Megalourethra: a report of four cases and review of the literature

Megalourethra is a rare congenital anomaly characterized by severe dilatation of the penile urethra. Four cases of congenital megalourethra were seen at Sir Padampat Mother and Child Health Institute, Jaipur, during the last 10 years. Three cases of scaphoid megalourethra had no other associated congenital anomalies and were treated successfully without any complications, while one patient with a fusiform megalourethra had severe associated congenital anomalies and died. These cases are reported with a review of the literature.     

A complex case of megalourethra and its repair

Megalourethra is a rare malformation of the urethra caused by a lack of corpus sponigosum and in some cases corpora cavernosa in the region of the distal urethra. The absence of these structures causes a ballooning of the urethra despite there being no mechanical obstruction. A male child presented with so-called fusiform megalourethra, with absence of the corpora cavernosa and urethral duplication. A voiding cystourethrogram was used to diagnose a fusiform megalourethra with pronounced meatal stenosis and extreme stenosis of pendulous urethra. In addition, there was urethral duplication in the form of an accessory urethra stretching from the urethral colliculus to the perineum. Absence of the corpora cavernosa was also suspected in the distal urethra. The surgical procedure involved pendulous urethroplasty with an onlay technique using urethral duplication and penile reduction. This method of treating megalourethra has not been previously reported. The operative technique for fusiform megalourethra with genital malformation has to be tailored to each individual case, depending on the intraoperative and endoscopic findings.     

Rare congenital abnormalities of the anterior urethra

Congenital abnormalities of the anterior urethra other than hypospadias (valve, diverticulum, and megalourethra) are rare conditions and can be difficult to diagnose. Based on five recent cases of these urethral abnormalities, we review the embryology and the English literature, and have found reports of about 100 cases of valve and diverticulum and 50 cases of megalourethra. For all these abnormalities, the clinical presentation depends on age and the diagnosis depends essentially on voiding cystourethrography, which must show the entire urethra, and careful urethroscopic examination.     

Megalourethra associated with complete urethral obstruction and seen on antenatal ultrasound as a cystic mass

A case of congenital megalourethra associated with structural obstruction of the urethra and prune belly syndrome is presented. The dilated urethra was shown as a cystic mass on antenatal ultrasonography.     

Megalourethra with posterior urethral valves

A case of scaphoid megalourethra associated with the posterior urethral valves was seen in a 4-year-old male. The embryology of the anomaly and its management are discussed with a review of the literature. Accepted: 30 November 1998     

Megalourethra — a spectrum of disease

Three children with megalourethra are reported, two typifying the classical fusiform and scaphoid types and the third confirming a further, less severe variety of the condition that should be considered in children with dorsal penile curvature. Offprint requests to: D. M. Burge     

Congenital megalourethra

Congenital non-obstructive dilatation of penile urethra (megalourethra) can result from absence of corpus spongiosum alone (scaphoid) or alongwith the absence of corpora cavernosa (fusiform). Associated urogenital or other systemicanomalies are usually present and require detection and appropriate management. Urethroplasty (Nesbitt) gives desirable results. Condition has been reviewed with report of a case.     

Congenital megalourethra

Congenital megalourethra, an anomaly of the penile urethra, is discussed, and 15 cases from the English literature are reviewed. The relationship of this anomaly to other abnormalities of the genitourinary system is stressed.     

Congenital megalourethra associated with urethral duplication and imperforate anus

A newborn with a prenatal diagnosis of right hydroureteronephrosis and enlarged penis is presented. At birth, the baby had an imperforate anus (IA) with a megalourethra; radiologic and ultrasonographic studies showed a left polycystic kidney and right hydroureteronephrosis, right vesicoureteral reflux, and an incomplete urethral duplication with dilatation of the posterior urethra. The IA was corrected on the 1st day of life and a vesicostomy was performed at 1 month. At 1 year of age the valve obstructing the ventral posterior urethra was resected and the vesicostomy was closed. At 14 months the baby underwent a urethroplasty with a vertical preputial tubularized island flap and excision of the penile urethral duplication. Exact knowledge of the malformation was essential in planning the appropriate surgical treatment. Accepted: 11 June 1997     

Hypospadias surgery: a 10-year review

Hypospadias surgery is one of the most difficult areas in pediatric urology and has been characterized by constant evolution. Some of the surgical techniques proposed in the past are now considered inadequate because of an unacceptable complication rate or poor functional and aesthetic results. The key for assessing a surgical technique (or a particular aspect of it) is continuous evaluation through long-term patient follow-up. We present the medical records of 693 patients over 10 years, all operated on by the same surgeon (the first author), with a minimum of 12 months of follow-up. The overall complication rate was 6.49% (45 cases). The most frequent complication was urethral fistula, occurring in 28 patients (62%). Other complications were redo for penile deformity or meatal retraction (eight cases, 17%), megalourethra (five cases, 11%), meatal stenosis (two cases, 4%), and urethral stenosis (two cases, 4%). We have noticed an increased rate of complications in patients older than 12 months (18.7% vs. 3.4% in patients younger than 12 months) and in patients operated on at puberty or later (15%). We have seen no difference in the complication rate related to the type of hospitalisation (day surgery vs. traditional hospitalisation). Regarding the relationship between the type of complication and the type of defect, except for the constant presence of fistulae, a high incidence of megalourethra was seen in proximal defects treated with preputial graft. The sexual outcomes of 32 subjects are presented. Multiple factors influence the final result, but the most important factor is the surgeons own experience. Knowledge of different techniques and delicate tissue handling are essential. Our experience shows that the ideal age for surgery is 8–12 months. Owing to a minimal emotional impact on the child and to a reasonable use of economical resources, we consider day surgery the ideal way to treat these patients whenever possible. Even in the absence of complications, follow-up must be continued at least until the end of puberty and, when possible, up to the patients sexual debut. Adequate interviews with the patients (principally teenagers and young adults) are the best way to evaluate their need for psychological support.     

Congenital megalourethra

Megalourethra, a rare congenital disorder involving the anterior urethra, is subdivided into two types : Fusiform and scaphoid. Two cases of scaphoid type megalourethra are reported. The first patient, a 5-weeks-old infant diagnosed at birth as having the prune belly syndrome was admitted to the hospital with vomiting and failure to thrive. Intravenous pyelogram revealed marked dilatation of the left ureter and a bladder diverticulum. A penile urethrostomy was performed. The second patient, born to healthy parents after an uneventful pregnancy and delivery, was found to have an enlarged and deformed penis. The baby voided with a poor stream and a concomitant swelling of the penis was noted. Retrograde uretrography showed a sac-like dilatation of the penile urethra. Surgical revision was carried out a 2 stage procedure and was completed (Nesbitt's operation) after 4 months. The patient did well postoperatively and voided with a normal stream without any abnormality of the penile shaft.     

Observational retrospective study on acquired megalourethra after primary proximal hypospadias repair and its recurrence after tapering

IntroductionAcquired megalourethra (AMU) after repair of proximal hypospadias can be a serious complication. An observational retrospective study of its incidence among different types of repair was performed.Materials and methodsClinical charts of patients operated on for proximal hypospadias were reviewed. Inclusion criteria: all primary hypospadias operated in 1991–2004, with the meatus positioned in proximal penile, scrotal or perineal position.ResultsOf 770 hypospadias cases treated, 130 (16%) were proximal. Seventy-two patients (55%) were treated using preputial flaps: 36 with a tubularized preputial island flap (TIF) and 36 an onlay island flap (OIF). Fifty-eight patients (45%) underwent staged repairs: Belt-Fuquà (BF) in 18 and Bracka procedure in 40 cases. After a mean follow up of 16 years (range 6–19) the overall incidence of complications for each technique was: TIF 36%; OIF 33%; BF 25%; two-stage Bracka 7.5%. The most common complication encountered was neo-urethral fistula. AMU occurred in only 5 cases, none with associated distal urethral stenosis, all in the TIF and OIF groups, and all successfully treated by reduction re-do urethroplasty.ConclusionA very small number of the patients operated using preputial island flaps techniques developed AMU. None of the staged repairs developed AMU, and this is the preferred choice in proximal hypospadias when the urethral plate requires division and/or substitution. All cases of AMU resolved after urethral tapering.     

Inguinal hernia in girls: desirability and dangers of bilateral exploration

ABSTRACT. One hundred and seventeen girls with inguinal hernias were the subject of 226 inguinal explorations. Seventy presented with a right-sided hernia, 38 with a left, and nine bilateral. Eight girls with a unilateral hernia had a unilateral exploration; two of these returned with a hernia on the opposite side. In 100 girls with a unilateral hernia, the opposite side was explored with a yield of a significant sac in about 40%, a significant sac having been defined as extending at least 1 cm below the internal ring before traction was applied. This incidence varied little with the age or side of presentation. The ovary and/or tube was seen on the affected side in approximately 25% of presenting hernias, and on the contralateral side in 5%. As this is the one potential source of harm by exploring the contralateral side, it is concluded that the risk is quite low compared with the benefits of the procedure.     

Children with a Pervasive Developmental Disorder, Children with a Language Disorder and Normally Developing Children in Situations with High- and Low-level Involvement of the Caregiver

The social behaviour of tow-functioning children with a Pervasive Developmental Disorder (PDD). high-functioning children with a PDD, children with a Language Disorder (LD) and normally developing children (ND| was investigated in a playroom session while they were iteracting with one of their parents. High-functioning children with a PDD were found to differ from the other groups in social responsivity. Low-functioning children with a PDD differed from high-functioning children with a PDD in the total number of social bids, type of social bids, number of looks at parent and social responsivity. When parents structured the behaviour of their children, this resulted in an increase in number of social bids and looks in the low-functioning children with PDD.     

Isolation of the left subclavian artery from the pulmonary artery in a patient with CHARGE association

A male infant diagnosed with CHARGE association presented with respiratory insufficiency and a cardiac murmur. Diagnostic evaluation showed a right sided aortic arch with an isolation of the left subclavian artery arising from the pulmonary artery through a left patent arterial duct. This is a rare cardiac disorder not previously described in patients with CHARGE association, detected with a 3D computed tomography scan, which emphasizes the importance of a thorough cardiac screening in patients with CHARGE association.     

Hypopituitarism and neurodevelopmental abnormalities in relation to central nervous system structural defects in children with optic nerve hypoplasia

OBJECTIVE: Optic nerve hypoplasia (ONH) is a heterogeneous disease with variable findings of pituitary insufficiency, CNS and neurodevelopmental abnormalities. We characterized the spectrum of endocrinopathy in a cohort of children with ONH and attempted to correlate the presence of different midline CNS findings with the degree of hypopituitarism. The correlation of variable CNS abnormalities with the presence of a seizure disorder and neurodevelopmental delay was also examined. METHODS: Charts of 56 patients with ONH referred to our endocrine clinics between 1990 and 2000 were reviewed. Neurodevelopmental assessment was based on questionnaires sent to families during the study period. RESULTS: Forty-six patients (82%) had hypopituitarism, with growth hormone deficiency being the most common endocrinopathy. All patients with diabetes insipidus, hypocortisolism, and hypogonadotropin hypogonadism also had combined pituitary hormone deficiency. Evolving pituitary hormone deficiency was observed in two of 37 patients diagnosed with hypopituitarism in the first 3 years of life. No single midline CNS finding correlated with the presence of hypopituitarism or a seizure disorder. However, hydrocephalus or a seizure disorder was more commonly seen in patients with developmental delay. CONCLUSION: ONH is a heterogeneous disorder with possible multifactorial etiologies. All patients with this diagnosis deserve a comprehensive endocrine and neurodevelopmental evaluation.     

Pediatric split liver transplantation after Fontan procedure in left isomerism combined with biliary atresia: A case report

LI is a subset of the heterotaxy syndrome and a rare birth defect that involves the heart and other organs. It can be combined with extracardiac abnormalities, especially BA. CHD can be associated with LI in up to 15% of cases, although it is rare in BA. Pediatric LT for a child with ESLD due to BA combined with LI and CHD is a challenging issue for a transplant surgeon. Herein, we report a successful split LT on a three‐yr‐old boy with LI who survived after a Fontan procedure due to single ventricle, but who suffered from HPS associated with BA.     

Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach

Two patients with incomplete pentalogy of Cantrell are described. The first was a girl with a large omphalocele with evisceration of the heart, liver and intestines with an intact sternum. Echocardiography showed profound intracardiac defects. The girl died 33 h after birth. The second patient was a female fetus with ectopia cordis (EC) without intracardiac anomalies; a large omphalocele with evisceration of the heart, stomach, spleen and liver; a hypoplastic sternum and rib cage; and a scoliosis. The pregnancy was terminated. A review of patients described in the literature is presented with the intention of finding prognostic factors for an optimal approach to patients with the pentalogy of Cantrell. In conclusion the prognosis seems to be poorer in patients with the complete form of pentalogy of Cantrell, EC, and patients with associated anomalies. Intracardial defects do not seem to be a prognostic factor. Statement of financial support: no financial assistance was received.