Anand Alladi

Ectopic testis in children: Experience with seven cases

BACKGROUND Ectopic testis is a rare congenital anomaly in which the testis is abnormally located away from normal line of decent. AIM To report varied clinical presentation, embryogenesis, and management aspects of ectopic testis with a brief review of the literature. MATERIALS AND METHODS A retrospective chart review of children with undescended testis from January 2008 to August 2011. RESULTS Seven children (3.6%) treated for ectopic testes were diagnosed among 190 children operated on for undescended testis. There were five perineal testes, one penile testis, and one transverse testicular ectopic testis. Laparoscopy was the diagnostic and therapeutic modality in transverse testicular ectopia. Other ectopic testes were managed by open orchidopexy. The length of the testicular vessels and vas deferens was adequate in every case. CONCLUSION Examination of boys with an empty scrotum should include examination of ectopic sites as well. The gubernaculum bulb has preprogrammed growth toward the scrotum unless anatomical blockade prevents its descent. Open orchidopexy reveals normal characteristics of perineal, penile testis, and its elements. Surgical correction for ectopic testis as early as possible facilitates proper psychological development and prevents complications.

Surgical complications of Ascaris lumbricoides in children

Aim: To report the surgical complications of Ascaris lumbricoides infestation in children. Materials and Methods: This is a retrospective study and cases of intestinal ascariasis managed conservatively were excluded. Results: Sixteen children presented with Ascariasis sequelae, which included ileal volvulus (n=5), perforations (n=4), intussusception (n=1), biliary ascariasis (n-1) and impacted multiple worm boluses (n=5). Plain abdominal radiographs showed pneumoperitoneum (3), cigar bundle appearance (3) and multiple air and fluid levels (13). Sonography showed floating worms with free fluid (2), sluggish peristalsis and moderate free fluid (7) and intestinal worm bolus (11). The surgical procedures included milking of worms (in all), bowel resection (6), closure of perforation (3) and manual reduction of intussusception (1). Biliary ascariasis was managed conservatively and the progress monitored with sonography. There were 3 deaths all of whom had intestinal volvulus, bowel necrosis and toxemia. Conclusion: Sonography can be helpful in diagnosing the presence of worms, its complications and in evaluating response to treatment. Early surgical intervention in those with worm bolus, peritonism, and volvulus may salvage bowel and reduce mortality.

Urethral duplication: Experience of four cases

Aim: Our experience of 4 cases of urethral duplication is reported here. Materials and Methods: A retrospective chart review. Results: The age at presentation varied from newborn to 10 years. The clinical presentation ranged from prepubic sinus to diphallus urethra. There were 2 each incomplete duplication with only external openings (Type IA) and complete duplication of Effmann Type IIA2. All underwent complete excision of accessory urethra and corrections of associated anomalies. Conclusions: Urethral duplications have a varied presentation. At follow up, all are asymptomatic with good cosmetic result.

Review of esophageal injuries and stenosis: Lessons learn and current concepts of management.

Aim: To review the patients with esophageal injuries and stenosis with respect to their etiology, clinical course, management, and the lessons learnt from these. Materials and Methods: Retrospective descriptive observation review of children with esophageal injuries and stenosis admitted between January 2009 and April 2015. Results: Eighteen children with esophageal injuries of varied etiology were managed and included, seven with corrosive injury, five with perforation due to various causes, three with mucosal erosion, two with trachea esophageal fistula (TEF), and one wall erosion. The five children who had perforation were due to poststricture dilatation in a child with esophageal atresia and secondary to foreign body impaction or its attempted retrieval in four. Alkaline button cell had caused TEF in two. Three congenital esophageal stenosis (CES) had presented with dysphagia and respiratory tract infection. Six corrosive stricture and two CES responded to dilatation alone and one each of them required surgery. Four of the children with esophageal perforation were detected early and required drainage procedure (1), diversion (1), and medical management (2). Pseudo diverticulum was managed expectantly. Among TEF, one had spontaneous closure and other one was lost to follow-up. All the remaining nineteen children have recovered well except one CES had mortality. Conclusion: Esophageal injuries though rare can be potentially devastating and life-threatening.

Neonatal mesenchymal hamartoma of liver: An unusual presentation

Mesenchymal hamartoma of the liver is the second most common benign liver tumor in children. Typically, it presents as a large benign cystic, solid or mixed liver mass in a child younger than 3 years and amenable to complete resection. We report a neonate with Mesenchymal hamartoma of the liver presenting as giant intra abdominal cyst and its rare association with malrotation of bowel.

Modified koyanagi repair for severe hypospadias.

Aim: To report the results of an early series of patients who underwent modified Koyanagi repair for severe hypospadias. Materials and Methods: A total of 24 boys (age: 9 months to 11 years) with proximal hypospadias, chordee, and poor urethral plate underwent modified Koyanagi repair between September 2008 and January 2012. Nine boys had associated penoscrotal transposition that was corrected simultaneously. Vascularized parameatal based foreskin flap was used to correct the hypospadias in a single stage. The follow-up ranged from 6 months to 3.5 years. Results: A total of 13 of the 24 children had a good outcome and were voiding normally, while 11 boys developed complications, 3 of which were major and 8 minor. The major complications were complete breakdown (n = 1), meatal and distal neourethral stenosis requiring laying open of distal urethra (n = 1), and glans breakdown (n = 1). The minor complications included fistulae (n = 5), meatal stenosis amenable to dilatation (n = 1), and lateral chordee (n = 1). Majority of the complications were in the initial patients, with successful outcomes in the last 1 year. Most of these complications were successfully managed by minor second procedures. Conclusion: Modified Koyanagi repair not only corrects severe hypospadias with chordee but also corrects the associated penoscrotal transposition in a single stage. The results are good once the learning curve is crossed.

Embryogenesis and types of subcostal hernia—A rare entity

BACKGROUND/PURPOSE Four infants with congenital subcostal hernia are reported, as it is a rare entity with only two cases previously reported. Further, there are no reports concerning the complex multisystem subtype. Embryogenesis of the associated anomalies and subcostal hernia and their management are discussed. MATERIALS/METHODS Clinical features, history, investigations, associated anomalies, and management data of four patients with subcostal hernia were collected and analyzed. RESULTS The following associated anomalies were detected: renal agenesis (2), musculoskeletal abnormality (3), congenital heart disease (2), müllerian-renal-cervicothoracic somite abnormalities and vertebral-anorectal-cardiac-tracheoesophageal-renal-radial-limb anomalies (1). The subcostal hernias were treated by laparoscopic assisted (3) or laparoscopic herniorrhaphy (1). CONCLUSIONS Subcostal hernia is a rare entity with varied clinical presentations and presents either as an isolated defect or as a complex multisystem defect. The exact etiology is still unknown. Phenotypic manifestation of the complex defect is probably due to developmental gene defect affecting the coordinated growth of mesoderm around 4th to 10th weeks of fetal life.

Giant lipoblastoma of the thigh: a rare soft tissue tumor in an infant.

Lipoblastoma is a rare lipomatous tumor encountered almost exclusively in infants and young children. It arises from embryonic white fat. The common site of involvement is the extremities. In spite of their potential for local invasion, they are benign tumors. We report a case of a lipoblastoma in an infant and review the literature pertaining to clinical management of these tumors.

Imperforate hymen: Varied presentation, new associations, and management

Aim: Imperforate hymen is an isolated and sporadic event. The aim of this study was to report varied clinical and management problems of consecutive imperforate hymen in children and to compare the genetic review with literature. Materials and Methods: This is a retrospective analysis of eight consecutive imperforate hymen children admitted during 2010–2015. Results: Among eight girls, two were infants and six were in the adolescent group. Clinical presentations included varied degree of genitourinary obstruction (7) and incidental finding (1). Genetic analysis of imperforate hymen suggested sporadic event (5), associations (2), and syndromic (1). Ultrasound and magnetic resonance imaging revealed the level of obstruction. Hymenectomy was done in neonate (1), adolescent (6), and one has been under observation. Abdominoperineal pull-through was done in concomitant proximal vaginal atresia. Conclusions: Hymen development origin is variable and complex. Imperforate hymen is rarely a part of systemic/genetic anomaly. Genital examination at birth or during puberty is mandatory which often guides the timing of hymenectomy and prevents the sequelae of imperforate hymen. Hymenectomy is ideal during puberty and resolves all genitourinary obstructions.

Adrenal mass: Unusual presentation and outcome

Aim: Adrenal mass may be functioning or nonfunctioning with varied clinical presentations. This study aimed to report the nature and management of uncommon adrenal mass and to review literature. Materials and Methods: This was an retrospective observational analysis of children with uncommon adrenal mass admitted during 2009–2015. Clinical features, investigations, and management of patients were analyzed. Results: Among six, two each were adolescent and neonate, and one each was young infant and prenatal. Clinical presentation was variable; hypertensive retinopathy,[1] virilization[1] and bleeding diathesis,[1] antenatal suprarenal mass,[1] prenatal adrenal angiolipoma,[1] and spontaneous resolution of Stage III suprarenal mass.[1] Ultrasound and contrast-enhanced computed tomography revealed well-defined, heterogeneous adrenal mass. Size varied from 2 to 15 cm. Urinary metanephrine and serum testosterone were raised in adolescent hypertensive boys and virilized girls, respectively. Laparoscopy-assisted adrenalectomy was done in two and other four were managed conservatively. Histopathology of tumor revealed pheochromocytoma and borderline oncocytoma. Spontaneous resolution of adrenal mass had varied etiology; adrenal hemorrhagic lesion,[1] simple cyst,[1] neuroblastoma.[1] Follow-up varied from 3 months to 2 years. All patients were asymptomatic on last follow-up. Conclusion: Close clinical follow-up, contrast-enhanced tomography, and limited/specific endocrine work-up have definite role in the management of uncommon adrenal mass.