Treatment Outcome of Tardy Ulnar Nerve Palsy Associated with Traumatic Cubitus Valgus by Supracondylar Shortening Wedge Rotary Osteotomy and Ulnar Nerve in Situ Tension Release

Background Tardy ulnar nerve palsy is a common late complication of traumatic cubitus valgus. At present, the treatment of tardy ulnar nerve palsy associated with traumatic cubitus valgus is still controversial, whether these two problems can be corrected safely and effectively in one operation is still unclear. To investigate the supracondylar shortening wedge rotary osteotomy combined with in situ tension release of the ulnar nerve in the treatment of tardy ulnar nerve palsy associated with traumatic cubitus valgus. Methods Between 2012 and 2019, 16 patients who had traumatic cubitus valgus deformities with tardy ulnar nerve palsy were treated with simultaneous supracondylar shortening wedge rotary osteotomy and ulnar nerve in situ tension release. we compared a series of indicators of preoperative and postoperative follow-up for at least 24 months, (1) elbow range of motion; (2) the radiographic correction of the preoperative and postoperative humerus-elbow-wrist angles; (3) the static two-point discrimination and grip strength; and (4) the preoperative and postoperative DASH scores of upper limb function. The minimum follow-up was 24 months postoperative (mean, 33 months; range, 24ཞ44 months). Results The mean ROM was improved from 107 ° preoperatively to 122 ° postoperatively (P = 0.001). The mean preoperative elbow wrist angle was 24.6 °, and the mean postoperative humerus-elbow wrist angle was 12.1 ° (P < 0.001). The average grip strength and static two-point discrimination improved from 28 kg force and 8 mm to 21 kg force and 4.0 mm (P < 0.001 and P < 0.001, respectively). The ulnar nerve symptoms were improved in all patients except one. The mean HASH score improved from 29 to 16 (P < 0.001). Conclusion Supracondylar shortening wedge rotary osteotomy combined with in situ tension release of ulnar nerve is an effective method for the treatment of traumatic cubitus valgus with tardy ulnar nerve palsy, which restored the normal biomechanical characteristics of the affected limb and improved the elbow joint function.

Decoding enigma: Turner syndrome with ring chromosome

ABSTRACT Ring chromosome X is one of the rarest with some unique phenotypical features in Turner syndrome. A young female presented to us with anasarca developed over the past 2 months due to congestive cardiac failure along with jaundice and orthopnea. She had growth retardation, intellectual disability, primary amenorrhea, lack of secondary sexual character development and dysmorphic features like low posterior hairline, shield chest and cubitus valgus. She had dilated cardiomyopathy (DCM) with intracardiac thrombus on echocardiography. Skeletal survey revealed short fourth metacarpal/tarsal on limbs. Karyotyping showed a mosaic pattern, with 45, X/46, X,r(X)(p22.3q28), i.e. Turner syndrome karyotype with ring chromosome. Her heart failure with reduced ejection fraction was managed with vasopressor along with anticoagulant and given oral contraceptive pills for hormone replacement therapy. The ring chromosomal pattern of karyotype in this patient and DCM is a rare cardiological phenomenon that can be associated with Turner syndrome, making this case a unique one.

Prenatal Diagnosis of Holt–Oram Syndrome With a Novel Mutation of TBX5 Gene: A Case Report

Background: Holt–Oram syndrome (HOS) is an autosomal dominant disorder caused by mutations of TBX5 gene.Case presentation: We report a fetus with HOS diagnosed sonographically at 23 weeks of gestation. The fetal parents are non-consanguineous. The fetus exhibited short radius and ulna, inability to supinate the hands, absence of the right thumb, and heart ventricular septal defect (VSD), while the fetal father exhibited VSD and short radius and ulna only. Fetal brother had cubitus valgus and thumb adduction, except for VSD, short radius and ulna. The pregnancy was terminated. Whole-exome sequencing (WES) revealed a novel mutation in the TBX5 (c.510+1G&gt;A) in the fetus inherited from the father. The variant (c.510+1G&gt;A) occurs at splice donor and may alter TBX5 gene function by impact on splicing. It was not previously reported in China.Conclusion: Our case reported a novel mutation in TBX5, which expanded the known genetic variants associated with HOS.

Congenital Anterior Radial Head Dislocation with Valgus Deformity: A Case Report

Congenital radial head dislocation is a rare anomaly of the pediatric musculoskeletal system. The most common type is posterior, with anterior and lateral dislocations being less common. Unilateral cases are even more uncommon and were earlier thought to be non-existent. Our case report describes a young girl with unilateral congenital anterior radial head dislocation with progressive development of cubitus valgus deformity, which is a very rare occurrence. On reviewing literature, only few such cases have been described. Our patient also had a history of developmental dysplasia of hip and a congenital intra-hepatic porto-systemic shunt, which in addition to the morphological appearances of the radial head and capitellum, suggested a congenital etiology.

The effect of staged surgical treatment for cubitus valgus after non-union of lateral condylar fracture of distal humerus in older children

Background: The purpose of this study was to investigate the effect of staged surgery (open reduction/internal fixation and osteotomy) for cubitus valgus after non-union of lateral condylar fractures of the distal humerus in older children.Methods: From January 2010 to January 2013, 9 patients were treated with two-staged surgery (open reduction/internal fixation and osteotomy). The study included 5 males and 4 females, with a mean age of 12.7 years. The interval from fracture to the first surgery was 8.2 years on average. All patients had symptoms of injury of the ulnar nerve and instability of the elbow. The first surgery included internal reduction, internal fixation, and bone grafting, exposing the elbow through a lateral approach. The procedure included clearing the peripheric callus and proximal distal fracture end cicatrix with rongeur until cancellous bone was exposed, and fixation of the lateral condylar fragment with a hollow screw 4.0 mm in diameter and smooth Kirschner wire. The limb was immobilized in a long arm cast with the elbow at 90 degrees of flexion and the forearm in neutral rotation for 3 weeks, and active exercises were begun after removal. The second surgery, osteotomy of the supracondylar humerus, was completed after 6 months to correct cubitus valgus. Internal fixation from the osteotomy was removed 6 months later.Results: Six months after the second surgery, follow-up revealed that in all patients the lateral condylar fractures attained clinical union and cubitus valgus was corrected. Elbow function recovered well without arthrochalasis or humeral condylar avascular necrosis. All patients’ ulnar nerve injury symptoms disappeared.Conclusion: Staged surgery to treat cubitus valgus secondary to lateral condylar fracture non-union in older children serves to first provide elbow stability, then to correct cubitus valgus. Staged treatment may make up for the deficiencies of conventional treatment. However, due to our relatively short follow-up time, the long term effects are unknown.

The Effect of Staged Surgical Treatment for Cubitus Valgus After Non-Union of Lateral Condylar Fracture of Distal Humerus in Older Children

Background: The purpose of this study was to investigate the effect of staged surgery (open reduction/internal fixation and osteotomy) for cubitus valgus after non-union of lateral condylar fractures of the distal humerus in older children.Methods: From January 2010 to January 2013, 9 patients were treated with two-staged surgery (open reduction/internal fixation and osteotomy). The study included 5 males and 4 females, with a mean age of 12.7 years. The minimum interval from fracture to the first surgery was 7 years and the maximum interval was 10 years (average 8.2 years). All patients had symptoms of injury of the ulnar nerve and instability of the elbow. The first surgery included internal reduction, internal fixation, and bone grafting, exposing the elbow through a Kocher lateral approach. The procedure included clearing the peripheric callus and proximal distal fracture end cicatrix with rongeur until cancellous bone was exposed, and fixation of the lateral condylar fragment with a hollow screw 4.0 mm in diameter and smooth Kirschner wire. The limb was immobilized in a long arm cast with the elbow at 90 degrees of flexion and the forearm in neutral rotation for 3 weeks, and active exercises were begun after removal. The internal fixation hardware was removed after 3 months. The second surgery, osteotomy of the supracondylar humerus, was completed after 6 months to correct cubitus valgus. Internal fixation from the osteotomy was removed 6 months later.Results: Six months after the second surgery, follow-up revealed that in all patients the lateral condylar fractures attained clinical union and cubitus valgus was corrected. Elbow function recovered well without arthrochalasis or humeral condylar avascular necrosis. All patients’ ulnar nerve injury symptoms disappeared.Conclusion: Staged surgery to treat cubitus valgus secondary to lateral condylar fracture non-union in older children serves to first provide elbow stability, then to correct cubitus valgus. Staged treatment may make up for the deficiencies of conventional treatment. However, due to our relatively short follow-up time, the long term effects are unknown.

Pregnancy in a Patient With Mosaic Turner Syndrome: A Case Report

Turner Syndrome (TS) is a chromosomal sex disorder, phenotypically characterized by short stature, webbed neck, cubitus valgus, and rarely with slight intellectual disability. A majorityof TS patients (95%-98%) have infertility due to ovarian failure. Pregnancy in TS patients is an unusual case; however, pregnancy could rarely occur in mosaicism TS patients withoutany assistance. Pregnancy in such patients is associated with high risks of maternal mortality, spontaneous abortion, as well as the congenital and karyotype abnormalities of the fetus. A30-year-old pregnant woman has referred to our genetics lab with a history of polyabortivity. Her menarche occurred at the age of 13 years and her menstruation was claimed to be in aregular cycle. The patient’s two first pregnancies resulted in stillbirth, whereas the third one was delivered through caesarian surgery, but spoiled after 8 days. Our case was characterizedby mosaicism 45, X/45, XX, after referring as a multi-abortion case. The fourth pregnancy has happened at the age of 31 years and a healthy embryo with normal heart function wasdiagnosed by sonography in 17 weeks of gestation. The result of amniocentesis confirmed a healthy female embryo with 46, XX karyotype. Spontaneous pregnancy is regarded as aprecarious situation terrifying by abortion or malignancy; also, chromosomal abnormalities, like trisomy 21 and TS, are prevalent in offspring. Therefore, it is strongly recommended tohave cohort studies based on karyotype characterization to decrease the patient’s concerns as well as to follow more practical clinical approaches.