Oral Propranolol in the Treatment of Ulcerated Bleeding Scrotal Arteriovenous Malformation.

Arteriovenous malformations (AVMs) of the scrotum are very rare, with only 35 adult cases in the literature. An 8-year-old boy presented with an ulcerated bleeding AVM of the scrotum. The patient was resuscitated and managed conservatively initially. After the control of bleeding, oral propranolol was started. There was a decrease in the size of scrotal and penile swelling, healing of ulcer with total healing by 1 month, and no recurrence of bleeding episode. To the best of our knowledge, this was the first case of pediatric scrotal AVM treated by oral propranolol.

Anorectal Malformation Associated with Klippel-Feil Syndrome: A Rare Association.

Anorectal malformations (ARMs) are a complex group of malformations associated with various congenital anomalies. Klippel-Feil syndrome (KFS) is characterized by fusion of cervical vertebrae, short neck, torticollis, and/or facial asymmetry and very rarely associated with ARM. In the presence of cervical vertebral anomalies in ARM, one should search for the presence of KFS as an association. If this anomaly is found to be associated, caution is needed during positioning for examination, surgery, during laryngoscopy, and intubation due to risk of neurological damage. We hereby present a very rare association of KFS with ARM with solitary kidney and ipsilateral vesicoureteral reflux.

Undescended testicle: An update on fertility in cryptorchid men.

Cryptorchidism or undescended testis is one of the most common anomalies encountered in paediatric urology and is estimated to affect 1 to 4 per cent of full term and upto 30 per cent of preterm male neonates. The associated problems of sub-fertility or infertility and malignant transformation have been recognized for long. Fertility is impaired after both unilateral and bilateral cryptorchidism. The reported paternity rates in adults are about two-third for unilateral undescended testis and less than one-third for bilateral disease. Over the last five decades, the concepts related to cryptorchidism have changed dramatically as knowledge about its effects has accrued from research conducted worldwide. The recommended age of orchidopexy has fallen progressively from adolescence to less than one year. The realization that the infantile testes are not in a state of 'suspended animation' and the recognition of the defect in the androgen dependent transformation of gonocytes into adult dark spermatogonia in cryptorchidism have been recognized as the primary cause of sub-fertility in these patients. This has paved the way for hormone therapy in an attempt to simulate the 'post-natal gonadotropin surge' or 'mini-puberty'. This review summarizes the current knowledge about the various factors affecting the fertility status in cryptorchidism with a particular focus on the derangements in the development and maturation of the germ cells and the role of surgery, hormone therapy and antioxidants in reversing these changes.

Percutaneous trans-hepatic gelfoam-cyanoacrylate glue embolization for ruptured post-traumatic hepatic artery pseudo-aneurysm in a limited-resource scenario.

INTRODUCTION: We present our experience with an indigenously designed percutaneous trans-hepatic ultrasound-guided Gelfoam sponge cum cyanoacrylate glue-based embolization technique for the treatment of a ruptured post-traumatic aneurysm of a branch of the right hepatic artery (RHA) as a 'life-saving emergent' procedure in a patient unfit for surgery or endovascular intervention and in a 'limited-resource' scenario (non-availability of Digital Subtraction Angiography Suite). CASE DETAILS: An 8-year-old boy sustained crush-injury to the right lobe of the liver in a road-traffic accident and presented in shock. After resuscitation, a laparotomy and repair of the right lobe of liver were undertaken. Bleeding restarted 1 week after the surgery; the patient bled from drain site and went into shock. Exploration was not advisable in view of poor general condition, and sepsis, deranged coagulation and parental reluctance in view of guarded prognosis. Multi-detector Computed Tomography Angiography was performed after resuscitation which revealed active bleed from a ruptured pseudo-aneurysm of a branch of RHA. TECHNIQUE: The bleeding artery was identified with duplex sonography and was embolized by the percutaneous trans-hepatic route proximal to the site of pseudo-aneurysm and rupture by a two-step process. Initially, a thin paste/'slurry' made of powdered gelfoam dissolved in sterile saline was injected into the bleeding vessel. Subsequently, the area was sealed by injecting 1.0 ml of N-butyl-2-cyanoacrylate glue. Hemostasis was confirmed by Color and Power Doppler Ultrasonography both post-procedure and after 48 h. With supportive management, the patient showed a rapid recovery and was discharged after 2 weeks. He continues to be well at 3-month follow-up. CONCLUSION: The technique was effective in controlling hemostasis and life-saving in our set-up.

Surgical Technique of Glans Wings Creation with Preservation of Glanular Vascular Arcade and Creating Flaps for Tension-Free Glansplasty in Hypospadias Repair.

OBJECTIVE: The objective of this study is to report the technical nuances of glans wings creation in anatomical plane facilitating liberal glans wings mobilization with preservation of glanular vessels for tension-free glansplasty in surgery of hypospadias in primary and redo cases. MATERIALS AND METHODS: Eighty-six primary hyposapdias and 7 cases of distal hypospadias, operated elsewhere and presented with glans and urethroplasty dehiscence, undergoing tubularized-incised-plate (TIP) repair were included after ethical approval. Technical points of glans wings creation in the subfascial plane included (i) creation of Buck's fascia window, just proximal and lateral to the point of bifurcation of corpus spongiosum, (ii) creation of subfascial-pret unical plane on the tunica albuginea of corpora cavernosa up to ventral limit of laid open glanular meatus, (iii) release of pillars of corpus spongiosum off the glans base keeping the basal lamina propria covering the vascular arcade intact, (iv) release of glans base off the tip of corpora cavernosa and composite flap of corpus spongiosum pillars with Buck's fascia off the corpora cavernosa, (v) approximation of glans wings over the tubularized-incised-plate covered with dartos, and (vi) approximation of fasciospongioplasty flaps at hypospadiac meatus. Outcome measurement included (i) intra and postoperative problems and (ii) elimination of glans dehiscence. RESULTS: There occurred 1 injury to the tunica albuginea of ventral corpora cavernosa and 1 button-hole injury to subcoronal mucosal collars. Flaps for fasciospongioplasty were short in 11. One glans dehiscence occurred in flat glans. CONCLUSIONS: This technique is effective in creating glans wings in the anatomical plane with the preservation of glanular vessels and flaps for fasciospongioplasty eliminating the possibility of glans dehiscence in both fresh and redo cases.

Study of pelvic floor and sphincter muscles in congenital pouch colon with the help of three-dimensional CT scan.

INTRODUCTION: Congenital pouch colon (CPC) is a pouch-like dilatation of shortened colon associated with anorectal malformation (ARM). The disease is prevalent in northern India. Postoperatively, the continence results are not as good as in other ARMs and there is higher incidence of incontinence and perineal soiling in these patients. The present study aimed to evaluate the pelvic floor and sphincter muscle characteristics in patients of CPC with the help of 64-slice computerized tomography with three-dimensional (3D) volumetric reconstructions of images, thus, to know the overall quality of these muscles in the patients. MATERIALS AND METHODS: The study was conducted in patients admitted over a period of July 2007 to November 2008 in our department. Totally, eight patients of CPC were subjected to 64-slice CT with three-dimensional reconstructions of images and different parameters such as quality of pelvic floor muscles, configuration of vertical and parasagittal fibres, shape and thickness of sphincter muscle complex, attenuation values of sphincters were studied. RESULTS: The 3D reconstructed images of pelvis in patients of CPC showed a well-developed pelvic floor and sphincter muscle complex. The length of the parasagittal fibres, transverse width of the vertical fibres and CT attenuation values of these structures with overall muscle quality were found to be good in these patients. CONCLUSION: In cases of CPC, the pelvic floor muscles including striated muscle complex (vertical and parasagittal fibres) are well developed. Higher rates of incontinence and soiling in CPC are not because of poorly developed pelvic floor and sphincter muscles. Three-dimensional CT can also provide important anatomical information that can help the operating surgeon while performing surgery.

Study of Variations in Axial Pattern Vessels of Penile Dartos in Hypospadias and Implied Surgical Significance.

OBJECTIVES: To report the anatomical variations in axial pattern vessels of penile dartos in hypospadias. METHODS: Eighty hypospadias undergoing repair with tubularized incised plate urethroplasty were studied with preoperative examination of visible vein through penile skin. Course of vein proximal to root of penis was studied with vein viewer. Modified penile degloving beginning at preputial edge, splitting inner preputial urothelium off the preputial dartos and proceeding to penile shaft in subdartos plane was performed. Axial pattern veins visible on penile dartos were studied. Axial pattern penile dartos arteries were studied by recording arterial pulsations. If arterial pulsations were not seen, arterial mapping on penile dartos completed with use of Doppler and images recorded. Variations in axial pattern dartos vessels were analyzed. RESULTS: In 32 hyposapadias of 1 group, median superficial vein along the dorsal midline of penile dartos drained into left saphenous vein. In this group, dorsolateral vessels were not seen on either side in 20 cases. Unilateral dorsolateral vessel in 7 and bilateral dorsolateral vessels in five were seen. In 48 hypospadias of other group, median superficial vein was not seen. Bilateral dorsolateral vessels in 11, unilateral dorsolateral vessels in 27 and no definite pattern in 10 were seen. In both groups, superficial median artery was not discovered. Vessels were longitudinally oriented. Penile dartos was equally distributed. CONCLUSION: In hypospadias, penile dartos vessels have axial pattern course longitudinally but not identical to normal. Eight patterns can be identified which can be diagnosed on careful clinical examination of penis.

Evaluation of nebulized N-acetyl cysteine in outcome of esophageal atresia with tracheoesophegeal fistula.

AIM: To evaluate the role of nebulized N-acetyl cysteine (NAC) in liquefying the airway secretions and improving the outcome of patients of esophageal atresia with tracheoesophageal fistula (EA + TEF). METHODS: It was a non-randomized interventional study. Two milliliters of 10% NAC was given in a nebulized form (2:5 dilution, every six hourly) to patients of ET + TEF, along with regular suction of upper esophageal pouch. The group was compared with control, which comprised patients of EA + TEF receiving only saline nebulization. The consistency of the secretions was compared by hand held consistometer in unit of time (seconds) required to cross a predetermined distance along with gravity. RESULTS: Sixty patients were assessed. Of these, 30 patients were present in both groups. The study group showed significant (p = 0.01-0.0001) decrease in consistency of secretions from the control group after day 2 of NAC nebulization. Patients' discharge was significantly (p = 0.01) earlier in cases. There was no significant (p = 0.41) difference in mortality between the groups. No specific adverse effects were observed in the study group. CONCLUSION: It appears that nebulized NAC decreases the consistency of secretions in EA + TEF patients. It is interesting to note that the group of patients that received NAC was discharged earlier than the control group and had a higher survival rate than the control group. Whether this is directly attributable to the use of NAC is unknown. A prospective double-blinded randomized clinical trial is warranted to confirm these results. LEVEL OF EVIDENCE: Level II, prospective comparative study (non-randomized).

Presentation of high ano-rectal malformation beyond neonatal period.

Anorectal malformation is one among the common congenital malformations usually encountered. The babies usually present early after birth with complaints of missing anal opening, or abnormally placed anal opening, but some patients have presented beyond the early newborn period without recognition of their anorectal malformation. We are presenting a case of a male child with high ARM, who unusually presented first at the age of 45 days, without gross abdominal distension or septicaemia.

Modified method of primary esophageal anastomosis with improved outcome in cases of esophageal atresia with tracheoesophageal fistula.

Survival rates for infants who have esophageal atresia (EA) with or without fistula (TEF) have improved dramatically in the past 50 years. Despite excellent long-term survival for patients with esophageal atresia with tracheoesophageal fistula (EA-TEF), many significant complications can occur. Anastomotic leak at the esophagoesophagostomy site is one such problem resulting in considerable morbidity and mortality in these patients. The methods of esophageal anastomosis for long period has remained the simple end to end anastomosis of esophageal ends with various modifications described from time to time. The present study aims to study the effect on the early postoperative complications, following horizontal mattress suture technique on the primary esophageal anastomosis in cases of EA-TEF. A total of 32 patients with EA-TEF, were operated by our technique during a period of 1 year (2007-2008). The results were compared with the patients (n = 66), who were operated by the traditional simple technique during the same period. Among those patients in whom the esophageal anastomosis was done by horizontal mattress suture, only one had major anastomotic leak, while two had minor anastomotic leaks, as compared to six and nine cases correspondingly in other patients in whom anastomosis was done by simple technique. There was single mortality. We propose that, the utilization of our technique of horizontal mattress suture in primary anastomosis of esophagus in cases of EA-TEF significantly reduces the risk of anastomotic leaks and subsequent morbidity and mortality.

Evaluation of congenital diaphragmatic hernia in a tertiary health center of a developing country: management and survival.

AIM: The purpose was to evaluate the diagnosis and efficacy of management of congenital diaphragmatic hernia (CDH) in a tertiary health center of a developing country. METHODS: Forty-six children aged from 1 day to 7 years were studied. Parameters studied were age, sex, clinical features, and management. RESULTS: Fifty-six percent of patients presented in the neonatal period; however, none of them presented on the first day of life. The majority (91.3%) of patients had left-sided CDH. Respiratory distress was the most common clinical feature observed (91.3%). Chest X-ray confirmed the diagnosis in 82.6% of patients, and contrast study was needed in the remaining 17.4%. The survival rate was 87%. It was better in patients presenting late than those presenting in the early neonatal period. Stabilization in the preoperative period improved survival. Not using a chest tube had no adverse effect on survival. CONCLUSION: The relatively increased survival rate of CDH in a tertiary health center of a developing country is attributed to delayed arrival to the center. Respiratory infections compound the survival. More studies are needed before it can be safely said that not using a chest tube has no adverse outcome. Late presentation has been associated with varied manifestations, hence proper clinical evaluation, a high index of suspicion and adequate management, which includes imaging and surgery after stabilization, gives excellent results.

Congenital megalourethra.

The authors report on two patients who had fusiform megalourethra with unusual features. The first patient had left hydronephrosis with duplication of the ureter, oblique sagittal septum in the urinary bladder, and Y duplication of urethra with megalourethra of both channels. The second had features of the abdominal muscle deficiency syndrome, with anterior ectopic anus, rectourethral fistula, and fusiform megalourethra. Both patients were treated by preliminary urinary diversion. The first patient subsequently had staged reconstruction. The other patient died of urinary sepsis. The authors believe that preliminary urinary diversion followed by planned staged reconstruction offers hope for the reconstruction and rehabilitation of these cases.

Anterior sagittal anorectoplasty for anorectal malformations and perineal trauma in the female child.

Vestibular fistula and perineal ectopic anus are the most common anorectal malformations in female children. Anterior saggital anorectoplasty (ASARP) was used to treat 416 patients with these anomalies, as well as cases of perineal canal and third-degree perineal tear, during a 20 year period. Preliminary colostomy was performed in only four patients, who had a perineal tear. For ASARP, a midline incision was made and the rectum was separated from the vagina and placed in the center of the sphincteric muscle complex. The perineal body was reconstructed, and normal appearance of the perineum was achieved. Six patients had complications postoperatively (4 had secondary hemorrhage, 2 had wound infection). One patient with hemorrhage and one with infection required laying open of the wound and revision surgery after 12 weeks. Both recovered well. Of the 416 patients, 397 returned for follow-up 12 weeks after the surgery. Of these, 390 had a normal-looking perineum and normal defecation. Of the remaining seven patients, three had retraction of the rectum, one had recurrence of the fistula, and another had acquired perineal canal. These five children required revision ASARP and recovered successfully. The other two patients had mucosal prolapse, which reduced spontaneously. Three hundred twenty-six children of the 416 patients have had follow-up beyond the age of 3 years. Of these, 291 had normal bowel habits without the use of laxatives. Of the other 35, four had fecal impaction of unknown etiology, which was managed conservatively, 6 had anal stenosis (four underwent posterior Barrow's flap interposition and two responded to dilatation), and 25 had a posterior ledge that caused constipation and required cutback. Overall, eight patients (1.9%) required revision ASARP and 40 others (9.8%) had minor complications. This operation provides consistently good results, and the authors recommend it for the management of anorectal malformations and perineal trauma in female children.

Coloplasty for congenital short colon.

Seventy-two patients with complete short colon (CSC) were treated at the authors' institution between 1971 and 1994. Initially, one-stage coloplasty with abdominoperineal pull-through was performed in 10 neonates, with a 50% survival rate. The subsequent 62 patients had staged management in which a window colostomy was performed at the time of presentation. Of the 50 survivors in this group, 20 had coloplasty with a abdominoperineal or sacroabdominoperineal pull-through by June 1994. A protective ileostomy was done at the time of coloplasty in 10 patients, and was closed 6 to 12 weeks later. Two of the 20 patients who had staged reconstruction died because of complications; the others recovered well. The overall survival rate after coloplasty was 76.7%. Eighteen of the 23 survivors have had follow-up for up to 8 years. They have shown normal growth and good continence and have been free of significant long-term complications. The other 30 patients are awaiting definitive surgery. A staged management plan is advocated for complete short colon because it provides consistent results and good quality of life.

Delayed presentation of strangulated congenital diaphragmatic hernia: learning from our experience.

BACKGROUND: Strangulation is very rare in congenital diaphragmatic hernia (CDH) of the Bochdaleck variety. Here, we share our experience with six cases of delayed presentation of strangulated CDH. The aim of this article is to provide information on how to diagnose and manage this situation using a systematic approach. MATERIALS AND METHODS: A retrospective review identified six cases of strangulated/obstructed CDH from 1998 to 2011. Demographic data, clinico-radiological findings, management and complications, along with final outcome were recorded. RESULTS: Small bowel gangrene was found in one patient, gastric perforation in three, transverse colon perforation in one and colonic obstruction in one patient. Video-assisted thoracoscopic surgery (VATS) was used in all but one patients for definitive diagnosis, diaphragmatic repair, pleural lavage and management of empyema. Laparotomy was needed for management of strangulated or perforated bowel. Three patients in this study survived. CONCLUSION: Clinicians should always consider a diagnosis of obstructed Bochdaleck hernia in children, because X-ray findings are not always typical or even normal in complicated CDH. VATS may be considered as both diagnostic and therapeutic. Preventive measures for empyema or early intervention in the evolving stage can significantly reduce morbidity.

Simultaneous occurrence of jejuno-jejunal and ileo-ileal intussusception in a child: a rare occurrence.

Intussusception is the most common cause of intestinal obstruction in infants and children. This condition is frequent in children and presents with the classic triad of cramping abdominal pain, bloody diarrhoea and a palpable tender mass. Small bowel intussusceptions are much less common, with jejuno-ileal and duodeno-jejunal intussusceptions being the rarest types of all. Multiple simultaneous intussusception is a peculiar variety of intussusception. The authors report the simultaneous occurrence of jejuno-jejunal and ileo-ileal intussusception in a patient. As this is an extremely uncommon entity, it is being reported with a brief review of the relevant literature.

Delayed presentation of anorectal malformations.

AIMS AND OBJECTIVES: Delayed management of anorectal malformation (ARM) increases the surgical and functional complications for the patient. We defined "delayed presentation of ARM" and reviewed our patients with ARM to find out the incidence and causes of delayed presentation. MATERIALS AND METHODS: Patients satisfying the criteria of "delayed presentation of ARM" were involved. Detailed information of each patient including the mode of presentation, associated anomalies, plan of management and follow-up was obtained from the hospital records. RESULTS: Between 2003 and 2006, 43 patients satisfied our criteria of "delayed presentation of ARM". There were 21 males and 22 females. Seventeen of these males presented with low-type ARM. Eleven of them were managed by a single-stage procedure. These "delayed presenters" had to live with constipation, inadequate weight gain and parental anxiety for a greater time. Analysis of the outcomes showed more functional complications in patients who had undergone failed perineal surgery previously. In females with low ARM, the procedure of choice was anterior sagittal anorectoplasty (ASARP). Single stage surgery provides good outcomes for most of low type of ARMs. High-type ARMs in males and females were managed by a staged procedure. CONCLUSION: "Delayed presentation of ARM" is a major group of ARM in our setup. The management and results of their treatment are not different from those of the early presenters. The most common cause of delayed ARM is wrong advice given by the health care providers followed by inadequate treatment elsewhere. Corrective surgeries taking second attempt in perineum always produces poor outcomes.

Esophageal atresia: Factors influencing survival - Experience at an Indian tertiary centre.

OBJECTIVE: To study the clinical profile of the cases of esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) and various factors affecting the surgical and early postoperative management and their outcome. MATERIALS AND METHODS: A prospective analysis of 127 cases of EA from February 2004 to May 2006 was performed. Waterston prognostic criteria were used for grading. RESULTS: EA with TEF was the commonest type in 117 cases (92%). Associated congenital anomalies were present in 52 (41%) patients, the commonest being the cardiac anomalies, which was followed by the gastrointestinal anomalies. VACTERL was found in 6 (5%) cases. Prematurity, associated congenital anomalies, gap between esophageal ends and preoperative respiratory status were the significant factors affecting the survival (P = < 0.001). Primary extrapleural repair was the surgical approach in most of the patients. Azygos vein was preserved in 46 cases and no retropleural drainage was used in 27 cases. Staged procedures were performed in 19 cases, including 6 cases of isolated esophageal atresia. Pneumonitis and sepsis were the most common early postoperative complications (42%). Hypoxia and cardiorespiratory arrest were the most common causes of mortality (11 cases). Anastomotic leak complicated 13 cases, including 9 major and 4 minor leaks. Major leak followed by sepsis caused 7 deaths. Survival as per Waterston criteria was 100% in group A, 83% in group B and 22% in group C. CONCLUSION: Factors affecting the survival are major or life-threatening associated anomalies, long gap, pneumonia and sepsis at presentation or that acquired during hospitalization and major leaks. The high incidence of low birth weight, delayed diagnosis, poor referral, low-socio economic status and lack of advanced neonatological back up are important contributory factors to poor outcome.

Azygos vein preservation in primary repair of esophageal atresia with tracheoesophageal fistula.

The aim of this study is to report a series of patients with the Azygos vein preserved during the surgery for esophageal atresia with tracheoesophageal fistula (EA&TEF), highlighting the advantages in terms of survival and prevention of anastomotic leak. Ninety-six neonates with EA&TEF, admitted to the Department of Pediatric Surgery, King George Medical University between 2004 and 2006, were reviewed prospectively; the babies were randomly allocated to two groups: Group A (n = 46) in which the Azygos vein was preserved and Group B (n = 50), wherein it was ligated. The two groups were comparable in respect to sex, weight, prematurity, associated anomalies, Waterston classification, Spitz classification and distance between the pouches after mobilization. Anastomotic leak occurred in three cases (6%) in Group A and ten cases (20%) in Group B and was responsible for mortality in one (2%) case in Group A and six cases (12%) in Group B. Preservation of Azygos vein resulted in significant reduction in the number of anastomotic leaks. We propose that preservation of the Azygos vein prevents early postoperative edema of the esophageal anastomosis by maintaining the venous drainage and thus may form an additional protective factor against anastomotic leaks.