Delayed Presentation of Malrotation: Case Series and Literature Review.

Background: Intestinal malrotation is a congenital anomaly resulting from abnormal or incomplete rotation and fixation of the midgut during embryogenesis. It commonly presents in the neonatal period (75%) with sudden onset bilious vomiting and rarely beyond infancy (<10%). Aim: The aim of the study was to highlight the clinical features, radiological findings, and treatment outcomes of patients with malrotation presenting beyond infancy. Materials and Methods: Eleven consecutive cases of delayed presentation of malrotation presented over a period of 5 years (2017-2021). Data were analyzed retrospectively. Results: Out of the 11 patients, four were female and seven were male. The age of patients ranged from 14 months to 18 years. Patients beyond infancy present usually with diffuse pain abdomen compared to neonates which present with sudden onset bilious vomiting and therefore difficult to diagnose. Five patients had associated abnormalities such as intussusception or nutcracker syndrome or mesenteric cyst or jejunal stricture or mesenteric lymphadenopathy along with malrotation. Patients underwent ultrasonography, upper gastrointestinal contrast study, and contrast-enhanced computed tomography abdomen to confirm the diagnosis. All patients underwent the Ladd procedure with four requiring resection anastomosis and one requiring excision of the mesenteric cyst. Eight out of eleven patients had favorable outcomes, two develop adhesive intestinal obstruction and required re-exploration, and one had persistent complaints of hematochezia. Conclusion: Malrotation beyond infancy is a rare diagnosis. Malrotation in older children is usually not suspected because of the wide range of symptoms. A high index of suspicion on ultrasound or computed tomography is required to demonstrate the reversal of superior mesenteric artery and superior mesenteric vein position and related conditions. Early intervention and treatment can prevent catastrophic events such as intestinal volvulus and intestinal ischemia in these patients.

A rare presentation of transverse testicular ectopia and role of laparoscopy.

Transverse testicular ectopia (TTE) is an uncommon congenital anomaly, in which both the gonads migrate towards the same hemi-scrotum. We report a rare case of TTE discovered coincidentally during surgery in a 1-year-old boy who presented with bilateral undescended testis and right inguinoscrotal swelling. The use of laparoscopy during evaluation for impalpable testis can aid in the diagnosis of TTE, before inguinal exploration. Findings such as the vascular supply and vas deferens of the crossed testis and the identification of Müllerian structures can be confirmed by laparoscopy. Diagnostic laparoscopy was suggestive of both the testes entering in the right-sided open deep inguinal ring, while left-sided deep inguinal ring was found to be closed. There was no evidence of persistent Müllerian structures. On exploration, both testes were present in the right inguinal region. A bilateral trans-septal orchidopexy along with right herniotomy was performed.

Anatomical study of hypospadias penis using magnetic resonance imaging in children.

INTRODUCTION: To assess the various anatomical patterns of the hypospadias penis, anatomical and histological study of the penile tissues, planes, and vascular patterns, and imagings such as ultrasound of penis, elastography, and Magnetic resonance imaging (MRI) of penis have been described in the literature. All these have been done to attempt the identification of anatomical variations that may influence surgical outcomes. There are very limited MRI studies of hypospadias penis to look for the pristine anatomy. OBJECTIVE: The objective was to identify anatomical variations in hypospadias penis such as the penile tissues and planes and the vascularity using MRI. MATERIAL AND METHODS: The total number of patients enrolled was 24 from January 2019 to July 2020. This included all the cases of hypospadias at any location aged ≥5 years. MRI penis was done using 3T (3 Tesla) MRI scanner (GE Healthcare signa 3T Scanner machine) with 3 mm body coil slice thickness and the surface coil of 3 inches. Non-contrast images were taken using fast spin-echo sequences in sagittal, coronal, and transverse planes. The findings analyzed were: presence and distribution of penile tissue and fascial structures, urethral plate thickness, and penile vasculature. RESULTS: The mean age was 7.62 ± 2.14 years. The types of hypospadias included were Coronal 1/24 (4.2%), Subcoronal 14/24 (58.3%), Distal penile 3/24 (12.5%), Midpenile 5/24 (20.8%) and Penoscrotal 1/24 (4.2%) (Summary Table 1). The mean urethral plate thickness was 1.33 ± 0.38 mm. The penile soft tissues were well visualized along with their fascial planes. The majority of patients (91.7%, 22/24) had Superficial Dartos vessels with both branches. Bulbourethral vessel was present in 18 (75.0%) cases but could not be visualized in the rest. Ventral and Lateral Dartos vessels were seen in 20 (83.3%) cases. Perforators distal to meatus were visualized in 21 (87.5%) cases and not visualized in 3 (1 each in Penoscrotal, Midpenile, and Coronal hypospadias). Collaterals at corona sulcus were visualized in 23 (95.8%) cases, at paraurethral spongiosum in 15 (62.5%) cases, and at dorsum in 22 (91.7%) cases. CONCLUSION: 3T MRI gives precise images in hypospadias with relation to the tissue and fascial planes of the penis. The vascular pattern visualization in these patients may be confirmed by the availability of a dedicated penile coil which will help to improve the resolution of the penile structures. Analyzing the penile vascular pattern and correlating it with surgical outcomes may aid the surgeon's knowledge of hypospadias, develop new surgical techniques and hence reduce complications.

Large falciform ligament cyst in a child: A rare entity of peritoneal cysts and review of the literature.

Peritoneal cysts are not uncommon in children - mesenteric/omental cysts being the commoner entity. Peritoneal cysts in the falciform ligament are a very rare entity reported in the literature. We present a 5-year-old boy who presented with pain upper central abdomen and few episodes of non-bilious vomiting for 1 year. He was stable on examination, with abdominal examination revealing the fullness of the abdomen with palpable generalised cystic mass which was mobile transversely. Ultrasound and contrast-enhanced computed tomography of the abdomen revealed intra-peritoneal cystic lesion measuring 13 cm × 11.5 cm × 9 cm with septations seen in the abdominal cavity from the epigastric region to the infraumbilical region (D11-L5 level). Laparoscopy showed a large cyst of the abdomen arising from the anterior abdominal wall, along the falciform ligament. The dark yellow fluid was aspirated and the cyst was excised leaving a part which was adherent to the anterior wall. Histopathology revealed cuboidal epithelium lined by the fibrous wall. The patient is doing well at 6 months follow-up. Falciform ligament cysts are very rare and laparoscopy can confirm the diagnosis as well as help in excision of the cyst with good results.

Role of uroflowmetry before and after hypospadias repair.

AIMS: To study the role of uroflowmetry in the preoperative and early postoperative period in children undergoing hypospadias repair. MATERIALS AND METHODS: Twenty-six cases undergoing hypospadias repair over 1 year (tubularized incised plate [TIP] Snodgrass [17 patients], TIP with Snodgraft [5 patients], Duckett's onlay flap [2 patients], and Duckett's tube [2 patients] urethroplasty) were prospectively evaluated with preoperative ultrasound and uroflowmetry and postoperative uroflowmetry at 3 months after the surgery and at 6 and 9 months interval if these dates fell within the study period on follow-up. The parameters studied were maximum flow rate (Qmax), average flow rate (Qav), total voided volume, voiding time, and type of curve. Preoperative and postoperative uroflow data were compared. RESULTS: Twenty-six cases comprised of anterior hypospadias (n = 8), mid penile (n = 11), and posterior hypospadias (n = 7). Fourteen patients had obstructed flow rates preoperatively. While 69% patients (18/26) had obstructed flow rates at 3 months postoperatively, it dropped to 43% at 9 months. Following TIP (Snodgrass) repair, 88% (15/17) had obstructed flow rates postoperatively. Best results were seen in patients undergoing circumferentially epithelialized urethral reconstruction (TIP with Snodgraft, Duckett's onlay flap, and Duckett's tube). CONCLUSIONS: Abnormal uroflow is an inherent aspect of hypospadias in 50% of the cases. Both preoperative and postoperative uroflow evaluation is necessary for meaningful conclusion. Patients with preoperative normal flow rates but obstructed postoperative flow rates need clinical evaluation. Obstructive flow rates are more common after TIP (Snodgrass) repair. The urinary flow rates improve with time.

Congenital Duodenal Obstruction, Situs Inversus Totalis, and Gastric Perforation in a Neonate.

We report a rare case of incomplete congenital duodenal obstruction (Type 1 duodenal atresia) in association with situs inversus totalis presenting with gastric perforation in a neonate. The infantogram was suggestive of perforation with air under diaphragm along with dextrocardia. On exploration, a pin point perforation at fundus near lesser curvature along with situs inversus was noted. Primary closure of gastric perforation was done. Patient was then discharged on full breast feeds but was readmitted with intolerance to feeds and recurrent bilious vomiting. Further, upper GI contrast study revealed partial duodenal obstruction. On re-exploration, duodenal web with central aperture was seen and duodeno-duodenostomy was done.

Mesenteric Cyst in Association with Type-II Jejunoileal Atresia.

A rare case of type-II jejunoileal atresia with mesenteric cyst in a neonate is being reported here with a brief review of literature.