Surgical correction of epispadias associated with buried penis.

INTRODUCTION AND IMPORTANCE: Isolated male epispadias is a rare entity with incidence of approximately 1 in 120,000 live births. Epispadias usually presents with a phimotic preputial orifice where glans is not visible and hence is also known as concealed epispadias. Buried penis in children is defined as a congenital insufficient penile skin with an unretractable foreskin that keeps the penis deep inside the pre-pubic fat. This congenital malformation of the penile envelopes is usually isolated. However, in some cases the concealed penis hides an underlying penile anomaly. We present surgical repair of a very rare case with concealed epispadias. CASE PRESENTATION: A nine-month-old infant had buried his penis, and his mother was seeking his circumcision. Local examination revealed concealed penopubic epispadias. A pediatric surgeon operated on this patient using the modified partial penile disassembly technique. The patient was doing well at follow-up visits at one, three, and six months. There were no urethral stricture or obstructive urinary symptoms. The parents were satisfied with the cosmetic outcome. CLINICAL DISCUSSION: The embryogenesis and development of the urethra and the prepuce are linked. Urethral development defects (as in hypospadias or epispadias) are frequently coupled with faulty prepuce on the same side. The goal of surgical management for epispadias is to correct the dorsal chordee and reconstruct the epispadiac urethra and glans. Based on the cosmesis of the penis reconstruction, preservation of erectile function, and achieving urine continence, the outcome is evaluated. CONCLUSION: Concealed epispadias is frequently ignored because patients appear with buried penis, non-retractile prepuce, and a normal urine stream. Preoperative diagnosis and parent counseling are critical for the effective treatment of this uncommon entity. The modified partial penile disassembly procedure, in which the tunica albuginea is stitched to the pubic periosteum at 3 and 9 o'clock, can be used to correct buried epispadias.

Chylolymphatic cyst as a rare cause of acute intestinal obstruction in infants.

INTRODUCTION AND IMPORTANCE: Chylolymphatic cysts are benign pathology in lymphatic vessels due to obstruction in the lymphatic system. Their incidence is still unknown. However, they account for approximately 3 % to 9 % of all pediatric lymphangiomas. Most of these lesions are asymptomatic and are discovered accidentally. They can present with non-specific abdominal pain, vomiting and changes in bowel habits. Rarely, Chylolymphatic cysts can cause fatal complications such as intestinal obstruction, volvulus or even torsion. CASE PRESENTATION: A 3-month-old male presented to our unit with manifestations of acute intestinal obstruction. Abdominal examination revealed signs of generalized peritonitis and intestinal obstruction. Abdominal ultrasound revealed minimal turbid intraperitoneal collections and no motility of bowel loops. Plain erect abdominal radiograph revealed multiple small bowel air fluid levels. Abdominal exploration revealed a large Chylolymphatic cyst causing acute intestinal obstruction. Excision of the cysts was done with preservation of adjacent bowel loop. The patient was discharged in good health on the 6th day postoperative. A chylolymphatic cyst with a diameter of 5 cm was identified by histological investigation. CLINICAL DISCUSSION: Mesenteric cyst is a rare intra-abdominal tumor with prevalence of about 1: 20,000 in pediatric age group. They are benign cystic pathology lined with a thin endothelium or mesothelium occur due to gross proliferation of isolated or ectopic lymphatics in mesentery that lack communication with the rest of the lymphatic system. Regarding pathology, mesenteric cysts are classified into four types including type 1 (Pedicled) and type 2 (Sessile), which are limited to the mesentery, hence can be excised completely with or without resection of the involved gut. Type 3 and type 4 are multicentric, so they require complex surgery and often sclerotherapy because of their extension into retroperitoneum. The majority of mesenteric cysts may present as asymptomatic abdominal mass or incidental finding on imaging or during laparotomy for other abdominal conditions. Preoperative diagnosis of mesenteric cyst in most cases is difficult as they have no definite characteristic features. Abdominal ultrasound which is the imaging modality of choice can reveal mesenteric cysts as cystic tumors. Surgical removal of the cyst is the standard treatment for chylolymphatic cysts. CONCLUSION: Most Chylolymphatic cysts in infants are asymptomatic, but they can cause fatal complications such as intestinal obstruction, volvulus or even torsion.

Spontaneous evisceration of infantile umbilical hernia.

INTRODUCTION AND IMPORTANCE: Infantile umbilical hernia is common in children. It has a regressive course in most cases. Conservative management is the standard in most cases before the age of 3 years unless there are complications such as incarceration, rupture with evisceration which are extremely rare and warrants emergency surgery. CASE PRESENTATION: Our case was a full term 6-month-old male of normal birth weight with history of umbilical hernia but with no obvious risk factors to develop complications. The loops evisceration was spontaneous with a small umbilical skin damage. The poor parental consultation on early surgical management and delayed presentation of the infant after evisceration could be the possible risks for ischemic changes and shock state at the time of presentation, however, prompt medical resuscitation and surgical management relatively improved postoperative outcomes. CLINICAL DISCUSSION: Infantile umbilical hernia is considered one of the most encountered abnormalities of infancy. Most umbilical hernias are asymptomatic and discovered after birth. Complications of infantile umbilical hernia as incarceration or spontaneous evisceration are very rare but fatal. Certain factors increase the risk for developing spontaneous rupture of infantile umbilical hernia including the age of the infant or child, the defect size, umbilical sepsis or ulceration and any condition which raises intra-abdominal pressure, i.e., crying, coughing or positive ventilation. CONCLUSION: Although infantile umbilical hernia is clinically benign condition with a regressive course in majority of cases, the risk of rupture of an umbilical hernia is exceedingly rare in pediatric population; physicians should be warranted with the possible risk factors for spontaneous rupture and in these patients expedite surgical repair.

Perforated gallbladder in neonates with review of literature.

INTRODUCTION: Neonatal perforated gallbladder is a rare and implausible surgical emergency. Pneumoperitonium and neonatal intestinal obstruction are the main clinical presentations. Many cases of neonatal perforated gallbladder had no clear pathology. Most proper treatment is cholecystectomy during formal exploration. CASE PRESENTATION: We reported a case of male neonate in his first days of life, presented with abdominal distention and pneumoperitonium and so, surgical exploration revealed perforated gallbladder for which cholecystectomy done. CLINICAL DISCUSSION: Idiopathic gallbladder perforation is seldom condition and is hard to discover before exploration. Besides, pathogenesis remains unknown. In our presented case, the real cause of perforation was unknown and main presentation was pneumoperitonium. CONCLUSION: Although perforated gallbladder is a rare entity and in most of cases does not cause pneumoperitonium, but perforated gallbladder should be taken in account of all cases of pneumoperitonium.

Use of antenatal fluorinated corticosteroids in management of congenital heart block: Systematic review and meta-analysis.

OBJECTIVE: To evaluate outcomes of fluorinated corticosteroids, with or without other medications, for treatment of congenital heart block in-utero. STUDY DESIGN: A search was conducted through MEDLINE, EMBASE, WEB OF SCIENCE and SCOPUS from inception to October 2017. Only comparative studies are considered eligible. Outcomes include fetal death, downgrade of heart block, neonatal death, need for neonatal pacing, fetal and maternal complications. Random effects model was used. RESULTS: Out of 923 articles, 12 studies were eligible. Compared to no treatment, there was no significant difference in incidence of fetal death (OR 1.10, 95%CI 0.65-1.84), neonatal death (OR 0.98, 95%CI 0.41-2.33), or need for pacing (OR 1.46, 95%CI 0.78-2.74). Heart block downgrade was significantly higher in treatment group (9.48%vs.1.76%, OR 3.27, 95%CI 1.23-8.71). CONCLUSION: antenatal fluorinated corticosteroids do not improve fetal/neonatal morbidity or mortality of congenital heart block and are associated with higher incidence of fetal and maternal complications.