Antenatally Detected Choledochal Malformation: What Are We Waiting For?

INTRODUCTION: An increasing proportion of congenital choledochal malformation (CCM) are being detected on antenatal ultrasound. However, the actual timing of its surgical correction remains controversial with some series showing an excess of complications the earlier the operation. The aim of this study was to characterize the pathophysiological aspects of this cohort from the perspective of age at surgery in order to inform a more rational basis for clinical decision-making. METHODS: We analysed a prospective database of CCM acquired over a 26-year period (Jan. 1997 to Dec. 2022) for patient demography; details of pre- and post-natal imaging; age at surgical intervention; operative complications (Clavien-Dindo classification) and outcome. Data are quoted as median (IQR). All comparisons are non-parametric. A P value of 0.05 was accepted as significant. RESULTS: There were 58 (72% female) children with an antenatally-detected CCM from a total of 265 (21.8%) in the series. These were classified as Type 1C (n = 47; 81%), type 4C (n = 3; 5%) and Type 5 (n = 8; 14%). There were no Type 1F lesions in this cohort. Median age at surgery was 113 (IQR 57-198) days. Postnatal cyst (US) size varied from 12 to 130 mm but there was little evidence of a relationship between this and post-natal liver biochemistry (e.g. bilirubin rS = 0.01, P = 0.44; AST rS = 0.14, P = 0.19). Choledochal pressure measurements (n = 46) showed resting pressures of 12 (9-21) mmHg with no significant correlation with age (P = 0.4) or aspartate aminotransferase (P = 0.2) or γ-glutamyl transferase (P = 0.06). The cohort was divided into 2 groups (Early and Late) based on the median age at surgery (all open) (113 days). Biliary obstruction was more common in the Early group (10 vs. 2; P = 0.01). Two perforations occurred, both in the Early group. With a deliberate policy of regular ultrasound-based follow-up we have seen no anastomotic complications (leak, stenosis, persistent intrahepatic biliary dilatation or stones) or post-operative cholangitis in any child [median follow-up 3.42 (1.30-8.05) years]. CONCLUSIONS: This is one of the largest series documenting the outcomes of antenatally-detected CCMs, certainly in Europe and North America. Such lesions are invariably cystic in nature, and either Types 1C, 4C or 5. The absence of complications using a policy of early intervention (where possible) in experienced hepatobiliary units was shown. EVIDENCE LEVEL: II.

Fetal ascites in cloacal malformations-a red flag.

INTRODUCTION: Cloacal malformation is a rare anomaly that remains a diagnostic challenge prenatally, despite the current advances in ultrasonography and MRI. This condition can in some, present with isolated ascites or with other findings, such as a pelvic cyst or upper urinary tract dilatation. In a minority, the ascites may be progressive, questioning the role of antenatal intervention. METHODS: We report on ten patients that have been identified from our Cloaca database between 2010 and 2022. RESULTS: The presence of ascites was associated with extensive bowel adhesions and matting, leading to a challenging initial laparotomy and peri-operative course. CONCLUSIONS: Antenatal finding of ascites in newborns with cloacal malformations should raise a red flag. The surgeon and anaesthetist should be prepared for the operative difficulties secondary to bowel adhesions and the higher risk of haemodynamic instability at the initial surgery. An experienced team at initial laparotomy in such patients is vital. LEVEL OF EVIDENCE: II.

Prepubertal gynecomastia is not always idiopathic: case series and review of the literature.

Although pubertal gynecomastia is a common clinical presentation of adolescent males, prepubertal gynecomastia is uncommon and mostly idiopathic. However, pathological causes of prepubescent gynecomastia are encountered in clinical practice. This manuscript carries an important message to general pediatricians, to care about exclusion of pathological causes for every patient of prepubertal gynecomastia. We present four different patients with pathological gynecomastia. One of them revealed to be secondary to Sertoli cell tumor, while the second patient describes trauma as a rare cause of prepubertal true gynecomastia. To the best of our knowledge, this is the first time to report occupational trauma as a cause of true gynecomastia as confirmed by pathological specimen, in a prepubertal boy. The third patient presented with retro-areolar mass and bloody nipple discharge secondary to mammary duct ectasia and had favorable self-limited course. Hyperprolactinemia secondary to neglected congenital hypothyroidism was the cause beyond gynecomastia in the fourth patient and this cause has been reported only once in the literature.Conclusion: Despite being rare, pathological causes of prepubertal gynecomastia are encountered in clinical practice, and full investigations including breast and testicular ultrasound are needed to exclude any pathology before diagnosing idiopathic gynecomastia. Repeated friction of the breast can lead to true gynecomastia not only to pseudogynecomastia as previously known. What is Known: • It has been reported that trauma can cause pseudogynecomastia due to hematoma or fat necrosis. • Prepubertal gynecomastia is mostly idiopathic. What is New: • Long-term breast trauma can cause true gynecomastia (adenosis). • Although being mostly idiopathic, pathological causes of prepubertal gynecomastia must be ruled out.

Post Circumcision Intraperitoneal Rupture of the Urinary Bladder: A Rare Complication.

Circumcision is one of the most common pediatric surgical procedures performed all over the world and especially in Arab and Islamic countries. Many complications have been documented following this maneuver. We report on a rare case of intraperitoneal bladder rupture in a 7-day-old baby who was circumcised on his second day using the guillotine method. He presented to us with gangrene of the tip of the penis and a failure to void urine associated with progressive abdominal distension. Ultrasound revealed severe ascites. Aspiration and analysis confirmed the fluid to be urine. Ascending cystourethrogram was performed revealing a perforation of the posterior bladder wall near the trigone. Exploration was performed and repair done. Postoperative course was uneventful.