In patients with severe glenohumeral dysplasia, glenoid realignment osteotomy in conjunction with soft-tissue rebalancing permits maintenance of joint reduction and functional improvement in the short term. In our view, external rotation osteotomy of the humerus is no longer the only surgical option for these cases.

Septic Arthritis of the wrist is rare in the paediatric population due to its extraarticular metaphysis. We report here a case of wrist septic arthritis in a neonate caused by an uncommon causative organism, Streptococcus cristatus.A 15 days old male child was referred with the complaint of swelling and decreased movement of the left wrist for 5 days. Local examination revealed warm, tender, erythematous and fluctuant swelling over the dorso-ulnar aspect of the left wrist. Ultrasonography of the affected region was suggestive of focal fluid collection in the wrist and periosteal elevation of the distal ulna. Aspiration followed by arthrotomy of the wrist joint was performed and multiple holes were made in the distal ulnar metaphysis using 0.8mm k-wire. The pus culture was positive for Streptococcus cristatus, sensitive to vancomycin, which was given for a total of 4 weeks. At one year follow up the child had a full, painless range of motion with no functional deficit. Final follow up x rays of the left wrist were normal.Streptococcus cristatus strains are described as Gram-positive, catalase-negative cocci, approximately 1 mm in diameter growing in chains and were originally isolated from the human throat and oral cavities.Its association with bone and joint infections has not been described in the literature. To our knowledge, this is the first case of isolated septic arthritis of wrist in a 15 days old child caused by Streptococcus cristatus.To conclude, wrist septic arthritis in a neonate is a rare entity. With the advanced diagnostics, specieslevel identification of rare organism like Streptococcus cristatus is possible along with antibiotic sensitivity for appropriate therapy. Early surgical decompression and intravenous culture-directed antibiotics are the mainstays of management.

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Background: Fractures around the shoulder region in infants are treated by arm-chest bandaging, adhesive strapping, or sling application. These conventional treatment methods are associated with issues like skin irritation, excoriation, movement of bone fragments causing muscle spasm, and difficulty in nursing care. We describe the technique of modified Velpeau sling application and reviewed its efficacy at a long-term follow-up. Methods: A retrospective study was conducted with 19 infants who presented between 2009 and 2018 to a tertiary care center in western India with either clavicle or humerus fracture. Inclusion criteria was any infant with humerus or clavicle fracture that underwent modified Velpeau sling application and had a minimum follow-up of 2 years. Patients were followed at weekly intervals until sling discontinuation based on the fracture healing. At further follow-up, children were assessed for the presence of angular or rotational malalignment and limb length discrepancy. Functional outcome was measured by the Paediatric Adolescent Shoulder Survey at the final follow-up. Results: Seven clavicle and 12 humerus fractures were treated with this technique. The mean age at presentation was 50 days (range, 1 d to 7 mo). The average follow-up was 6 years (range, 2 to 10 y). Two infants had underlying osteogenesis imperfecta. Infants without underlying pathology had no angular/rotational malalignment or limb length discrepancy, while both infants with underlying osteogenesis imperfecta had a varus alignment and shortening of the humerus. Infants without underlying pathology demonstrated good functional outcomes measured by the Paediatric Adolescent Shoulder Survey questionnaire, while those with pathology had functional limitations. Conclusions: The modified Velpeau method is an effective way of treating fractures around the shoulder region in infants without underlying pathology. Several advantages such as accessibility of sling material, easy application, inexpensive material, and absence of skin-related complications make this an effective treatment. Level of Evidence: Level IV—therapeutic study.

Background Mucopolysaccharidosis IVA (Morquio syndrome A, MPS IVA) is an autosomal recessive lysosomal storage disorder caused due to biallelic variants in the N-acetylgalactoseamine-6-sulfate sulfatase (GALNS) gene. The mutation spectrum in this condition is determined amongst sub-populations belonging to the north, south and east India geography, however, sub-populations of west Indian origin, especially Gujarati-Indians, are yet to be studied. We aimed to analyse the variants present in the GLANS gene amongst the population of Gujarat by sequencing all exons and exon–intron boundaries of the GALNS gene in patients from 23 unrelated families. Results We report 11 variants that include eight missense variants: (p.L36R, p.D39G, p.P77R, p.C79R, pP125L, p.P151L, p.G255A and p.L350P), one splice site variant: (c.121-7C > G), one small insertion: (c.1241_1242insA, p.I416HfsTer2) and one small deletion: (c.839_841delACA). Of these, three missense variants (p.D39G, p.G255A and p.L350P), one splice site and the two indels mentioned above are novel. Interestingly, we observed a higher than anticipated prevalence of p.P77R variant in our cohort (n = 14/25, 56%). Haplotype analysis in cases with p.P77R variant and 63 ethnicity matched healthy population controls suggested a 4 SNP haplotype block present in cases compared to controls (likelihood ratio test p-value = 1.16 × 10–13), thereby suggesting p.P77R variant as a founder variant in the Gujarati-Indian population. Furthermore, age of mutation analysis suggested the variant to have arisen approximately 450 years ago in the population. Conclusion p.P77R variant in the GLANS gene is likely to be a founder variant in MPS IVA patients of Gujarati-Indian ancestry and appeared approximately 450 years ago in the population. To our knowledge, this is the first variant to be posited as a founder variant in the GLANS gene in patients with MPS IVA syndrome.