digital artery and perforating branch of the dorsal metacarpal artery can be applied for repair of soft tissue defects at middle-distal part of 2nd～5th finger and donor site with easy procedure, reliable blood supply, satisfactory results, good appearance as well as less complications at donor sites.