Ulnar Ray Deficiency: A New Classification and a Unique Case Report.

Ulnar ray deficiency is a rare congenital upper limb defect. We report on a unique case with hand-on-flank deformity on the one side and limb truncation on the contralateral side. The standard of care for the hand-on-flank deformity is to do humerus osteotomy to reposition the hand anteriorly. However, the right limb truncation in our patient made the senior author decide not to do the osteotomy. Final assessment showed that the posterior hand position enabled the patient to reach the ano-genital areas, the pocket, and the mouth. It was concluded that in case of hand-on-flank deformity in one limb and limb truncation of the contralateral limb, osteotomies to bring the hand anteriorly are not advised.

My journey in hand surgery: combining patient care, clinical and basic science research.

This review is about my clinical and research journey in hand surgery. The journey has been a worthwhile and meaningful one, especially when I felt there were areas I could influence management, whether this be rare cases, common conditions or where a suggested algorithm may be helpful. I also had the unique privilege of working with geneticists, which has resulted in clinical-pathological publications that could influence patient management, as shared from a clinician's perspective. It is hoped this article will inspire young clinician scientists to pursue a journey of collaboration with other researchers.

Distal Phalanx Intraosseous Epidermoid Cyst.

An intraosseous epidermal cyst is a benign cystic lesion that occurs in the bones. It is assumed to be caused by congenital causes or trauma, and because the cyst forms in the soft tissue surrounding the bone, it can lead to bone loss. Intraosseous epidermal cysts have a well-defined radiolucent lesion with cortical extension on radiography. Due to clinical and radiological signs being similar, it is vital to distinguish an intraosseous epidermal cyst from other diseases that develop at the distal phalanx. A rare example of intraosseous epidermal cysts at the distal phalanx is reported. We describe the clinical, radiological, and pathologic aspect of this lesion, as well as our current therapeutic strategy.

The Association of Cone-shaped Epiphysis and Poland Syndrome.

Poland syndrome is a congenital anomaly with two clinical features: unilateral agenesis of the pectoralis major and ipsilateral hand symbrachydactyly. We report a rare case of bilateral Poland syndrome with several unique features. Poland syndrome is thought to be due to a vascular insult to the subclavian axis around the sixth week of gestation. Our patient has multiple rare and unique features of Poland syndrome. For the first time in the literature, we associate Poland syndrome with cone-shaped epiphysis of the metacarpals of all fingers. Bilaterality, cleft hand deformity, and dextrocardia were also rare features in our patient.

Congenital hairy nevus: A case report of long-term follow-up following curettage of the nevus at the age of 6 hrs.

INTRODUCTION: Management of giant hairy nevi depends on various factors including the size and anatomical area. CASE PRESENTATION: We report a case of a giant hairy nevus treated successfully with curettage at the age of 6 hrs after birth. There was partial recurrence of pigmentation and hair on long-term (10 years) follow-up. DISCUSSION: Although curettage is a known method of treating hairy nevi, long term results are lacking when the nevus is treated in the first few hours after birth. CONCLUSION: Following curettage of hairy nevi in the first few hours after birth, recurrence of pigmentation and hair may still occur on long term follow up.

Herpetic Whitlow of the Hand in Infants.

Introduction Herpetic whitlows in infants are rare. Previous authors only reported individual case reports. We present a case series of six infants. Materials and Methods This is a retrospective study of six cases of herpetic whitlows in infants seen by the senior author (MMA) over the past 23 years (1995-2017 inclusive). The following data were collected: age, sex, digit involved in the hand, mode of transmission, time of presentation to the author, clinical appearance, presence of secondary bacterial infection, presence of other lesions outside the hand, method of diagnosis, treatment, and outcome. Results All six infants initially presented with classic multiple vesicles of the digital pulp. In all cases, there was a history of active herpes labialis in the mother. Incision and drainage or deroofing of the vesicles (for diagnostic purposes) resulted in secondary bacterial infection. Conclusion The current report is the first series in the literature on herpetic whitlows in infants. We stress on the mode of transmission (from the mother) and establishing the diagnosis clinically. In these cases, no need for obtaining viral cultures or polymerase chain reaction; and no medications are required. Once the vesicles are disrupted, secondary bacterial infection is frequent and a combination of oral acyclovir and intravenous antibiotics will be required.

Genetics, Clinical Presentation, Radiological Features, and Midterm Outcome of Closing Wedge Osteotomy in Children With Brachydactyly Type C.

BACKGROUND: Brachydactyly (BD) type C is a rare form of familial BD caused by GDF5 mutations. Some of the affected children have severe clinodactyly requiring surgery. The literature is limited to case reports. PATIENTS AND METHODS: The current retrospective study included 15 Saudi Arabian families with 42 affected children seen by the author for 25 years. A total of 23 digits (in 23 hands) underwent surgical correction of clinodactyly using a closing wedge osteotomy. The current study reports on the genetics, clinical presentation, radiological features, and midterm outcome of surgery. RESULTS: Genetic analysis was done in 6 families and confirmed the presence of 2 novel missense mutations (p.Met173Val in 3 families and p.Thr203Asn in 3 families) in the GDF5 gene. All cases in the study group demonstrated the classical clinical and radiographic features of BD type C. However, only 1 hand showed all the features of angel-shaped bony defect. The clinodactyly defect was mostly observed in the index or middle fingers. Surgery for the clinodactyly defect was only done if there was finger overlap. Closing wedge osteotomy was done in a total of 23 digits with a satisfactory outcome. CONCLUSIONS: This study represents the largest reported series of children undergoing surgery for correction of BD type C clinodactyly with a uniform technique performed by a single surgeon. The closing wedge osteotomy used resulted in good midterm outcomes, although long-term follow-up is lacking.

Skin Preservation in the Debridement of Necrotizing Fasciitis: A Demonstrative Case Report.

Necrotizing fasciitis (NF) is a rare yet potentially fatal soft-tissue, polymicrobial infection. Aggressive debridement of the fascia and overlying skin as well as antimicrobial coverage constitute the mainstay of management, often leaving large skin defects. However, we demonstrate in this paper a case of a young woman who developed NF after liposuction and was treated by fascial debridement with minimal skin excision. Skin preservation will reduce the morbidity and improve the final aesthetic outcome.

The Pathogenesis of Pierre Robin Sequence through a Review of SOX9 and Its Interactions.

The literature does not offer any review of the pathogenesis of the clinical features of syndromes with Pierre Robin sequence (PRS). The senior author (MMA) proposed a hypothesis that SOX9 and its interactions may play a key role in this pathogenesis. The current review aims to test this hypothesis. Methods: Three literature searches were made: the first aimed to document the main syndromes associated with PRS; and the second was to document the main functions of SOX9 in development; and the third was to investigate if SOX9 and its interactions may play a role in the pathogenesis. Results: SOX9 is the main positive regulator in the development of the mandibular cartilage and it also enhances collagen type II (the main collagen type in cartilage) expression in the mandibular cartilage. Furthermore, SOX9 participates in neural crest development, binds to the exon junction complex, and participates in sex determination. The interactions of SOX9 could explain the pathogenesis of the clinical features of syndromic PRS. These included interactions with collagen type II (in Strickler syndrome), exon junction complex (in Richier-Costa-Periera syndrome), glucose (in Catel-Manzke syndrome), RNA-binding proteins (in TARP syndrome), and the spliceosome (in cerebra-costo-mandibular syndrome). Finally, SOX9 mutations cause campomelic dysplasia. Conclusions: The review supports the hypothesis of the participation of SOX9 in the pathogenesis of the clinical features of syndromic and nonsyndromic PRS. This should guide future research on the topic.

A technical tip in the use of abdominal pedicled flaps in hand reconstruction: A case repot.

INTRODUCTION: Abdominal pedicled flaps are one of the options utilized in the reconstruction of complex hand defects. In this communication, the author presents a case report demonstrating a new technical tip which was utilized in a young child. IMPORTANCE: The technical tip will help closure of further hand defects utilizing the same flap. CASE PRESENTATION: An abdominal pedicled flap was used to reconstruct the dorsal aspect of the hand and wrist following a deep contact burn. An "extension" of the flap from the abdomen at the time of flap division was harvested and utilized to cover an adjacent defect in the fingers. CLINICAL DISCUSSION: Several technical tips in the utilization of the pedicled groin/abdominal flaps in hand reconstruction have been reported. The author presents another tip. CONCLUSION: When utilizing abdominal pedicled flaps for hand reconstruction, an "extension" of the flap from the abdomen at the time of flap division may be harvested. This "extension" may be utilized to cover further adjacent defects in the hand.

Challenging the Subcostal Incision Scar with the Two-staged Abdominoplasty: An Innovative Approach.

Abdominoplasty is a commonly sought-after procedure due to its life-transforming results, but is limited, as in any other operation, by a number of contraindications. One of these contraindications is a subcostal scar, which may jeopardize blood supply to the upper flap of the abdominoplasty, resulting in skin necrosis. Herein, we challenge this dogma by introducing the two-staged abdominoplasty with the utilization of a delayed flap in a 48-year-old multiparous woman presenting with a Kocher incision of open cholecystectomy, with good results and a complication-free course of 3 postoperative months. We recommend this approach in patients with subcostal scars. However, more research into the utilization of delayed flaps in abdominoplasty should be done to have a more well-founded conclusion.

Type II D Phalangeal Neck Fractures in Children: A Series of 20 Cases Treated According to a Preset Stepwise Algorithm.

Introduction Type II phalangeal neck fractures are defined as displaced fractures with bone-to-bone contact at the fracture site. In the type II D subtype, the distal fracture fragment is thin. A review of the literature did not reveal any study investigating the outcome of management of these fractures. Patients and Methods This is a retrospective study of 20 consecutive children with type II D phalangeal neck fractures treated over the past 4 years. Demographic data were reviewed. All cases were managed according to the preset stepwise algorithm. The outcome of management at final follow-up was documented using Al-Qattan's grading system. Results The mean age was 30 months (range 12-80 months). There were 12 males and 8 females. The mean follow-up was 2 years (range 7 months to 3 years). The largest two categories within the management algorithm were patients with minimally displaced fractures treated conservatively ( n = 8) and those with displaced fractures treated with closed reduction and percutaneous K-wire fixation ( n = 9). Fischer exact test was used to compare the outcome in these two groups and the p value was significant ( p = 0.015), indicating a significantly better outcome in the former group. Conclusion Several conclusions were made from the study. Type II D of phalangeal neck fractures tend to occur in young children and the majority involve the middle phalanx. The thinness of the distal fracture fragment makes standard techniques of closed reduction more difficult. However, flexion of the proximal and distal joints appears to be effective in reducing dorsally displaced type II D fractures by closed means. Finally, a more conservative approach to minimally displaced type II D fractures results in a better outcome compared with closed reduction and percutaneous K-wire fixation.

Fixation at the Inferior Orbital Rim in Medially Rotated Zygomatic Complex Fractures.

BACKGROUND: Zygomatic complex fractures are quadri-pod fractures because of the involvement of the zygomaticotemporal, zygomaticomaxillary, zygomaticofrontal, and zygomaticosphenoid junctions. The need to address one or more fixation points of these four articulations mainly depends on the degree of displacement. In this article, the authors present a series of medially rotated zygomatic complex fractures to demonstrate that one-point fixation at the inferior orbital rim (IOR) is sufficient in most cases. METHODS: This is a retrospective study of all medially rotated zygomatic complex fractures treated by the authors over the last 4 years. RESULTS: The patients were six men with a mean age of 41 years. All patients sustained medially rotated zygomatic complex fractures. Three patients had symptomatic orbital floor defects. One patient had concurrent displaced superior orbital rim fracture. Surgery was done using the transconjunctival approach. A titanium mesh was used to fix the IOR. For orbital floor reconstruction, the same titanium mesh was extended into the floor to cover the defect. The patient with concurrent superior orbital rim fracture required a second point of fixation at the lateral orbital rim. CONCLUSIONS: Single-point of fixation at the IOR is sufficient in most medially rotated zygomatic complex fractures as long as there is minimal displacement at other fracture points. Some of these patients may have symptomatic orbital floor defects. Simultaneous fixation of the IOR and orbital floor reconstruction may be done via a transconjunctival approach.

"Trap Door" Orbital Floor Fractures in Adults: Are They Different from Pediatric Fractures?

BACKGROUND: "Trap door" orbital floor fractures are usually seen in children. In the linear fracture type, a break occurs in the bones of the orbital floor that permits orbital tissue to prolapse into the fracture site during fracture formation. The bony fragments of the fracture then return to their original position, entrapping the prolapsed orbital tissue. In the hinged fracture type, the fractured part of the floor is minimally displaced into the maxillary sinus and acts as the hinge of a "swinging door" entrapping the orbital tissue. METHODS: We report on a series of 10 adult patients with hinged orbital floor fractures. RESULTS: The mean patient age was 35 years. Seven patients had a pure orbital floor fracture and the remaining 3 patients had fractures of both the orbital floor and the inferior orbital rim. All 10 patients had diplopia in the upgaze. None of the patients had signs of the oculocardiac reflex. None of the CT scans showed a "tear-drop" sign. However, the sagittal CT scans showed evidence of entrapment of the inferior rectus. All patients had a small area of a slightly depressed orbital floor at the site of entrapment, without a bony defect. All patients underwent surgery through an infraorbital incision and a titanium mesh was used in all patients. There were no postoperative complications. All patients regained full range of motion of the globe. CONCLUSION: This is the first series in the literature that reports on the characteristics and outcome of trap door orbital floor fractures in adults.

Pedicled Abdominal Flaps for Hand Reconstruction in Adults: Physiotherapy of the Attached Hand.

Hand reconstruction using pedicled abdominal flaps has several disadvantages, including delayed hand therapy leading to stiffness. METHODS: This is a retrospective study of 70 cases of pedicled abdominal flaps used for hand reconstruction in adults in whom physiotherapy of the attached hand was implemented. The review aims to investigate the rate of flap dehiscence, infection, hematoma, and flap edge necrosis in our cases, and to establish that physiotherapy of the attached hand is not associated with an increased risk of complications. The review also aims to establish the effectiveness of physiotherapy of the attached hand in reducing the risk of stiffness by documenting the range of motion of the uninjured digits immediately after flap division and at final follow-up. RESULTS: There were no cases of flap dehiscence, infection, or hematoma. Six cases had minor partial flap edge necrosis that was treated conservatively and allowed to heal by secondary intention. In 62 patients, the range of motion of the uninjured digits was 90%-100% of the normal range of motion at day 1 post-flap division, and all of these patients recovered a full range of motion (in the uninjured digits) within 2 weeks of follow-up. Eight patients were reluctant to do the exercises as instructed because of low pain threshold; 4 of these 8 patients had residual stiffness at the final follow-up. CONCLUSION: The implementation of active exercises of the attached hand is feasible in selected cases and it helps minimize the risk of stiffness of the hand.

Hand Surgery in Saudi Arabia.

Hand surgery is a unique field that incorporates multiple specialties, aiming to provide the patient with a best possible functional and aesthetic results. Hand surgeons deal with different pathologies that require skills in several aspects of surgery. The field of hand surgery has evolved significantly over the past decades across the globe. This specialty has also been evolving in Saudi Arabia over the past 25 years. Some of the services offered to patients include specialized centers for brachial plexus, peripheral nerve, and pediatric hand surgery as well as centers for work-related hand injuries. There has also been significant contribution to the hand surgery literature from the hand surgeons working in Saudi Arabia, with hundreds of papers published in journals pertaining to hand surgery, orthopedic surgery, and plastic surgery, as well as the publication of several novel mutations causing congenital hand defects in journals concerned with genetics. The recent approval of a hand and microsurgery fellowship program in Saudi Arabia will also help boost this field in the country and the region.

The Classification of VACTERL Association into 3 Groups According to the Limb Defect.

The VACTERL association (VA) is defined as the nonrandom co-occurrence of 6 anomalies: vertebral anomalies (V), Anal atresia (A), Cardiac defects (C), Tracheo-esophageal fistula (TE), Renal defects (R), and Limb anomalies (L). The current communication presents an argument that patients with VA should be classified into three district groups based on their limb defects: VACTERL1: patients with normal limbs; VACTERL2: patients with limb anomalies other than radial ray defects of the upper limbs; and VACTERL3: patients with radial ray defects of the upper limbs. The author will demonstrate that the rationale behind the L1-3 classification in patients in VA is based on the embryogenesis of the 6 affected anatomical areas in VA. The pathogenesis of VACTERL1 is secondary to perturbations of Sonic Hedgehog (SHH) interactions. SHH signaling is known to have a major role in the normal development of the vertebrae, ano-rectal area, heart, tracheo-esophageal area, and kidney. However, SHH is not involved in the development of the radial ray; hence, patients present with no limb defects. The pathogenesis of VACTERL2 is variable depending on the type of gene mutation. The pathogenesis of VACTERL3 is related to errors in a group of proteins (namely, the proteins of the TBX5-SALL4-SALL1 loop and the FGF8-FGF10 loop/ pathway). These proteins are essential for the normal development of the radial ray and they interact in the development of the other anatomical areas of VA including the heart and kidney. Hence, VACTERL3 patients present with radial ray deficiency.