Limb Defects Research Articles

Congenital hand anomalies and rare syndromes in children

Congenital hand anomalies and associated rare syndromes in children are commonly seen in clinical practice and require complete clinical, radiological and genetic evaluation. The purpose of this case series is to describe four rare syndromic children with varied hand anomalies and differences. Three of the children are female and one is a male. Distal limb defects from simple polydactyly, clinodactyly to cleft hand were identified including preaxial anomalies. Significant clinical problems noted were chiefly cosmetic and altered self-image was also noted in one child. The orofacial clefting noted in the child with ectrodactyly ectodermal dysplasia (EEC) syndrome required repeated surgery though the child had minimal feeding difficulty and significant speech delay. Multidisciplinary management is necessary with the early involvement of clinical geneticist.

Factors limiting the motor rehabilitation of elderly disabled persons with lower limb loss

Background. Amputation of a limb leads to significant changes in a person's life, since mobility restrictions affect the ability to perform daily tasks and professional duties.
 Aim. To identify factors that negatively affect the results of motor rehabilitation of elderly disabled people with lower limb loss.
 Material and methods. Data on patients with postamputation lower limb defects, who were divided into two groups, were analyzed. The first group (comparison group) included patients aged 60 years and younger 171 people, including 141 men and 30 women; the second group (main group) consisted of patients over 60 years of age 260 people, including 207 men and 53 women. Patients of the second group with a satisfactory result of rehabilitation were included in subgroup 2A, with an unsatisfactory result in subgroup 2B. The influence of gender, age, level of mobility, cognitive abilities, daily activity, level of amputation, presence of concomitant diseases, body mass index, duration of the post-amputation period on the result of rehabilitation in the second group were assessed. Statistical analysis used the 2 test, the MannWhitney U test for independent groups, and binary logistic regression.
 Results. The indicators of mobility before prosthetics in elderly people with disabilities did not differ significantly from those in people under the age of 60, however, by the end of the rehabilitation course, the optimal level of mobility in the room (without using a wheelchair) was reached by 75.4% of the comparison group and only by 52.7% of the main group (ꭓ2=22.59, р 0.001). Further analysis showed that at the age of over 66.5 years, the risk of obtaining an unsatisfactory result of motor rehabilitation is 2.3 times higher (p=0.006) than at the age of 6066.5 years. In older women, this risk is 2.7 times higher than in men of the same age (p=0.014). With a body mass index of more than 28.5 kg/m2, the risk increases by 3.2 times (p=0.001), and with a duration of the post-amputation period at the time of primary prosthetics of more than 1 year, it increases by 2.6 times (p=0.008) compared with lower values of these indicators in elderly people with disabilities.
 Conclusion. The factors associated with the unsatisfactory result of motor rehabilitation of elderly disabled people with lower limb loss are: age over 66.5 years, female sex, body mass index over 28.5 kg/m2, duration of the post-amputation period at the time of primary prosthesis over 1 year.

Distinctive Cranial Abnormalities in Children with Early Infancy Insulin Dependent Diabetes in Connection with Wolcott-Rallison Syndrome

Background: Abnormal craniofacial contour and deformities of the appendicular skeleton of the lower limbs can occur in connection with a long list of skeletal dysplasia. The natural history of chronic/unusual disorders can be a precise key factor towards a successful diagnostic process. Material and Methods: Three unrelated children aged 18-months, 2 and 7 year (two boys and one girl) presented with generalised skeletal mal-development compatible with multiple epiphyseal dysplasia. However, in the early months of life these children have been diagnosed with early infancy insulin dependent diabetes. Strikingly, all manifested Wormian bones over the lambdoid sutures. In addition to Progressive malalignment of the lower limbs and thoracic kyphosis associated with significant growth deficiency were the major skeletal abnormalities. Endochondral ossification was diffusely affected associated with multiple epiphyseal dysplasia and appearance of irregular and fragmented epiphyses. Results: Based on early permanent neonatal or early-infancy insulin-dependent diabetes, associated with the clinical and radiological phenotypic characterizations of multiple epiphyseal dysplasia and growth retardation as the major abnormalities in these children. Wolcott-Rallison syndrome was the highly likely diagnosis. The most interesting findings in this study; The Wormian bones, not only involved the lambdoid sutures, but also extended to involve the occipitomastoid suture. We confirmed our diagnosis via sequence analysis which revealed a homozygous nonsense gene mutation (EIF2AK3) resulting in a premature stop codon (c.2707 C>T, p.R903X). Parents in two families were identified as heterozygous carriers of this mutation. Conclusion: The significance of this study is to point out the importance of analysing the skull bones in children with skeletal dysplasia. Interestingly, for the first time we detected the downward bulging of the occiput because of the abundancy of the Wormian bones (sites of maximal skull bone fragility) along the lambdoid and the occipito-mastoid sutures is by itself a highly alarming sign of progressive weakness of the weight-bearing zone (which is represented by the lambdoid and the occipitomastoid sutures). The exerting load of the cerebrum on these vulnerable and soft sutures can lead to brain swelling (a serious condition). Despite that lower limb defects in children are the most frequent deformities encountered in the departments of paediatric orthopaedics, because of the real or apparent detrimental effect that they have on gait and upright activity, and it draw immediate and alarming attention to parents. But, nevertheless, it is the responsibility of physicians to pay prompt attention for the necessity to scrutinize the skulls and the craniocervical junction in patients with long term bone disorders.

Fetal phenotype of Cornelia de Lange syndrome with a molecular confirmation

ObjectiveTo present the fetal features of Cornelia de Lange Syndrome (CdLS) with a molecular confirmation. Study designThis was a retrospective study of 13 cases with CdLS diagnosed by prenatal and postnatal genetic testing and physical examination. Clinical and laboratory data were collected and reviewed for these cases, including maternal demographics, prenatal sonographic findings, chromosomal microarray and exome sequencing (ES) results, and pregnancy outcomes. ResultsAll of the 13 cases were detected to have a CdLS-causing variant, with 8 variants identified in the NIPBL gene, 3 in SMC1A, and 2 in HDAC8. Five had normal ultrasound scans during pregnancy; all were caused by variants of SMC1A or HDAC8. For the eight cases with NIPBL variants, all had prenatal ultrasound markers. Three had first trimester ultrasound markers including increased nuchal translucency in one and limb defects in three. Four presented with normal ultrasound in the first trimester, but abnormal ultrasound in the second trimester, including micrognathia in two, hypospadias in one and intrauterine growth retardation (IUGR) in one. IUGR as the isolated feature was identified in one case in the third trimester. ConclusionThe prenatal diagnosis of CdLS caused by NIPBLvariants is possible. It seems to remain challenging to detect non-classic CdLS only relying on ultrasound examination.

Ambient air pollutants in the first trimester of pregnancy and birth defects: an observational study

ObjectivesAs current studies on the relationships between air pollutants exposure during the first trimester and birth defects were not fully elucidated, this study aimed to assess the association between selected...

Application of Three Different Types of Pedicled Latissimus Dorsi Flaps in Repairing Nearby Deep Soft Tissue Defects: Cosmetic Result and Shoulder Functionality Evaluation in One Center.

The latissimus dorsi (LD) flap has generally been considered a workhorse flap in clinics. However, the impairment of shoulder function and the dramatic appearance in the donor site are the major problems associated with traditional latissimus dorsi myocutaneous flap (LDMF). Here, we analyzed the reliability of three types of LD flaps in repairing deep soft tissue defects in the upper limbs, shoulder, back, and chest wall. From December 2016 to December 2020, 21 patients from our center underwent reconstruction of deep soft tissue defects using different types of LD flaps. The distribution of the thoracodorsal artery and the location of its branches were confirmed by imaging examination. Based on the defects, traditional LDMF, thoracodorsal artery perforator flap with capillary perforators (TAPcp), or low-skin-paddle pedicled LDMF was selected and specifically designed for each patient. The appearance satisfaction and shoulder functional of daily life recovery were evaluated. A total of 12 traditional LDMF, 4 TAPcp, and 5 low-skin-paddle pedicled LDMFs were used. All flaps survived well. The donor site was sutured directly with satisfactory appearance (n = 7) or repaired using skin grafts (n = 14). Compared to traditional LDMF, TAPcp and low-skin-paddle pedicled LDMF have faster shoulder function of daily life recovery. Based on the characteristics of defects, personalized design of different types of LD flaps is a reliable option to repair different defects.

FZD2 regulates limb development by mediating β-catenin-dependent and -independent Wnt signaling pathways.

Human Robinow syndrome (RS) and dominant omodysplasia type 2 (OMOD2), characterized by skeletal limb and craniofacial defects, are associated with heterozygous mutations in the Wnt receptor FZD2. However, as FZD2 can activate both canonical and non-canonical Wnt pathways, its precise functions and mechanisms of action in limb development are unclear. To address these questions, we generated mice harboring a single-nucleotide insertion in Fzd2 (Fzd2em1Smill), causing a frameshift mutation in the final Dishevelled-interacting domain. Fzd2em1Smill mutant mice had shortened limbs, resembling those of RS and OMOD2 patients, indicating that FZD2 mutations are causative. Fzd2em1Smill mutant embryos displayed decreased canonical Wnt signaling in developing limb mesenchyme and disruption of digit chondrocyte elongation and orientation, which is controlled by the β-catenin-independent WNT5A/planar cell polarity (PCP) pathway. In line with these observations, we found that disruption of FZD function in limb mesenchyme caused formation of shortened bone elements and defects in Wnt/β-catenin and WNT5A/PCP signaling. These findings indicate that FZD2 controls limb development by mediating both canonical and non-canonical Wnt pathways and reveal causality of pathogenic FZD2 mutations in RS and OMOD2 patients.

Fetal Hands: A Comprehensive Review of Prenatal Assessment and Diagnosis Over the Past 40 Years.

Fetal skeletal dysplasias involving limbs and hands are rare congenital malformations. Prenatal two-dimensional ultrasound diagnosis of fetal limb defects has a sensitivity of about 30%; however, an increased detection rate may be obtained using three-dimensional (3-D) ultrasound in the rendering mode. 3-D ultrasound may be used as a complementary method providing additional information. Currently, magnetic resonance imaging (MRI), with the emergence of ultrafast imaging techniques and new sequences, allows for better diagnosis of several fetal skeletal dysplasias such as limb reduction defects and neuromuscular disorders. 3-D volumetric images from ultrasound or MRI scan data allow 3-D ultrasound reconstructions of virtual/physical models, and virtual reality can help researchers to improve our understanding of both normal and abnormal fetal limb/hand anatomy. In this article, we review the embryological development of fetal hands and their main anomalies including prenatal diagnostic methods, genetic counseling, the role of orthopedic and plastic surgery reconstruction, and new perspectives in fetal surgery.

Epidermal nevi and epidermal nevus syndromes in pediatric practice

Epidermal nevi are hamartomas of ectodermal origin. Epidermal nevus syndromes are defined as the presence of an epidermal nevus with the involvement of other organ systems. Epidermal nevi are a manifestation of cutaneous mosaicism. Mosaicism may result from postzygotic somatic mutations. Epidermal nevi are usually located along the Blaschkos lines. There are organoid (sebaceous nevus, comedonic nevus, eccrine nevus) and non-organoid epidermal nevus (keratinocytic epidermal nevus, inflammatory linear verrucous epidermal nevus). Epidermal nevi can be combined with extracutaneous malformations: skeletal anomalies, neurological changes, eye defects, and then it can be related with epidermal nevus syndrome. The presence in a child of large epidermal nevi and nevi located on the face and scalp requires a multidisciplinary approach involving a pediatrician, neurologist, ophthalmologist and geneticist. Currently, several well-defined epidermal nevus syndromes characterized by organoid and non-organoid (keratocytic) nevi have been described: Schimmelpennings syndrome, phakomatosis pigmentosa, Beckers nevus syndrome, comedonal nevus syndrome, angora hairy nevus syndrome, Proteus syndrome, and CHILD syndrome (congenital hemidisplasia with ichthiosiform nevus and limb defects). Thus, follow-up of patients with epidermal nevus syndromes should be individualized and include a thorough history, clinical examination, and, if necessary, genetic testing. Typically, children with small, isolated epidermal nevus do not require further evaluation.

Application of augmented reality technique in repairing soft tissue defects of lower limbs with posterior tibial artery perforator flap

To investigate the accuracy and reliability of augmented reality (AR) technique in locating the perforating vessels of the posterior tibial artery during the repair of soft tissue defects of the lower limbs with the posterior tibial artery perforator flap. Between June 2019 and June 2022, the posterior tibial artery perforator flap was used to repair the skin and soft tissue defects around the ankle in 10 cases. There were 7 males and 3 females with an average age of 53.7 years (mean, 33-69 years). The injury was caused by traffic accident in 5 cases, bruising by heavy weight in 4 cases, and machine injury in 1 case. The size of wound ranged from 5 cm×3 cm to 14 cm×7 cm. The interval between injury and operation was 7-24 days (mean, 12.8 days). The CT angiography of lower limbs before operation was performed and the data was used to reconstruct the three-dimensional images of perforating vessels and bones with Mimics software. The above images were projected and superimposed on the surface of the affected limb using AR technology, and the skin flap was designed and resected with precise positioning. The size of the flap ranged from 6 cm×4 cm to 15 cm×8 cm. The donor site was sutured directly or repaired with skin graft. The 1-4 perforator branches of posterior tibial artery (mean, 3.4 perforator branches) in 10 patients were located by AR technique before operation. The location of perforator vessels during operation was basically consistent with that of AR before operation. The distance between the two locations ranged from 0 to 16 mm, with an average of 12.2 mm. The flap was successfully harvested and repaired according to the preoperative design. Nine flaps survived without vascular crisis. The local infection of skin graft occurred in 2 cases and the necrosis of the distal edge of the flap in 1 case, which healed after dressing change. The other skin grafts survived, and the incisions healed by first intention. All patients were followed up 6-12 months, with an average of 10.3 months. The flap was soft without obvious scar hyperplasia and contracture. At last follow-up, according to the American Orthopedic Foot and Ankle Association (AOFAS) score, the ankle function was excellent in 8 cases, good in 1 case, and poor in 1 case. AR technique can be used to determine the location of perforator vessels in the preoperative planning of the posterior tibial artery perforator flap, which can reduce the risk of flap necrosis, and the operation is simple.

Homozygosity for a novel DOCK6 variant in an individual without aplasia cutis congenita of the scalp and terminal transverse limb defects.

Homozygosity for a novel DOCK6 variant in an individual without aplasia cutis congenita of the scalp and terminal transverse limb defects.

Sural Artery as a Recipient Vessel for Free Flaps.

Introduction The axial vessels like the anterior and posterior tibial emerge as the first choice of recipient vessels, in free flaps for lower limb trauma. When the defects are located more proximally in the leg, the deeper course of the axial vessels makes the dissection more tedious. Alternative vessels like the descending genicular, medial genicular, and distal end of the descending branch of the lateral circumflex femoral can be used for an end-to-end anastomosis, well away from the zone of trauma. The objective of this study was to define the indications and technique of the use of the sural vessels as the recipient pedicle for proximal and middle third leg defects. Patients and Methods For the period 2006 to 2022, 18 leg defects following road traffic accidents were covered with latissimus dorsi muscle flap using sural vessels as the recipient pedicle. Results Among 18 patients, 8 patients had defect in proximal third, 8 had a combined defect in proximal and middle third leg, and 2 had defect in middle third leg. Two patients developed arterial thrombosis and one patient had venous thrombosis for which re-exploration was performed. Two flaps were lost and sixteen had successful wound coverage. Conclusion The sural vessels as recipient pedicle are easier to access and can be considered as a reliable option for free flaps in limb defects of proximal and middle third leg. Using the submuscular part of the vessel ensures a better distal reach of the flap.

Reliability of Single Perforator Based Free Anterolateral Thigh Flap Reconstruction among Children with Post Traumatic Limb Defects

Aim: To evaluate reliability of anterolateral thigh flap based on a single perforator in pediatric population for lower limb post traumatic defects. Study design: Retrospective cross sectional study Place and duration of study: Pediatric plastic surgery department of the children hospital & university of child health science, Lahore from January 2018 to January 2022. Methodology: After approval from ethical review board, 50 patients who underwent a free Alt flap reconstruction were included in this retrospective analysis. All flaps for lower limb post traumatic raised on a single perforator were included and reliability of flap was assessed for technical feasibility, outcomes and safety. Data was entered and analysed in SPSS ver: 21.0 and presented as frequency and percentages. Results: Mean age was 8.2±3.45 years 64% were males.96% had undergone road traffic accident (4% burn).Lower limb was affected in majority of patients 80% (upper limb 20%). 78% of patients were discharged at 10th post-operative day and 8% had partial flap loss. Conclusion: We concluded in this studyfree tissue transfer of anterolateral thigh flap based on a single perforator in children for covering large defects is a reliable method with good safety margin and minimal complication rate. Keywords: Anterolateral thigh flap, single perforator, pediatric population, free flap, free tissue transfer, perforator flap reliability

TMEM161B regulates cerebral cortical gyration, Sonic Hedgehog signaling, and ciliary structure in the developing central nervous system

Sonic hedgehog signaling regulates processes of embryonic development across multiple tissues, yet factors regulating context-specific Shh signaling remain poorly understood. Exome sequencing of families with polymicrogyria (disordered cortical folding) revealed multiple individuals with biallelic deleterious variants in TMEM161B, which encodes a multi-pass transmembrane protein of unknown function. Tmem161b null mice demonstrated holoprosencephaly, craniofacial midline defects, eye defects, and spinal cord patterning changes consistent with impaired Shh signaling, but were without limb defects, suggesting a CNS-specific role of Tmem161b. Tmem161b depletion impaired the response to Smoothened activation invitro and disrupted cortical histogenesis invivo in both mouse and ferret models, including leading to abnormal gyration in the ferret model. Tmem161b localizes non-exclusively to the primary cilium, and scanning electron microscopy revealed shortened, dysmorphic, and ballooned ventricular zone cilia in the Tmem161b null mouse, suggesting that the Shh-related phenotypes may reflect ciliary dysfunction. Our data identify TMEM161B as a regulator of cerebral cortical gyration, as involved in primary ciliary structure, as a regulator of Shh signaling, and further implicate Shh signaling in human gyral development.

Adult patient diagnosed with NADSYN1 associated congenital NAD deficiency and analysis of NAD levels to be published in: European Journal of Medical Genetics

IntroductionNicotinamide adenine dinucleotide (NAD) is an essential cosubstrate/coenzyme in multiple cellular redox processes and a substrate in several non-redox reactions. NADSYN1 encodes NAD synthetase 1, an enzyme in the NAD de novo synthesis pathway and the Preiss-Handler pathway, and biallelic pathogenic variants causes NAD deficiency associated with vertebral, cardiac, renal and limb defects. Szot et al. and Kortbawi et al. have reported a total of seven patients with NADSYN1 associated congenital NAD deficiency disorder with the oldest patient being seven years old. Patient dataWe present a male patient age 30 with a height of 130 cm and numerous skeletal malformations including segmentation defects of the spine, rib anomalies and unequal leg length as well as bilateral ptosis, cleft palate and asymmetric dysmorphic facial features. The patient underwent surgery for an aortic stenosis due to a bicuspid valve. No malformations of the kidneys or urinary tract were identified. ResultsTrio exome sequencing revealed a homozygous missense variant in NADSYN1 c.1717G > A (p.Ala573Thr). Both parents were unaffected carriers of the variant. Analysis of NAD levels showed that the patient had a lower NAD pool compared to his unaffected siblings. The NAD pool rose approximately 25% after supplementation with nicotinamide, a NAD precursor for the salvage pathway. ConclusionThe variant was previously reported in four patients and functional analyses by Szot et al. support the pathogenicity of the variant. We report an adult patient with NADSYN1 associated congenital NAD deficiency disorder and expand the phenotypic spectrum. We also present analysis of the NAD levels before and after supplementation with nicotinamide.

Is the Tradeoff between Folic Acid or/and Multivitamin Supplementation against Birth Defects in Early Pregnancy Reconsidered? Evidence Based on a Chinese Birth Cohort Study.

Several studies have reported conflicting results on the association between maternal exposure to folic acid (FA) and/or multivitamin (MV) supplements and the risk of birth defects (BDs), especially for different subtypes of BDs. The present study aimed to identify the association between maternal exposure to FA or/and MV and BDs in offspring. In the Chinese Birth Cohort Study initiated from 20 November 2017, 120,652 pregnant women completed follow-up until 20 August 2021. The participants were classified into four groups: without exposure to FA and MV, exposure to only FA, exposure to only MV, and exposure to FA and MV. Birth defects were coded by the International Classification of Diseases (ICD)-10. In order to explore the structural relationship between maternal FA or MV supplements and BDs, directed acyclic graphs were drawn. Then, an inverse probability treatment weighting was utilized to reduce the systematic differences in the baseline characteristics among the different groups. Lastly, a two-level mixed-effect log binomial regression analysis was used to estimate the relative risk (RR) value of the different subtypes of BDs under different exposures to FA and/or MV. Compared with the maternal group without exposure to FA and MV, the RR values of nervous system defects, face, ear, and neck defects, limb defects, and CHDs in the maternal group with only FA supplementation were less than 1.0, but they were not statistically significant. The RR values of genitourinary defects, abnormal chromosomes, and oral clefts were more than 1.0, and they were also not statistically significant. However, the risk of genitourinary defects (RR: 3.22, 95% CI: 1.42-7.29) and chromosomal abnormalities (RR: 2.57, 95% CI: 1.16-5.73) in the maternal group with only MV supplementation increased more than those in the maternal group without exposure to FA and MV. In addition, the RR values of all subtypes of BDs in the maternal group with exposure to FA and MV were closer to 1.0 than those in maternal group with exposure to only MV, but they were not statistically significant. It was indicated that the simultaneous supplementation of FA and MV in early pregnancy may have an interaction for the prevention of BDs and may have inconsistent effects for different subtypes of BDs. At the same time, excessive FA supplementation in pregnant women may increase the risk of BDs in their offspring. Although the mechanism is not clear, this evidence reminded us that more trade-offs are necessary for formulating strategies for the prevention of BDs with FA and/or MV supplementation in early pregnancy.

Significance of foetal autopsy in diagnosis of VACTERL association

VACTERL association occurs in 1 in 10000- 40000 live births and is associated with vertebral defects, anal atresia, cardiac malformations, tracheoesophageal fistula, renal dysplasia and limb defects. A 19-week-old male foetus with antenatal diagnosis of foetal hydronephrosis on antenatal ultrasound in the left kidney was medically terminated and brought for foetal autopsy. No other malformation was reported on antenatal scan. On autopsy, facial abnormalities, upper and lower limb defects, coronal vertebral clefts and imperforate anus with a two vesselumbilical cord were found. Both environmental and genetic factors are considered to be responsible for this association. The present case illustrates the importance of foetal autopsy for making a definitive diagnosis in the missed sonographic findings like anal atresia and absence of upper- and lower-limb bones.

The deep inferior epigastric artery perforator flap: a narrative review on its various uses in non-breast reconstruction.

The deep inferior epigastric artery perforator (DIEP) flap was first described by Koshima and Soeda in 1989 and is now well-established as the gold standard in breast reconstruction. Lately, this issue has been explored in the context of head and neck reconstruction, highlighting growing interest in the use of the DIEP flap beyond breast reconstruction, but its usage in other anatomical regions appears elusive. Nevertheless, DIEP flap reconstruction may be a viable choice for complex, three-dimensional head and neck deformities while upholding the criteria of minimal donor site morbidity, according to a recent review. To determine whether the DIEP flap may be used successfully in other types of reconstruction, we conducted a review on the use, applications, and outcomes of the DIEP flap in non-breast reconstruction. This is, as far as we are aware, the first comprehensive analysis of all applications of the DIEP flap other than for breast reconstruction. A literature review was performed using PubMed to include all relevant articles in English or French published up to February 2022. Keywords included "DIEP flap" and "deep inferior epigastric perforator flap". A total of 1,299 articles were identified with 105 on the use of the DIEP flap in non-breast reconstruction. This suggests increasing recognition of the DIEP flap as a feasible option for reconstruction of most anatomical regions, especially in lower limb and head and neck reconstruction, followed by gynecological reconstruction. The DIEP flap was also utilized in the reconstruction of upper limb, thigh and hip defects. Less commonly, it has been used for penoscrotal, groin, sternal, buttock and abdomen reconstruction. The scientific body of evidence showed the robustness and versatility of the DIEP flap in non-breast reconstruction, with its relative pros and cons at different anatomical regions.

Determination of the adequate vascular perfusion time of cross-leg free latissimus dorsi myocutaneous flaps in reconstruction of complex lower extremity defects.

Lower limb defects may be present due to various causes including infections, vascular diseases, tumor resections, and crush or avulsion injuries. Management of lower leg defects is acomplex problem, especially when they are large with deep soft tissue loss. These wounds are difficult to be covered with local skin flaps, distant skin flaps or even conventional free flaps because of the compromised recipient vessels. In such cases, the vascular pedicle of the free flap could be anastomosed to the recipient vessels of the contralateral healthy leg temporarily and then divided after adequate neo-vascularization of the flap from the wound bed. The ideal time to divide such pedicles should be investigated and accurately assessed to have the maximum success rate possible for these challenging conditions and procedures. Sixteen patients who did not have asuitable adjacent recipient vessel for free flap reconstruction had been operated with cross leg free latissimus dorsi flap between February 2017 and June 2021. The mean soft tissue defect dimension was 12 ×11 cm (the smallest 6 × 7 cm; the largest 20 × 14cm). Gustilo type 3B tibial fractures were present in 12 patients, while no fractures were present in the other 4 patients. Preoperatively, arterial angiography was performed on all patients. After the fourth week post-operatively, non-crushing clamp was applied around the pedicle for 15minutes. The clamping time was increased by 15 minutes on each consequent day (average 14 days). On the last 2 days, the pedicle was clamped for 2 hours, and bleeding was assessed by aneedle prick test. Clamping time was assessed in each case to reach ascientific calculation of the adequate vascular perfusion time needed for full flap nourishment. All flaps survived completely except two cases of distal flaps necrosis. Cross-leg free latissimus dorsi can provide asolution for large soft-tissue defects in lower extremities especially with absence of any suitable recipient vessels or when the use of vein grafts would not be feasible. However, ideal time before dividing the cross vascular pedicle should be identified to have the maximum success rate possible.

VACTERL association with non-rotated bowel: A rare case report

VACTERL is an acronym for a group of congenital anomalies where V stands for vertebral defects or vascular anomalies(single umbilical artery), A stands for anal atresia, C stands for cardiac defects, TE denotes trachea-esophageal fistula, R stands for renal abnormalities and L stands for limb defects such as polydactyly or syndactyly. Atleast, three of above features are considered necessary for diagnosis of the condition [1].It may or may not be associated with other bowel pathologies (most commonly duodenal obstruction).If there is accompanying hydrocephalus, then the term VACTERL-H is used.The estimated incidence is 1 in 10,000 – 40,000 births. Few familial cases have been identified and maternal diabetes and maternal diabetes is only environmental influence implicated. It is called an association, rather than a syndrome because the complications are not pathogenetically related, tend to occur more frequently than expected and are thought to be linked to embryonic mesodermal defects.It is thought to be autosomal recessive in nature [2].It has been statistically associated with maternal exposure to exogenous sex hormones during the first trimester of pregnancy [3].It has also been associated with Fanconi Anemia [4].