West syndrome: A study of 26 patients receiving short-term therapy.

OBJECTIVE: We describe the electroclinical characteristics of a series of 26 patients with idiopathic West syndrome (WS), who had an excellent response to treatment with vigabatrin (VGB) and corticosteroids alone or in combination. METHODS: Evaluating the records of 178 patients with WS studied at Garrahan Hospital, Niño Jesús Hospital, and Clínica San Lucas between January 2005 and June 2017, we selected 26 patients that met the inclusion criteria of idiopathic WS. The inclusion criteria for idiopathic WS were (1) no personal history of disease, (2) normal neurological examination and neurodevelopment, (3) symmetric spasms in clusters not preceded by any other type of seizure, (d) symmetric hypsarrhythmia, (e) normal electroencephalogram (EEG) background, e.g., normal sleep EEG pattern, (f) normal magnetic resonance imaging (MRI) recording, (g) normal neurometabolic and genetic studies, and (h) at least 2 years of follow-up. RESULTS: Fifteen boys and 11 girls met the inclusion criteria of idiopathic WS. The current age of the children ranges between 2 years 10 months and 12 years 10 months. Age at first epileptic spasms (ES) ranged from 4 to 11 months, with a mean age of 7 and a median of 7.5 months, respectively; ES were in clusters, bilateral and symmetrical in all cases. Spasms were flexor in nine (34.7%), mixed flexor-extensor in 15 (57.7%), and extensor in three (7.6%). In all patients the EEG showed typical pattern of hypsarrhythmia. CONCLUSION: These patients with idiopathic WS who have an excellent response to initial treatment should be treated for a short period of time with adrenocorticotropic hormone (ACTH) and VGB alone or in combination.

Outcomes for adults with anorexia nervosa who do not respond early to outpatient treatment.

OBJECTIVE: To better understand those patients with anorexia nervosa who do not show early response to treatment and are likely to have poorer outcome. METHOD: From an existing data set of 187 patients with anorexia nervosa across 22 eating disorder outpatient services in the United Kingdom, participants who had started treatment and had at least one body mass index (BMI) observation in the first 6 weeks of treatment were eligible for these secondary analyses (N = 65), a latent class analysis of BMI change over the first 6 weeks of treatment. Fifty-six patients showed no early change in BMI. We used logistic regression to examine predictors of good outcome in the 40 participants who had 12-month follow-up data. Predictors included global EDE-Q, negative affect (Depression, Anxiety, and Stress Scales) and functional impairment (Work and Social Adjustment Scale). RESULTS: Good outcome was achieved by 23% of patients and remission by 15%. Good outcome was predicted by less functional impairment at baseline. DISCUSSION: Further work that can identify sub-groups of patients with anorexia nervosa who do not achieve good outcome after treatment will inform the development of targeted engagement approaches.

Ventral foramen magnum neurenteric cysts: a case series and review of literature.

Neurenteric cysts (NEC) are uncommon, benign, congenital lesions. Ventral foramen magnum (FM) location is very rare. The difficulties in diagnosis and management aspects are detailed with a review of the pertinent literature. We report four new cases of ventral FM NEC, all managed surgically and present a literature review of ventral FM NEC. A retrospective analysis of histopathologically confirmed cases of ventral FM NEC, operated from 2010-2013 at our institute, was performed. For review, only those cases of NEC extending from the lower clivus to the C2 level constituting the foramen magnum were included. Including our four cases, a total of 47 cases were identified. The male to female ratio was 1.2:1. Mean age was 33.5 years (range 1-60 years). Neck pain and occipital headache were the most common symptoms, followed by limb weakness and cranial nerve paresis. Recurrent meningitis was noted in three cases. Hyperintensity on both T1- and T2-weighted sequences with absent enhancement was the most common finding on MRI. Surgical approaches were as follows: suboccipital (n = 21), far/extreme lateral (n = 18), retrosigmoid (n = 6), and transoral (n = 4). The extent of resection was as follows: total, 26; near total, 6; subtotal, 9; and partial, 3 cases. Cerebrospinal fluid diversion was done in four cases for intracranial hypertension. Mean follow-up duration was 26.8 months (range 1 month-9 years). Recurrence was noted in four (8.5 %) cases. One (2 %) case had malignant transformation. Mortality rate was 4 %. Foramen magnum neurenteric cysts are rare, benign tumors of the central nervous system. Accurate preoperative diagnosis can often be established with MRI. Surgical removal is the treatment of choice. Complete excision is ideal but often not possible. Near total removal would suffice with good progression-free periods. A long-term follow-up with radiological studies is necessary as delayed recurrences can occur.

Benign neuromyelitis optica is rare in Japanese patients.

Good-outcome neuromyelitis optica (NMO) is defined as an Expanded Disability Status Scale (EDSS) score of ≤3.0 at 10 years after onset. The clinical courses of 80 consecutive patients with NMO were analyzed to identify the frequency and features of Japanese patients with good-outcome NMO. Of the 80 patients, 37 had a disease duration of >10 years; of these, eight (21.6%) presented a good outcome. These cases presented lower EDSS scores during the early phase of disease compared with those with conventional NMO. However, half of these patients developed severe disabilities later on, indicating that truly benign NMO is rare.

Schwannoma in the Thenar Eminence: a Case Report.

This report describes a rare presentation of schwannoma in the thenar aspect of a surgeon and reviews the literature. A 35-year-old surgeon had a slow-growing swelling in his left thenar eminence. Clinical and radiological findings suggested it was a well-encapsulated mass within the abductor pollicis brevis muscle. A complete surgical excision was done, and the histopathological findings confirmed schwannoma, a benign peripheral nerve tumor. The surgeon remained symptom-free and had no recurrence at 1 year of follow-up. Though a benign peripheral nerve tumor is rare in the hand, it remains one of the differential diagnoses for a thenar eminence swelling. Surgical enucleation preserving the nerve fascicles achieves an excellent functional outcome.

Isolated insular stroke: topography is the answer with respect to outcome and cardiac involvement.

Background and purpose: Isolated insular strokes (IIS) are a rare occurrence due to the frequent concomitant involvement of adjacent territories, supplied by the M2 segment of the middle cerebral artery (MCA), and clinical aspects are sometimes contradictory. We aimed to describe clinical and radiological characteristics of a pure IIS case series, focusing on its functional outcome and cardiac involvement. Methods: We identified 15 isolated insular ischemic strokes from a pool of 563 ischemic strokes occurred between January 2020 and December 2021. Data collection consisted of demographic and baseline clinical characteristics, comorbidities, electrocardiograms, echocardiograms, stroke topography and etiology, reperfusive treatments, and outcome measures. Descriptive statistical analysis was carried out. Results: Newly detected cardiovascular alterations were the prevalent atypical presentation. Cardioembolism was the most frequent etiology. Most of patients had major neurological improvement at discharge and good outcome at 3-months follow-up. Discussion and conclusion: IIS are extremely rare, representing according to our study about 2.6% ischemic strokes cases per year, and patients have peculiar clinical manifestations, such as dysautonomia and awareness deficits. Our data suggest the possibility for these patients to completely recover after acute ischemic stroke notwithstanding the pivotal role of the insula in cerebral connections and the frequent association with MCA occlusion. Moreover, given the central role of the insula in regulating autonomic functions, newly detected cardiac arrhythmias must be taken into consideration, as well as a full diagnostic work-up for the research of cardioembolic sources. To our knowledge, this is the largest monocentric case series of IIS and it might be useful for future systematic reviews.

Non-vascularized toe phalangeal transfer for reconstruction of a giant cell tumour of the proximal phalanx.

This article describes a 50-year-old woman with a giant cell tumour involving the base of the proximal phalanx, which was resected and reconstructed with a non-vascularised toe phalanx graft. The toe phalanx graft united well, and there was no tumour recurrence at the 24-month follow-up.

Distal Nerve Transfers in Hand and Forearm for Traumatic Brachial Plexus and Peripheral Nerve Injuries: A Narrative Review.

Background: Various studies published good outcomes in brachial plexus injuries using nerve transfers for shoulder and elbow functions. However, little is known about the outcome of the distal nerve transfers in the forearm and hand. Targeting the nerve distally produces an early return of function in brachial plexus and peripheral nerve injuries (BPPNI). Therefore, researchers have focused on nerve transfers from the motor branches of the ulnar, median, and radial nerve. Similarly, sensory reinnervation is also obtained by potential donor transfers in the forearm and hand. There have been various attempts by surgeons to target the muscle and promote early reinnervation by different nerve transfers. Conclusions: The distal nerve transfers in the forearm and hand are promising when performed early. It effectively restores hand and forearm functions and may be considered a better option than tendon transfer, which has a one-tendon-one function. This narrative review article discusses the different distal nerve transfers for various presentations of BPNNI.

Chronic proximal ulnar collateral ligament injuries thumb-a case report.

A 55-year-old woman presented with multiple episodes of falls that injured her right thumb and restricted her daily activities because of pain and instability. Ultrasound found the rare proximal metacarpal UCL avulsion. The repair was done using suture anchors under wide-awake anaesthesia with no tourniquet (WALANT). The patient regained her thumb movements, got a stable MCP joint, and remained symptom-free at one year of follow-up. Proximal avulsion of the UCL is rare. Ultrasound confirms the avulsion, and surgical reconstruction under wide-awake anaesthesia allows the surgeon and the patient to assess and appreciate the MCP joint stability, thereby efficiently rehabilitating the patient. The functional outcome of proximal UCL repair is good. This report describes a rare presentation of the proximal metacarpal attachment avulsion injury of the ulnar collateral ligament (UCL) in a thumb and reviews the management.

Hemi-capitate Arthroplasty for Dorsal Fracture-Dislocations of the Proximal Interphalangeal Joint: Surgical Technique and Illustrative Case.

Osteochondral graft from the carpal bone allows anatomical joint reconstruction in unstable dorsal fracture-dislocations with > 50% of the articular surfaces. The most used graft is the dorsal hamate. Hemi-hamate arthroplasty is technically challenging and has anatomical incongruity, and many authors have evolved various modifications in the palmar buttress reconstruction of the middle phalanx base. Therefore, there are no universally accepted treatment modalities for these complex articular injuries. This article describes the dorsal capitate as the osteochondral graft for middle phalanx volar articular surface reconstruction. Hemi-capitate arthroplasty was done on a 40-year-old man with an unstable dorsal fracture dislocation of the PIP joint. The osteochondral capitate graft united well, and the joint congruency was good at the final follow-up. The surgical technique, illustrative images, and rehabilitation are discussed. With the evolving technical modifications and complications in Hemi-hamate arthroplasty, distal capitate may be considered a reliable and alternate osteochondral graft for unstable PIP joint fracture-dislocations. Supplementary Information: The online version contains supplementary material available at 10.1007/s43465-023-00853-2.

Wide-awake local anesthesia no tourniquet and dexamethasone (WALANT-D) for modified Camitz opponens plasty in severe carpal tunnel syndrome-a retrospective study of 30 cases.

Background: To analyze the outcome of modified Camitz opponens plasty using the wide-awake local anesthesia no tourniquet with dexamethasone (WALANT-D) technique in severe carpal tunnel syndrome (CTS). Methods: A retrospective review was performed in 30 hands of 27 patients who underwent Camitz opponens plasty for severe CTS between 2019 and 2021. All patients had 8 mg of dexamethasone mixed with WALANT. Preoperative active palmar abduction, grip strength, side, and pulp pinch strength, Kapandji score, and electrophysiological assessment of compound muscle action potential (CMAP) for abductor pollicis brevis (APB) were compared with the postoperative values. The palmaris longus had dual insertion into the abductor pollicis brevis and extensor expansion. The time interval of post-operative pain-free was noted. The Disabilities of the Arm, Shoulder, and Hand (DASH) and Carpal Tunnel Syndrome instrument (CTSI) also assessed the functional outcome. Results: The mean age of patients was 35 years (range 32-58 years). There were five male and 22 female patients. Of the female patients, three females had severe bilateral CTS. Twenty right and ten left hands were affected. The mean follow-up of our study was 12.5 months (range 10-14 months). The patients were pain-free for an average of 19.5 h postoperatively. There was a significant improvement in the thumb palmar abduction, grip strength, side, and pulp pinch strength, DASH score, CTSI, and APB-CMAP (p < 0.05) at the final follow-up. Conclusions: Modified Camitz opponens plasty with a dual insertion into APB and extensor expansion effectively improves thumb opposition and daily activities. The tendon tensioning, checking the pulley impingement, appreciation of active movements, and a comfortable patient operative experience are advantages of the WALANT. Adding dexamethasone as an adjuvant to WALANT prolongs the analgesia and duration of the nerve block. Level of evidence: IV, Retrospective case study.

Neuromyelitis optica spectrum disorders with a benign course. Analysis of 544 patients.

BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) most commonly cause severe disability which is related to disease attacks. However, some patients retain good neurological function for a long time after disease onset. OBJECTIVES: To determine the frequency, demographic and the clinical features of good outcome NMOSD, and analyze their predictive factors. METHODS: We selected patients who met the 2015 International Panel for NMOSD diagnostic criteria from seven MS Centers. Assessed data included age at disease onset, sex, race, number of attacks within the first and three years from onset, annualized relapsing rate (ARR), total number of attacks, aquaporin-IgG serum status, presence of cerebrospinal fluid (CSF)-specific oligoclonal bands (OCB) and the Expanded Disability Status Scale (EDSS) score at the last follow-up visit. NMOSD was classified as non-benign if patients developed sustained EDSS score >3.0 during the disease course, or benign if patients had EDSS score ≤3.0 after ≥15 years from disease onset. Patients with EDSS <3.0 and disease duration shorter than 15 years were not qualified for classification. We compared the demographic and clinical characteristics of benign and non-benign NMOSD. Logistic regression analysis identified predictive factors of outcome. RESULTS: There were 16 patients with benign NMOSD (3% of the entire cohort; 4.2% of those qualified for classification; and 4.1% of those who tested positive for aquaporin 4-IgG), and 362 (67.7%) with non-benign NMOSD, whereas 157 (29.3%) did not qualify for classification. All patients with benign NMOSD were female, 75% were Caucasian, 75% tested positive for AQP4-IgG, and 28.6% had CSF-specific OCB. Regression analysis showed that female sex, pediatric onset, and optic neuritis, area postrema syndrome, and brainstem symptoms at disease onset, as well as fewer relapses in the first year and three years from onset, and CSF-specific OCB were more commonly found in benign NMOSD, but the difference did not reach statistical significance. Conversely, non-Caucasian race (OR: 0.29, 95% CI: 0.07-0.99; p = 0.038), myelitis at disease presentation (OR: 0.07, 95% CI: 0.01-0.52; p <0.001), and high ARR (OR: 0.07, 95% CI: 0.01-0.67; p = 0.011) were negative risk factors for benign NMOSD. CONCLUSION: Benign NMOSD is very rare and occurs more frequently in Caucasians, patients with low ARR, and those who do not have myelitis at disease onset.

Extreme Oncoplastic Breast Surgery: A case series of three patients from a lower-middle income country.

Introduction: Oncoplastic breast surgery has become a major player in modern breast surgery.It broadens the indications for breast-conserving surgery. More challenging cases are being treated more with what so-called "extreme oncoplastic breast surgery" which is defined as a breast-conserving operation, using oncoplastic techniques, in a patient who, in most physicians' opinions, requires a mastectomy. Methods: Replacement and/or displacement oncoplastic techniques with contralateral symmetrization to three female patients with breast cancer were done by an oncoplastic breast surgeon. Outcomes: The three patients had smooth recoveries with good aesthetic,oncologic and psychological outcomes. Conclusion: Oncoplastic breast surgery can be a better option than mastectomy with good oncologic, Psychological and aesthetic outcomes, even with extreme cases, yet long-term studies are needed.

Adult embryonal rhabdomyosarcoma in the nasal cavity; a case report with a review of the literature.

Introduction: Rhabdomyosarcoma (RMS) is a primitive malignant soft tissue tumor arising from premature mesenchymal cells. The current study presents a rare case of embryonal rhabdomyosarcoma in the nasal cavity of an adult patient. Case presentation: An 18-year-old female presented with right nasal obstruction for five months. Examination showed a pale soft, painless mass in the right nasal cavity with attachment to the nasal septum at the osteocartilaginous junction. The patient was falsely suspected for bacterial infection, but later histological examination showed undifferentiated small round blue cell tumor with extensive necrosis. Immunohistochemistry confirmed the diagnosis of embryonal RMS. The patient was operated on for endoscopic sinus surgery to remove the mass with additional cleaning of surrounding sinuses. Discussion: Embryonal RMS is a rare type of malignant tumor that mostly affects the head and neck area in children while usually occur in the extremities of adults. Encountering an adult ERMS of the nasal chambers represents a small portion of head and neck cases that lack specific presentations. Conclusion: Adult embryonal RMS of the nasal cavity is exceedingly rare and presents a diagnostic and management challenge, with immunohistochemistry being the only definitive diagnostic method.

Transdural spinal cord herniation: A rare cause of neurologic impairment: Report of two cases and review of the literature.

INTRODUCTION: Herniation of the thoracic spinal cord is a rare pathology related to the genesis of a breach in the anterior dura. Adding to the previously published cases, we report our department's experience with 2 cases of young adults who presented with transdural spinal cord herniation following spinal trauma. PRESENTATION OF THE CASES: We report the cases of 2 men aged 27 and 57, with history of spinal trauma, who presented diverse clinical complaints (motor impairment, genito-sphincteric disorders, etc.). Spinal cord MRI showed an anterior displacement of the spinal cord. Both patients were operated. Peroperative findings and radiological features were concordant with transdural spinal cord herniation. DISCUSSION: Spinal cord herniation is a rare condition which is usually related to a misdiagnosed spinal trauma. Pathogenesis is still being debated. This condition may lead to a myelopathy potentially responsible for severe neurologic impairment, mainly presenting as a Brown-Sequard syndrome. Diagnosis is based on clinical and radiologic features on MRI. Surgical treatment is proposed when neurological signs are found. Long-term clinical and radiological monitoring is necessary for paucisymptomatic, non-operated patients. CONCLUSIONS: Based on the described cases, we tried to review the main clinical, radiological and therapeutic features related to this condition.

Appendiceal Schistosomiasis Presenting as an Appendiceal Mass.

Background: Schistosomiasis has been noted to be one of the most widespread parasitic diseases in the world. It is commonest in developing countries and 85% of the infections have been reported in Sub-Saharan Africa. Presentation with an appendiceal mass has been a rarity among the cases reported thus far. Case Presentation: We report the case of a 23-year-old man who presented with an appendiceal mass that responded to conservative treatment and had interval appendicectomy with histological confirmation of schistosomal appendicitis. He received post-operative medical therapy for schistosomiasis and had no sequelae thereafter. Conclusion: Appendiceal mass from schistosomal appendicitis responds to initial conservative management, followed by interval appendicectomy and post-operative anti-schistosomal medications serving as adequate treatment subsequently.

Treatment of Haemorrhoidal Disease by Laser Haemorrhoidoplasty at Dakar Preliminary Study of 21 Patients.

Background: Surgical treatment of haemorrhoidal disease by laser haemorrhoidoplasty is a minimally invasive procedure that facilitates the postoperative course. Due to less aggression on the anoderm and the anal canal mucosa, it causes less significant postoperative pain and low morbidity compared with conventional excision according to the Milligan-Morgan or Fergusson procedure. We report, through a preliminary study, our data on laser haemorrhoidoplasty and discuss the indications and results. Materials and Methods: This is a descriptive prospective study carried out on 21 patients operated on for haemorrhoidal disease by laser haemorrhoidoplasty with or without mucopexy. Results: The series was composed of 17 men and 4 women with a male/female ratio of 4.25:1. The average age was 39.6 years with a range of 27-62 years. The symptomatology was rectal bleeding in 16 cases (76%) and anal swelling in 18 cases (85.7%). These include grade 2 haemorrhoids in 2 cases (9.5%), grade 3 in 12 cases (57%), and grade 4 in 7 cases (33%). It was associated with an anal fissure in four cases (19%) and an anal fistula in three cases (14.2%). Mucopexy and laser coagulation were performed therapeutically in 13 cases (61.9%) and laser coagulation without mucopexy was performed in 8 cases (38%). The energy delivered was on average 1488 or 496 J per pile. It was associated with skin tag excision in 18 cases (85.7%), fissurectomy, sphincterotomy, anoplasty in 4 cases (19.2%), and fistulectomy for low anal fistula in 2 cases (9.5%). Piles retraction was judged sufficient in 17 patients (81%). The postoperative course was simple with no notable complaints in 16 patients (76%). Complications consisted of minimal bleeding in six cases (28%), significant bleeding in two cases with readmission, residual skin tag in six cases (28.5%), and subcutaneous fistula in two cases (9.5%). No recurrence of the symptoms of the haemorrhoidal disease was noted. Conclusion: Laser haemorrhoidoplasty is a minimally invasive alternative for the treatment of haemorrhoidal disease, especially for grade 2 and 3 haemorrhoids without major prolapse. Postoperative pain is minimal, and the risk of stenosis or incontinence is almost non-existent.

Modified Passerini-Glazel feminizing genitoplasty outcomes in adults: Two rare cases.

INTRODUCTION: Modified Passerini-Glazel feminizing genitoplasty is typically performed in children with atypical genitalia. In our article, we have performed the procedure in adults with genital anomalies. CASE PRESENTATION: The first case was a 22 years old woman who was planning to get married. She presented with a chief complaint of having no vaginal canal. Gynecological examination showed no vaginal opening. The common channel was visible, and the size of the perineal body was 3 cm. The patient underwent vaginal reconstruction using a modified Passerini-Glazel technique without amnion graft. The second case was a 20 years old girl planning to get married, with a chief complaint of small vaginal introitus. Gynecology examination showed small minor labia with an introitus size of 1 cm. The patients underwent vaginal reconstruction and labioplasty using a modified Passerini-Glazel technique with an amnion graft. Both patients have undergone anal atresia surgery in childhood. DISCUSSION: There was no difference in outcome between using amnion graft and without amnion graft following the modified Passerini-Glazel feminizing genitoplasty procedure. The first patient had been pregnant and had successful delivery by elective cesarean section. Moreover, the second patient had no complaint of sexual disorders after the procedure. No cases of dysuria, urinary tract infection, leukorrhea, hematocolpos, or malodorous vaginal discharge were reported in both cases. CONCLUSION: Modified Passerini-Glazel feminizing genitoplasty is a safe and effective procedure. Daily vaginal dilation in the postoperative period was unnecessary, and it allowed for an excellent cosmetic result.

Revisiting the Natural History of Chronic Scaphoid Nonunions: A Retrospective Study of 20 Cases.

Background The natural history of scaphoid nonunion is the development of degenerative arthritis. A lot of information is still unclear about this progression. The purpose of this study is to analyze patients with scaphoid nonunions who had not received any kind of treatment and to assess the functional outcome. Materials and Methods This is a retrospective study that analyzed the patients with chronic scaphoid nonunions between 2009 and 2019. None of the patients received any treatment. The age at the time of injury, examination, pattern of fracture, types of scaphoid nonunion, symptoms, and duration of nonunion were noted. Diagnosis was confirmed by radiographs, computed tomography (CT) scan, and magnetic resonance imaging (MRI). Scapholunate and radiolunate angles were recorded. Pain score, modified mayo wrist score, grip strength, range of movement, and the functional outcome of these scaphoid nonunions were analyzed. A statistical correlation between the scaphoid nonunion presentations and the functional outcome was assessed. Results The mean age of the patients was 62 years (range: 35-82 years.). There were 17 male and 3 female patients. There were 9 waist and 11 proximal pole scaphoid nonunions. The mean duration of scaphoid nonunion was 34 years (range: 10-62 years). None of the patients had avascular necrosis (AVN) of the proximal scaphoid. The age at examination, gender, side of injury, fracture pattern (waist/proximal pole), fracture displacement ≤ 1 mm or > 1 mm, nonunion duration, and radiographic arthritic parameters had no significant impact on the functional outcome. Conclusions Untreated chronic scaphoid nonunion leads to the development of degenerative arthritis over a period of years, which is still unpredictable. Most of the patients become aware of the nonunion following a precedent injury or other reasons. Most of the patients have fair/good functional outcome despite reduced range of movements and grip strength. Many do not favor surgical intervention in the course of nonunion. Chronic nonunions open a lot of unanswered questions. Clinical Relevance There have been numerous studies on the treatment aspects of scaphoid nonunion, with little knowledge about certain people with nonunion who did not have any kind of treatment. The demographics, clinical findings, and radiological parameters do confirm the progression of these nonunion to arthritis, but most of them had fair-to-good outcome throughout their life. It opens our thinking about the real need of treatment in such nonunions and raises numerous questions about the disease. Level of Evidence This is a Level IV study.