Disorders of sexual development; a cryptic combo of care

To evaluate the etiology, clinical presentations, investigations and surgical management of patients presenting with atypical genitalia in Pediatric surgery department. Disorders of Sexual development are one of the most complex congenital disorders encountered by the treating physicians. In recent years, the diagnostic ability and surgical techniques of gender reconstructions have improved remarkably. The spectrum includes chromosomal, genotype and phenotype abnormalities. The birth of a baby with atypical genitalia poses diagnostic and treatment challenges. This article focuses on etiology, clinical presentations, investigations and surgical management of patients presenting with ambiguous genitalia. A descriptive, prospective case series. Department of Pediatric Surgery, Military Hospital, Rawalpindi, Pakistan. October 2010 to June 2014. All patients with atypical genitalia, who underwent any kind of genital reconstruction surgery were included in the study. A total of 28 patients were operated upon during the study period. Age ranged from 11months to 12 years. Patients were divided into two groups on the basis of their karyotyping. Seventeen patients had 46 XY and 11 had 46 XX. No mosaic pattern was seen. Male genital reconstructive surgery was done in 16 patients while 12 underwent female genital reconstructive surgery. Disorders of sexual development present as a complex spectrum of psychosocial, sexual, endocrine and surgical implications, thus management demands a multidisciplinary team approach. Appropriately timed surgical intervention after thorough work up and detailed counseling, produce excellent cosmetic and functional results

Anterior saggital anorectoplasty; the treatment of anorectal malformations in female children

Anorectal malformation is the common congenital malformation. Ectopic anus and vestibular fistula are Intermediate types of anorectal malformations [ARM], which are the most common in female babies. Many surgical procedures have been described for the treatment of ARM. Anterior Saggital Anorectoplasty [ASARP] is not only convenient for the anesthetist for maintenance of anesthesia but also gives better exposure of surgical structures during surgery. To determine the technical suitability and outcome of ASARP in intermediate types of ARM in female children. This Descriptive study with prospective collection of data according to a set protocol. The study was carried out at the department of Pediatric Surgery, Military Hospital, Rawalpindi, Pakistan. November 2010 to March 2014, over the period of 3 years and 5 months. The data of all female patients presenting with intermediate types of ARM and undergoing ASARP, during the study period were analyzed, with respect to age, type, associated anomalies, complications and cosmetic outcome. A total of 36 patients of intermediate variety underwent ASARP. Age ranged from 6 months to 22 years. All patients had colostomy prior to this procedure. During surgery, posterior vaginal wall tear occurred in 2 patients [5.5%]. Postoperatively, 2 patients [5.5%] had retention of urine, 2 patients [5.5%] developed wound infection with superficial disruption, anal stenosis occurred in 2 patients [5.5%] and 1 patient [2.7%] had rectal mucosa prolapse. None of them required re_ do surgery. Cosmetic outcome was excellent in 31 patients [86.1%], while it was satisfactory in 5 [13.8%] patients. Anorectoplasty through anterior approach is not only technically easy but has good cosmetic results in intermediate type of imperforate anus in female children

Single scrotal incision orchidopexy for palpable undescended testis

To retrospectively asses the results of single scrotal incision orchidopexy [SSIO] performed at our centre with ligation of the patent processus vaginalis in children having palpable undescended testes [UDT]. Quasi-experimental with retrospective data. Department of Paediatric surgery Military Hospital Rawalpindi, from April 2007 to December 2011. After making a single transverse incision at superior scrotal border, the testis was identified; gubernaculum and the sac were dissected to the highest level and divided. The testis was places into the scrotum and fixed to the scrotal fascia/skin. All patients were assessed at 2 weeks, 2 months and 6 months post operatively, and then yearly. A total of 38 orchidopexies were performed in 33 patients. The patients' age ranged from 14 months to 7 years [mean: 2.1 years]. Bilateral UDT were found in 5 patients [15.1%]. Operative time ranged from 20 to 45 minutes [mean: 36 minutes]. The single scrotal incision technique was successful in all 38 cases [100%]. All testes were easily fixed in the scrotum. Two patients [5.2%] developed scrotal haematoma and one patient [2.6%] developed stitch abscess. All showed good anatomical and cosmetic results up to a minimum of six months of follow-up. Single scrotal incision orchidopexy for palpable undescended testis is a simple and safe technique. It has shown to consume shorter time and give good cosmetic results

Intralesional bleomycin sclerotherapy: an effective treatment of cystic hygroma in children

To study the outcome of intralesional sclerotherapy with injection Bleomycin in cystic hygroma in children. A case series. The department of Pediatric Surgery at Military Hospital, Rawalpindi, Pakistan from Jan 2011 to Dec 2012. All patients with peripheral cystic hygroma [CH] presenting to us, were enrolled in the study. The cyst was aspirated in the operation theater under sedation. Injection bleomycin 0.5 mg /kg diluted in 10-15 cc of distilled water was injected in the cyst at multiple sites. Injection was repeated after every month depending upon the response. A total of 30 patients reported to the department with superficial cystic hygroma, 12 were males [40%] and 18 were females [60%], age ranged from 15 days to 8 years. Cervico-facial was the most common site. Results were assessed in terms of excellent [complete resolution], good [> 50% reduction in size] and poor [< 50% reduction in size]. In 2 patients, complete resolution was achieved after maximum seven shots of intra-lesional bleomycin injections [IBI], while 18/30 [60%] resolved after single dose. Twenty seven patients [90%] resolved completely, 2 [6.6%] had good response, 1 [3.3%] showed poor response. Minor complications were noted which were treated by symptomatic treatment. No major side effects or recurrence were noted in maximum 2 years follow up. Intralesional bleomycin sclerotherapy is safe and effective as primary treatment in cystic hygroma

Enteric duplication in children: clinical presentation and outcome

<p><b>INTRODUCTION</b>Enteric duplication (ED) is an anomaly with varied presentations and possible involvement of the alimentary tract. Once diagnosed, resection of the lesion and the involved part of the gut is usually required. The aim of this study was to evaluate the clinical presentations, diagnostic investigations, management and outcomes of patients with ED.</p><p><b>METHODS</b>This was a descriptive case study conducted at the Department of Paediatric Surgery, Military Hospital, Rawalpindi, Pakistan, from January 2005 to January 2011. The medical records of all patients diagnosed with ED were retrospectively analysed with respect to age, presentation, investigations, site and type of lesion, surgical procedures, histological findings and complications.</p><p><b>RESULTS</b>A total of nine patients were managed during the study period. The patients' ages ranged from three months to five years. Four out of nine EDs were rectal duplications. Three EDs were of the cystic type, five were of the tubular type and one was a complex mixed anomaly. Patients presented with varied symptoms, with the two most common being the presence of an abdominal mass and bleeding per rectum. Diagnosis was mainly achieved based on magnetic resonance imaging and computed tomography, although Meckel's scan provided accurate diagnosis in three of the nine patients. All the cysts were resected without any major complications, and patients were event-free during the five-year follow-up.</p><p><b>CONCLUSION</b>EDs should be kept in mind when examining patients with an abdominal mass and bleeding per rectum. Meckel's scan can provide accurate diagnosis of EDs with bleeding. Prompt diagnosis and management results in satisfactory outcomes.</p>

Anterior sacral meningocele

Anterior sacral maningocele is a rare condition presenting as a lower abdominal mass. One such case was seen in a baby girl aged 2 months, who was admitted with abdominal distension and urinary difficulty for one week. She had a lower abdominal mass which investigated by MRI, turned out to be an anterior sacral meningosele causing her symptoms. The meningocele was excised successfully via an open abdominal approach. Postoperative recovery and follow-ups remained uneventful. Presentation, various diagnostic modalities and treatment options for an anterior sacral meningocele are briefly discussed

Preliminary results of laparoscopic assisted orchidopexy for intra-abdominal testis

To analyze the outcome Laparoscopic assisted Orchidopexy for Intra-abdominal Testis. A prospective, descriptive study from July 1997 to January 2007. The study was conducted in the Departments of Pediatric Surgery at Dow Medical College / Civil Hospital Karachi and National Institute of Child Health, besides some private hospitals of Karachi. All patients with impalpable testis. Patients with intra-abdominal testis were subjected to Laparoscopic assisted single stage or 2-stage Fowler-Stephens orchidopexy. All patients were reviewed two and six months after the procedure to assess the outcome of surgery. A total of 28 patients with 35 impalpable testes were seen during the study period. Of these 13 had impalpable testes on the right and eight on the left side, whereas seven were bilateral. Laparoscopic examination revealed 29 intra-abdominal testes. Amongst these, Laparoscopic assisted Single stage Orchidopexy was performed in four testes and the first stage of Fowler-Stephen Orchidopexy in 29 testes. Second stage of the Fowler-Stephens Orchidopexy was completed in 18 testes successfully, as we did not encounter testicular atrophy in any case so far. Postoperative complications were seen in two patients. One case developed port site haematoma which was drained and the other subcutaneous emphysema which was managed conservatively. Laparoscopic assisted single and 2-stage orchidopexy is a safe and successful treatment procedure for intra-abdominal testes

Abdominal masses in children: a diagnostic challenge

We report a 6 year old female child who presented with mass in right upper quadrant of abdomen. She was intensively investigated and with the suspicion of hydatid cyst liver received albendazole for many months. As mass increased further laparotomy was planned. At exploration it turned out to be a large retroperitoneal mass and dissected out easily. Biopsy report was that of benign teratoma

Thymolipoma in a child [a case Report]

A 2 year old male child with history of mild respiratory difficulty and repeated chest infection on CT scan, found to have an anterior mediastinal mass, which pre operatively was suspected as thymolipoma. Through median sternotomy mass was removed in totto. Histopathology confirmed the pre operative diagnosis. Post operative recovery was uneventful. At 6 months follow up there was no recurrence