Scrotoschisis in a newborn: A case report

Abstract

Scrotoschisis is a rare congenital malformation characterized by the externalization of one or both testicles. The etiology is poorly understood. We report the case of a newborn admitted at 5 days of live presenting with externalization of both testicles. Reintegration followed by bilateral orchidopexy was performed. The postoperative course was uneventful. After 18 months, the patient presented bilateral inguinal hernia. Early and well-managed treatment prevented complications.

1. Introduction

Scrotoschisis is a very rare congenital malformation characterized by a defect of the scrotum. The defect affect anterolateral part of all layers of the scrotum, causing the testicle to appear outside the scrotal limits.¹ First described in 1963 by Von Der Leyer, its prevalence remains unknown.² In the literature, around thirty cases have been reported. More than half of these cases come from sub-Saharan Africa,³ including one case in Côte d'Ivoire to our knowledge.⁴ We report a case of scrotochisis followed up in our hospital and a review of the literature concerning management and progression.

2. Case report

A 5 days male newborn, referred by tertiary health center for scrotal malformation, noted at birth. He was born to a 27-year-old mother, primigravida and primipara, at home, with four high-quality prenatal consultations. No antenatal diagnosis was made on antenatal ultrasound. Birth weight was 2100 g, 45 cm of length and 30 cm of head circumference. The parents reported applying poultice.

Newborn was in a good general condition, primitive reflexes preserved, and normal temperature with a skin recolouration rate of less than 3 seconds.

Physical examination of external genitalia revealed a normal size penis (3,2 cm) and a scrotal defect at the median raphe, with clear edges measuring 2 cm/1 cm, exposing the two testicles, which were covered with whitish deposits (Fig. 1).

Fig. 1.

Local view showing the externalised testicles.

Biological assessment carried out, namely blood count, prothrombin and activated cephalin time, was normal. Abdominal and cardiac ultrasound scans to check for other malformations were normal.

Under general anaesthesia, we performed asepsis followed by reintegration of the testicles, bilateral orchidopexy and scrotal suturing (Fig. 2). The immediate postoperative period was uneventful.

Fig. 2.

Postoperative picture showing scrotal closure.

At 18 months, healing was good and the testicles were present in the scrotum and of normal size. The patient presented with an uncomplicated bilateral inguinal hernia (Fig. 3).

Fig. 3.

Patient presenting with bilateral inguinal hernia after an 18-month follow-up period.

Scrotal ultrasound showed testicles of normal size and echostructure.

3. Discussion

Scrotochisis is a rare condition in newborns, and its incidence remains unknown. It is a urological malformation characterised by the externalization of one or both testicles through a scrotal defect.¹

Several cases have been reported, but its etiology is poorly understood and several theories have been proposed.^5,6 According to Balla,⁷ meconium periorchitis is the most widely accepted theory, as suggested by Kojori. However, other theories have suggested a failure of lip fusion, hyperactive phagocytosis of Von Der Leyen's gubernaculum, failure of cremasteric fibre or Gupta's mesenchyme development, infection or localised Heyns' ischaemia, and iatrogenic scrotal laceration during caesarean section. Our case presented with a scrotal defect with regular edges and an absence of greenish deposits suggestive of meconium.^1,7

This anomaly is generally isolated like our case. Some authors have described associated forms.

Lais et al.⁸ reported a case associated with jejunal atresia and arthrogryposis.

Management consisted of testicular reintegration, bilateral orchidopexy and scrotal suturing under general anaesthesia, as described in the literature. However, management under local anaesthesia may be an option.⁹

The immediate postoperative course was favorable, as described in the literature.^4,7 Certain complications such as infection, testicular torsion or testicular necrosis may occur, hence the importance of monitoring the condition of the testicle after treatment.^3,10 Uncomplicated bilateral inguinal hernia was observed during follow-up, which was not observed in other cases in the literature.

The functional prognosis of the testicles remains unknown as no studies have been conducted in this area.

4. Conclusion

Scrotoschisis is a rare urogenital malformation whose etiology remains poorly understood. Urgent treatment can prevent complications. The prognosis was favorable in our case, with a testicle of normal size. We found a persistent peritoneal-vaginal canal in our case. However, the functional prognosis of the testicles remains to be evaluated in the long term.

CRediT authorship contribution statement

Koffi Jean-Marie Konan: Conceptualization, Data curation, Methodology, Writing – original draft. Agnime Reine Christine Akproh: Investigation, Methodology, Visualization. Helen Audrey Thomas Danho: Visualization, Writing – review & editing. Kouame Soroboua Agbara: Validation, Writing – review & editing. Kouakou Jules N'guessan: Writing – review & editing. Adjoba Manuela Ehua Koua: Supervision, Writing – review & editing. Martial Olivier Moulot: Supervision. Roumanatou Sanni Bankole: Supervision.

Patient consent

Consent to publish the case report was not obtained. This report does not contain any personal information that could lead to the identification of the patient.

Authorship

All authors attest that they meet the current ICMJE criteria for Authorship.

Funding

No funding or grant support.

Declaration of competing interest

The following authors have no financial disclosures: (K J M K, A R C A, H A T D, K S A, K J N, A M E K, M O M, R S B).