Can we predict urethral length of boys? - An observational study.

INTRODUCTION: An additional knowledge about normal anatomy of urethra in boys can improve clinical outcome of any urological interventions. This will also reduce catheter related complications like intra vesical knotting and urethral injuries. At present there is no systematic data available which has studied the urethral length of boys. We have done this study to analyse the length of urethra in boys. AIM: The aim of the study is to measure the urethral length in Indian children from the age of 1 year till 15 years and plot a nomogram. Furthermore to analyse the influence of anthropometry on urethral length and derived a formula to predict the urethral length in boys. STUDY DESIGN: This is a single institution prospective observational study. After obtaining clearance from the institutional review board, a total of 180 children from the age of 1-15 were recruited for the study. The urethral length was measured during Foley's catheter removal. Other data like age, weight, and height of the patient are collected and the obtained values were analysed with SPSS software. The acquired figures were further used to derive formulae to predict urethral length. RESULTS: A nomogram of urethral length based on age was plotted. With collected figures, five separate formulae were derived to calculate the urethral length based on the age, height and weight. Furthermore for day-to-day use we have deduced a formulas to calculate urethral length which are simplified forms of original formulae. DISCUSSION: The length of urethra of a new-born male is 5 cm, which attains 8 cm by three years of age and to 17 cm by adulthood. There were attempts to measure the urethral length in adults using cystoscopy, Foley's catheter and different imaging techniques like Magnetic Resonance Imaging and dynamic retrograde urethrography. Simplified formulae which has been derived from this study for clinical use is Urethral length = 8.7 + Age (in year) × 0.55 CONCLUSION: Our results will complement the current anatomical understanding of the urethra. This evades some rare complications of catheterisation and facilitate reconstructive procedures.

Patient-Specific Targeting of the T-Cell Receptor Variable Region as a Therapeutic Strategy in Clonal T-Cell Diseases.

PURPOSE: Targeted therapeutics are a goal of medicine. Methods for targeting T-cell lymphoma lack specificity for the malignant cell, leading to elimination of healthy cells. The T-cell receptor (TCR) is designed for antigen recognition. T-cell malignancies expand from a single clone that expresses one of 48 TCR variable beta (Vß) genes, providing a distinct therapeutic target. We hypothesized that a mAb that is exclusive to a specific Vß would eliminate the malignant clone while having minimal effects on healthy T cells. EXPERIMENTAL DESIGN: We identified a patient with large granular T-cell leukemia and sequenced his circulating T-cell population, 95% of which expressed Vß13.3. We developed a panel of anti-Vß13.3 antibodies to test for binding and elimination of the malignant T-cell clone. RESULTS: Therapeutic antibody candidates bound the malignant clone with high affinity. Antibodies killed engineered cell lines expressing the patient TCR Vß13.3 by antibody-dependent cellular cytotoxicity and TCR-mediated activation-induced cell death, and exhibited specific killing of patient malignant T cells in combination with exogenous natural killer cells. EL4 cells expressing the patient's TCR Vß13.3 were also killed by antibody administration in an in vivo murine model. CONCLUSIONS: This approach serves as an outline for development of therapeutics that can treat clonal T-cell-based malignancies and potentially other T-cell-mediated diseases. See related commentary by Varma and Diefenbach, p. 4024.

Functional outcomes in children with reduction glossectomy for vascular malformations - "less is more!"

OBJECTIVES: Vascular malformation (VM) of the tongue can cause true macroglossia in children. Reduction glossectomy provides primary relief when sclerotherapy has failed or is not possible. In this study, we evaluated the surgical role in functional outcome of reduction glossectomy performed for VM of the tongue. PATIENTS AND METHODS: : We evaluated the functional and surgical outcomes of seven children who were treated at a tertiary care centre in Southern India between 2013 and 2018. RESULTS: : Six children underwent median glossectomy, while one child underwent lateral glossectomy. Functional assessment was performed at least 2 years after the date of surgery. At the time of assessment, speech was comprehensible for three children and was occasionally unintelligible in four children. Taste and swallowing were normal in all seven children. Six children exhibited a minimal residual lesion after surgery, of which only one was symptomatic. Residual lesions were managed with sclerotherapy (n=3), observation (n=2), or repeat surgery (n=1). CONCLUSION: Reduction glossectomy in children with macroglossia secondary to VMs has acceptable outcomes in terms of cosmesis and speech, with no gastronomic restriction.

Working classification and the quest for an effective, reconstructive management strategy in Y duplication of male urethra.

INTRODUCTION: Y-duplication is a rare subtype of urethral duplication whose surgical correction has traditionally yielded less than satisfactory results. Herein we report 18 patients, 17 of whom have completed successful urethral reconstruction. MATERIALS AND METHODS: A retrospective analysis was done on 18 children who have undergone repair of Y-duplication urethra in two tertiary care institutes from 2013 to 2020. The various subtypes encountered were classified in order to develop a rationale for reconstruction based on the underlying pathology. The various modalities used for reconstruction and the outcomes of the repair were studied. All but one boy voided mainly via the posterior (ventral) channel opening in the ano-rectum or perineum. These boys had the urethral reconstruction incorporating the healthy proximal ventral urethra at its origin. Further reconstruction up to the glans tip depended on the degree of dorsal (orthotropic) urethral patency, which forms the basis of our classification into Types I (completely stenotic), II (penile urethra patent), III (penile and bulbar urethra patent) and IV (fully patent dorsal urethra). RESULTS: Reconstruction resulting in voiding via a glanular or coronal meatus was completed in 17 boys. One boy is awaiting further surgery while being dependent on Mitrofanoff CIC. While urethral continuity could be achieved using patent segments of native urethra alone into two boys (Types II and III), all other children needed a neo-urethral segment to replace (Type I) or supplement (Type II) the dorsal urethra. Neo urethra was constructed from a tubularized preputial island flap (n = 11), Monti tube constructed from ileum (n = 3) or sigmoid colon (n = 4). A perineal operative exposure alone sufficed in 9, while the rest required an additional trans pubic approach. The only boy with Type IV anomaly underwent excision of the ventral urethral limb. Three boys with initially elevated serum creatinine have normal levels after reconstruction. CONCLUSION: Successful reconstruction of boys with Y- duplication of the urethra entailed elucidation of anatomical variations, adequate operative exposure (trans pubic and/or perineal) and innovative utilisation of local and/or distant tissues (preputial island flap, ileal/sigmoid Monti tubes).

Papillary Thyroid Carcinoma in Children: Clinicopathological Profile and Outcomes of Management.

BACKGROUND: We aim to analyze the clinicopathological profile and outcomes of management for children with papillary thyroid carcinoma (PTC). METHODS: Relevant clinical data of children ≤ 18 years of age managed for PTC between January 2006 and July 2018 as well as details of their follow-up till December 2019 were retrospectively collected and analyzed. RESULTS: There were 82 children with PTC that were managed during the study period. At presentation, 39 (47.6%) had cervical lymphadenopathy, while 9 (11%) had systemic metastasis. Majority of patients 39 (47.6%) underwent total thyroidectomy with a selective neck dissection, while total thyroidectomy alone was performed in 26 (31.7%). Following surgery, hypocalcemia was seen in 39 (47.6%): 28 (34.1%) were temporary, while 11 (13.4%) were permanent. Twenty-eight (34%) developed persistent disease after surgery and 131I therapy. Significant risk factors for persistence and metastatic disease were metastatic cervical lymph node at presentation (p = 0.002) and tumor size (p = 0.014), respectively. The mean duration of follow-up was 60.3 (range 12-150) months with a mean overall disease-free survival of 60 months (95% CI 57.11, 77.95). CONCLUSION: Children with papillary thyroid cancers present with aggressive disease, 47.6% with cervical nodal metastasis and 11% with distant metastasis in this cohort. The rate of post-thyroidectomy hypocalcemia in this study is substantial, and efforts to reduce it are actively being pursued. The presence of metastatic cervical lymph node at presentation (p = 0.002) and tumor size (p = 0.014) were the only significant risk factors for persistent and metastatic disease, respectively, in this study.

Long term outcome of cochlear implantation in five children with common cavity deformity.

INTRODUCTION: A common cavity (CC) deformity is a deformed inner ear in which the cochlea and vestibule are confluent forming a common rudimentary cystic cavity. The outcome of cochlear implantation (CI) in this deformity is not expected as good as in other cochlear deformity. Categories of Auditory Performance (CAP) score, Speech Intelligibility Rating (SIR) scale, Meaningful Auditory-Integration Scale (MAIS) and Meaningful Use of Speech Scale (MUSS) are scales designed to assess benefit of functional device (hearing aid, Cochlear implant, ABI) in young children. The aim of the study was to observe and report the long-term audiological progress and speech development of children with CC deformity after CI by means of CAP, SIR, MAIS, MUSS scores and CI aided threshold. METHODS AND MATERIALS: The retrospective and prospective study was carried out in five Indian children with CC deformity who underwent CI from 2004 to 2010. Demographic data of onset and/or duration of deafness, age at implantation, time with hearing aids before implantation, duration with CI and co-morbidity were retrieved from a local data base. Their outcome score in audition and speech development on the basis of their CAP and SIR score at the end of 1 year of habilitation were collected. All the patients were called to follow up to obtain latest CI aided audiogram, CAP, SIR, MAIS and MUSS scores. RESULTS: One male and four female children were detected with common cavity deformity among 258 deaf children of age below 6 years operated during study period. The CAP and SIR Scores increased from average of 0.4 and 1 to 3.4 and 2.4, respectively, over one year of habilitation. The CAP score improved in subsequent follow up to average of 5.0 after more than 3 years of follow up but the SIR score hardly improved. We assessed the MAIS and MUSS at last visit after 3 years. The mean of MAIS and MUSS was 32.4 and 26.4, respectively. CONCLUSION: Though the habilitation outcome was not up to the mark in children with CC deformity, CI provides excellent awareness of environment sound and development of few words.

Metaphyseal bands in osteogenesis imperfecta.

An increasing number of patients with osteogenesis imperfecta are undergoing pamidronate therapy to prevent the incidence of fragility fractures. The authors herein report a child aged 3 years who received five cycles of pamidronate, resulting in metaphyseal bands, known as "zebra lines."

Migrating transient osteoporosis of the hip in a 30-year-old man.

Transient osteoporosis of hip is a condition of unknown etiology, presenting as painful limping, and characterized by osteopenia of the involved joint without preexisting disease or immobilization. Most of the cases were reported in middle-aged men, and one-third of the cases develop in women in the third trimester of pregnancy. The hypothesis that this condition leads to avascular necrosis of the hip has been disproved by various reports and hence does not warrant any surgical interference. This is a self limiting condition, which needs regular follow-up. The authors report a case of migrating transient osteoporosis of the hip in a 30-year-old man successfully treated with antiresorptive treatment.