Alkaptonuria in an Elderly Presenting with Asynchronous Rupture of both Quadriceps Tendons and Chronic Kidney Failure: Case Report and Literature Review.

Introduction: Alkaptonuria (AKU) is a rare autosomal-recessive multisystemic disease. It is caused by a mutant homogentisate dioxygenase coding gene, leading to the accumulation of homogentisic acid (HGA), hence systemic manifestations. Renal manifestations and tendon rupture are rarely reported. Case Report: We report a 60-year-old male with chronic kidney disease for over a decade who was initially misdiagnosed with ankylosing spondylitis and rheumatoid arthritis. Later on, the patient presented to our institute with a non-synchronized (8 years) acute quadriceps tendon rupture. Conclusion: Physicians should be aware of the importance of prophylactic measures in the management of AKU, which is mainly medical management, to reduce the accumulation of HGA in the body. We further emphasize this point to reduce the incidence of subsequent tendon ruptures, as it significantly affects the quality of life.

Surgical Management of Traumatic Cervicothoracic Junction Spondyloptosis Without Neurological Injury: A Case Report and Review of the Literature.

Acute traumatic cervical spondyloptosis in neurologically intact patients is uncommon and involvement of the cervicothoracic junction is rare. Herein, we report a case of traumatic C7-T1 spondyloptosis in a 56-year-old neurologically intact male patient, with radiographic findings of C7-T1 grade V traumatic listhesis associated with C7 floating segment, cord compression with myelomalacia, extensive ligamentum injury, and intervertebral disc traumatic change and protrusion. He underwent global spine fixation starting with a posterior approach. Follow-up at six months showed good outcomes. The patient was neurologically intact and pain-free; radiographs showed well-maintained fusion and alignment. Controversy surrounds the management of cervical fracture dislocation from all aspects, from "when" to "what." This is the first case reporting a 540° posterior-anterior-posterior approach with successful outcomes. The rarity of cervical spondyloptosis without neurologic injury complicates the management approaches. As few cases are reported in cervicothoracic spondyloptosis literature, it is important to report the present case.

Prevalence and classification of accessory navicular bone: a medical record review.

BACKGROUND: The accessory navicular bone (ANB) is one of the most common accessory bones in the foot. Certain pathologies, such as posterior tibial tendon insufficiency are associated with ANB, and should be differentiated from midfoot and hindfoot fractures such as navicular tuberosity avulsion fractures. There are few studies addressing the prevalence and types of ANB in Saudi Arabia. OBJECTIVES: Determine the prevalence and morphological variations of ANB and its relation with age and sex in patients visiting foot and ankle clinics. DESIGN: Medical record review SETTING: Orthopedic foot and ankle clinic at a university hospital. PATIENTS AND METHODS: The presence of ANB was retrospectively analyzed in radiographs from patients who presented to the orthopedic foot and ankle at our university hospital from February 2010 to December 2020. The patients were stratified according to sex, age, and diagnosis. For each ANB, recorded information included site, size, classification, subtypes, and symptomatology. Purposive sampling was used to select the patients for the study (non-probability sampling). MAIN OUTCOME MEASURES: Prevalence of ANB in patients attending a foot and ankle clinic. SAMPLE SIZE: 117 patients and 194 feet. RESULTS: ANB was analyzed in 1006 radiographs from 503 patients. ANB was detected in 117 (23.3%) patients and 194 (19.3%) feet Prevalence was significantly higher in females (67.5%) than in males (32.5%) (Z=5.359, P<.001). The ages ranged from 19 to 86 years, with a mean age of 48.26 (14.5) years. The most common site was bilateral (77 patients, 65.8 %). Type I was the most common type, with a prevalence of 42.1%. There were no significant differences in types in relation to sex, but all types and subtypes differed significantly from each other. CONCLUSION: ANB was common among patients presenting to the foot and ankle clinic, with an overall prevalence of 23.3%. It should be considered among the differential diagnosis in chronic foot pain, and should be differentiated from midfoot and hindfoot fractures. Further studies with a larger, randomized sample are needed, for more accuracy and to confirm the reported results. LIMITATIONS: Retrospective chart review, non-probability sampling, and use of plain radiographs. CONFLICT OF INTEREST: None.

Rare Constellation of Pelvic Injuries: A Case Report.

Crescent fracture-dislocations are sunset of lateral compression injuries. They can be associated with vascular, bowel, genitourinary, or soft tissue degloving injuries. Here, we describe a patient with bilateral crescent fracture-dislocation, right common iliac artery (CIA) injury, and an extensive Morel-Lavallée lesion (MLL). A 35-year-old male was transferred to our hospital after being involved in a motor vehicle collision with an unknown mechanism. Upon evaluation, four rare injuries were found: right CIA injury, bilateral open iliac bone fracture, bilateral crescent fracture-dislocation, and an extensive MLL. The patient underwent successful right CIA thrombectomy and stenting, followed by irrigation and debridement of MLL and open pelvic fractures. However, his hospital course was complicated by septic shock with spontaneous rectal perforation, necessitating massive transfusion protocol activation and three relook laparotomies. Unfortunately, the patient died after 25 days of a turbulent hospital course. The combination of bilateral crescent fracture-dislocation, bilateral open pelvic fracture, CIA injury, and an extensive MLL is exceedingly rare. Each poses a challenge when encountered alone, nevertheless, after establishing hemodynamic stability, timely intervention is crucial to avoid possible morbidity and mortality.

Outcome of Vertical Expandable Prosthetic Titanium Rib (VEPTR) Instrumentation in Scoliosis Associated With 1p36 Deletion Syndrome: A Case Report.

Monosomy 1p36 deletion is a rare syndrome that consists of developmental delay, intellectual disability, seizures, hearing and vision defects, brain anomalies, orofacial clefting, congenital heart defects, cardiomyopathy, renal anomalies, and scoliosis. We report the case of an eight-year-old boy who presented to the orthopedic clinic with spinal deformity with a background of 1p36 deletion syndrome. The treatment modalities at this age include growing rods, vertical expandable prosthetic titanium rib (VEPTR), or posterior spinal fusion. Keeping in view the challenges in this case due to multi-organ involvement and severe intellectual disability, we decided to manage this patient with a VEPTR device to prevent the progression of scoliosis and allow spinal growth. Vertical expandable prosthetic titanium rib (VEPTR) instrumentation for progressive scoliosis in p36 deletion syndrome is an effective mode of treatment and leads to favorable outcomes.

Is It Compartment Syndrome? Two Case Reports and Literature Review.

Recently, a unique entity of acute compartment syndrome (ACS) has been termed "silent" compartment syndrome. These patients develop ACS in the absence of classic pain and physical findings. We report two cases of posttraumatic silent ACS in two healthy competent adult patients presenting mainly with swollen tense compartments. In the first case, ACS was suspected in a 37-year-old male with left tibia and fibula shaft fractures after reassessment of the patient's post-backslap application, which revealed painless swollen and tense compartments. In the second case, ACS suspicion was raised in a 27-year-old male with right comminuted tibial plateau fracture and a swollen but soft compartment that became tense over time. In both cases, intraoperative intracompartmental pressure testing during external fixator application confirmed the diagnosis, and both underwent emergent fasciotomy with good postoperative outcomes. The absence of pain does not exclude the diagnosis of ACS. Physicians must have a high index of suspicion when risk factors are present for ACS, and the diagnosis can be confirmed with intracompartmental pressure measurement. These case reports and literature review aim to enlighten the physicians about silent compartment syndrome.

Sinus Pericranii Complicated by Hydrocephalus: Case Report and Literature Review.

BACKGROUND: Sinus pericranii (SP) is a rare vascular condition that results when abnormal communication between the intracranial and extracranial venous systems is present. Here we report a rare case of hydrocephalus revealed through a SP scalp mass with a review of literature. CASE DESCRIPTION: A 13-year-old girl presented with a history of bulging left eye, bilateral gradual deterioration of vision, nausea, and progressive headaches. The family reported the sudden appearance of a scalp mass prior to these symptoms. Brain imaging showed supra- and infratentorial communicating hydrocephalus and subcutaneous vascular collaterals from the midportion of the superior sagittal sinus. After ventriculoperitoneal shunt insertion, interval reduction of the ventricular size and disappearance of extracranial veins without associated intra- or extracranial vascular anomalies was observed. CONCLUSIONS: SP is divided into two main types: (1) Spontaneous type, in which lesions are formed due to acquired causes without a syndromic association or other vascular anomalies or stenosis. The most commonly reported cause is trauma in which SP may develop at the trauma site rather than the midline. (2) Congenital type, in which case the most commonly reported association is craniosynostosis. It can also be seen with vein of Galen hypoplasia, vein of Galen malformations, dural sinus malformations, solitary developmental venous anomalies, and intraosseous arteriovenous malformations. We are reporting the first case of idiopathic SP with hydrocephalus without a congenital association or acquired cause, including trauma. The sudden change in SP size can indicate a change in intracranial pressure and the development of hydrocephalus.

Patterns and prognosis of ventriculoperitoneal shunt malfunction among pediatrics in Saudi Arabia.

OBJECTIVE: To examine the predictors of pediatric ventriculoperitoneal (VP) shunt malfunction in a university hospital. METHODS: A retrospective cohort was conducted. Patients under 18 years old who underwent VP shunt revision at least once between 2016 and 2019 were included. Data were stratified based on age, gender, diagnosis, type of valve, valve position, cause of revision, and part revised. RESULTS: A total of 45 patients (64% males and 36% females) were included in this study. Eighty-two revision surgeries were identified. The most common revised part was the entire shunt system. The most common type of valve which required revision was the low-pressure valve (15.5%). Since a p-value of less than 0.05 was considered significant, no significant differences among the 4 groups for different points. CONCLUSION: Younger age at initial VP shunt insertion is associated with a higher rate of shunt malfunction. Valve mechanical failures followed by infections are the most common causes for the first 3 revisions. A prospective multi-center study to confirm the current findings is recommended.