E-ISSN 1858-8360 | ISSN 0256-4408
 

Case Report 


SUDANESE JOURNAL OF PAEDIATRICS

2021; Vol 21, Issue No. 2

CASE REPORT

Accessory scrotum with perineal lipoma/hamartoma mimicking penoscrotal pseudoduplication

Paul Anthony Heaton (1) ORCID logo, Mohamed Sameh Shalaby (2) ORCID logo, Camelia Laura Vaina (1) ORCID logo, Sharon Elizabeth Brown (3), Elise Gradhand (4) ORCID logo, Siba Prosad Paul (1) ORCID logo

(1) Department of Paediatrics, Yeovil District Hospital, Yeovil, UK

(2) Department of Paediatric Surgery, Bristol Royal Hospital for Children, Bristol, UK

(3) Department of Radiology, Yeovil District Hospital, Yeovil, UK

(4) Department of Paediatric Histopathology, Bristol Royal Hospital for Children, Bristol, UK

Correspondence to:

Dr Siba Prosad Paul

Consultant Paediatrician, Level 10, Department of Paediatrics, Yeovil District Hospital, Higher Kingston, Yeovil, BA21 4AT, (UK)

Email: siba [at] doctors.org.uk

Received: 13 August 2020 | Accepted: 22 May 2021

How to cite this article:

Heaton PA, Shalaby MS, Vaina CL, Brown SE, Gradhand E, Paul SP. Accessory scrotum with perineal lipoma/hamartoma mimicking penoscrotal pseudoduplication. Sudan J Paediatr. 2021;21(2): 219–223.

https://doi.org/10.24911/SJP.106-1597356690

ABSTRACT

Congenital anomalies of the scrotum are thought to be caused by an abnormal migration of the labioscrotal swelling during the embryological process. We report the case of an infant in whom suspected penoscrotal pseudoduplication was apparent at delivery. Imaging using ultrasound scan and magnetic resonance imaging allowed anatomical delineation of the abnormality and the appropriate surgical intervention to be determined. Full surgical correction was done at 4 weeks of age with good outcome. We discuss the differential diagnosis and the management of congenital scrotal abnormalities.


KEYWORDS

Perineal lipoma/hamartoma; Penoscrotal pseudoduplication; Sacrococcygeal teratoma; Diagnostic dilemma.


INTRODUCTION

Congenital anomalies of the scrotum are uncommon, and are thought to be caused by an abnormal migration of the labioscrotal swelling, though the exact embryological process remains unclear [1]. Four categories of abnormalities are described: penoscrotal transposition, bifid scrotum, ectopic scrotum, and accessory scrotum; the latter two anomalies being extremely rare [1]. Perineal lipomas are known to be associated with an accessory scrotum in >80% of cases [1]. This paper describes a case of accessory scrotum with perineal lipoma or hamartoma, and the associated challenges in management.


CASE REPORT

A male infant born at 34 weeks gestation with a birth weight 1.96 kg was admitted to the Special Care Baby Unit for supporting his feeds and temperature regulation. There were no maternal risk factors for infection or family history of any congenital abnormality. Antenatal ultrasound scan at 20 weeks gestational age had been unremarkable.

Perineal examination showed suspected penoscrotal duplication located in the midline, posterior to anatomically normal male genitalia and anus (Figure 1). There were no other abnormalities found on neurological or abdominal examination, and the baby was otherwise well.

Ultrasound examination (Figure 2) showed normal renal tract with homogenous hyperechoic tissue identified within the accessory scrotum; no communication or connection was shown between the accessory scrotum and the anus or bladder. Testes were identified within the anterior (normally situated) scrotum. He was discharged home at 2 weeks of age with plan for further investigations and surgical management of the anomaly.

Pelvic magnetic resonance imaging (MRI) scan performed at 3 weeks showed a predominantly external lesion demonstrating high T1 and T2 signal with complete loss of signal on fat saturation images. The mass was confined to the intra-gluteal region, contained no solid or cystic components, and with no post-contrast enhancement (Figure 3). The remainder of the pelvic structures were normal.

At 4 weeks, the lesion was resected with the coccyx completely en bloc through an elliptical perineal wound which was closed in the midline natal cleft. The postoperative period was uneventful. Histology demonstrated the mass to be comprised of mature adipose tissue with some intersecting fibrous septa, vessels and nerve trunks; no immature, teratomatous, or malignant tissue was seen, and the resection margins were clear. The histopathological diagnosis was fibrolipoma/lipoma with the presence of some sort of organisation showing mature adipose tissue organised somehow by intersecting fibrous septa, the lesion is also representing a perineal hamartoma forming an accessory scrotum (Figure 4). He was reported to be well at a clinic review 8 weeks later.

Figure 1. Appearance immediately after birth.

Figure 2. Longitudinal ultrasound scan showing homogenous hyperechogenicity consistent with fatty tissue.

Figure 3. Pelvic MRI scan (A) sagittal and (B) coronal post-contrast fat saturated T1 weighted images showing homogenously low (saturated) signal suggestive of fat. No solid or enhancing elements.

Figure 4. Histology showing features of perineal fibrolipoma/lipoma. (A) Haematoxylin and eosin (H+E) stain×2, (B) H+E stain×10.


DISCUSSION

This case demonstrates the challenges of managing congenital abnormalities of the scrotum. Our patient was initially considered to have penoscrotal duplication as the appearance of the abnormality closely resembled that of an incompletely formed duplication of male genitalia. Penoscrotal duplication is an extremely rare anomaly affecting approximately one in every 5 million births, in which there is duplication of the phallus frequently associated with variable abnormalities of the scrotum, bladder and other ano-rectal anomalies [2-5]. Accessory scrotum is characterised by additional scrotal tissue, containing no testes, which is located beside a normally developed scrotum [1]. Absence of associated anomalies, combined with ultrasonography and MRI findings, indicated that lipoma or sacrococcygeal teratoma were diagnostic possibilities in our case, with the former being confirmed on histology following complete surgical excision.

Congenital perineal lipoma/hamartoma is considered to be extremely rare though there is no published incidence data [6]. The terms lipoma and hamartoma, once there is no central core of hyaline cartilage, are difficult to distinguish histologically; the differentiation between perineal lipoma and hamartoma in this context has no clinical relevance [7]. As the appearance may mimic that of external male genitalia, when congenital perineal lipoma/hamartoma occurs in a female infant, the condition may be mistaken for ambiguous genitalia [8]. A previous case report with both perineal lipoma and accessory scrotum, confirmed by histological examination was described [9]. Fathaddin [10] reported a rare case of accessory scrotum with congenital perineal lipoma in association with type 2 congenital pulmonary airway malformation. Most published reports of congenital perineal lipoma describe cases that have been diagnosed after delivery, though antenatal diagnosis on ultrasonography has also been described [11,12].

Differential diagnosis of a congenital perineal mass in the newborn includes hamartoma, sacrococcygeal teratoma, haemangioma, partial or complete genital duplication, lipoma, lipoblastoma, and incomplete conjoined twins [1,13,14]. Radiological imaging using ultrasound and MRI scan should be undertaken to delineate the internal urogenital and anorectal anatomy and to identify other associated abnormalities [13]. In cases where perineal lipoma is suspected, complete excision is recommended; the lesion is usually benign and does not recur [13].


CONCLUSION

Congenital perineal lipoma/hamartoma with accessory scrotum can cause diagnostic dilemma before specialist referral. There should be early discussion between relevant specialists, and radiological imaging to determine the precise anatomy and likely diagnosis, and to exclude any associated anomalies. The outcome of perineal lipoma/hamartoma (without additional abnormalities) after surgical excision is usually good.


ACKNOWLEDGEMENTS

The authors would like to thank the parents for providing consent to publish the case and the photographs of their child.


CONFLICT OF INTERESTS

The authors declare no conflict of interests.


FUNDING

None available for this case study.


ETHICAL APPROVALS

Signed informed consent for participation and publication of medical details was obtained from the parents of this child. Confidentiality of patient’s data was ensured at all stages. The authors declare that ethics committee approval was not required for this case report..


REFERENCES

  1. Park KH, Hong JH. Perineal lipoma in association with scrotal anomalies in children. BJU Int. 2006;98:409–12. https://doi.org/10.1111/j.1464-410X.2006.06244.x
  2. Mirshemirani AR, Sadeghyian N, Mohajerzadeh L, Molayee H, Ghaffari P. Diphallus: report on six cases and review of the literature. Iran J Pediatr. 2010;20:353–7.
  3. Dhua AK, Sinha S, Ratan S, Aggarwal S. Duplication of peno-scroto-testicular unit- a rare form of caudal duplication syndrome. APSP J Case Rep. 2013;4:45.
  4. Shaul DB, Monforte HL, Levitt MA, Hong AR, Peña A. Surgical management of perineal masses in patients with anorectal malformations. J Pediatr Surg. 2005;40:188–91. https://doi.org/10.1016/j.jpedsurg.2004.09.027
  5. Yamaçake KG, Giron AM, Tannuri U, Srougi M. A rare case of perineal hamartoma associated with cryptorchidism and imperforate anus: case report. Einstein (Sao Paulo). 2014;12:234–46. https://doi.org/10.1590/S1679-45082014RC2746
  6. Upadhyaya P, Spilde TL, Tsao K, Ostlie DJ. Anorectal hamartoma in a neonate: a unique anorectal mass. J Pediatr Surg. 2007;42(9):E31–3. https://doi.org/10.1016/j.jpedsurg.2007.06.021
  7. Amann G, Berger A, Rokitansky A. Accessory scrotum or perineal collision-hamartoma. A case report to illustrate a misnomer. Pathol Res Pract. 1996;192:1039–43. https://doi.org/10.1016/S0344-0338(96)80046-X
  8. Guerra-Junior G, Aun AM, Miranda ML, Beraldo LP, Guimaraes Moraes S, Baptista MTM, et al. Congenital perineal lipoma presenting as ambiguous genitalia. Eur J Pediatr Surg. 2008;18;269–71. https://doi.org/10.1055/s-2008-1038366
  9. Chatterjee S, Gajbhiye V, Nath S, Ghosh D, Chattopadhyay S, Das SK. Perineal accessory scrotum with congenital lipoma: a rare case report. Case Rep Pediatr. 2012;2012:757120. https://doi.org/10.1155/2012/757120
  10. Fathaddin AA. Accessory scrotum and congenital perineal lipoma in a child with type 2 congenital pulmonary airway malformation: a report of an unusual. Int J Health Sci (Qassim). 2018;12:85–6.
  11. Ahn KH, Boo YJ, Seol HJ, Park HT, Hong SC, Oh MJ, et al. Prenatally detected congenital perineal mass using 3D ultrasound which was diagnosed as lipoblastoma combined with anorectal malformation: case report. J Korean Med Sci. 2010;25:1093–6. https://doi.org/10.3346/jkms.2010.25.7.1093
  12. Wax JR, Pinette MG, Mallory B, Carpenter M, Winn S, Cartin A. Prenatal sonographic diagnosis of a perineal lipoma. J Ultrasound Med. 2010;29:1257–9. https://doi.org/10.7863/jum.2010.29.8.1257
  13. Periquito IR, Neves CI, Mota FC, Tome T. Congenital perineal lipoma: an unusual presentation. BMJ Case Rep. 2014;2014: bcr2013203495. https://doi.org/10.1136/bcr-2013-203495
  14. Ramzisham AR, Thambidorai CR. Perineal hamartoma with accessory scrotum, anorectal anomaly, and hypospadias-a rare association with embryological significance: case report. Pediatr Surg Int. 2005;21:478–81. https://doi.org/10.1007/s00383-005-1416-7


How to Cite this Article
Pubmed Style

Heaton PA, Shalaby MS, Vaina CL, Brown SE, Gradhand E, Paul SP. Accessory scrotum with perineal lipoma/hamartoma mimicking penoscrotal pseudoduplication. Sudan J Paed. 2021; 21(2): 219-223. doi:10.24911/SJP.106-1597356690


Web Style

Heaton PA, Shalaby MS, Vaina CL, Brown SE, Gradhand E, Paul SP. Accessory scrotum with perineal lipoma/hamartoma mimicking penoscrotal pseudoduplication. https://www.sudanjp.com/?mno=125218 [Access: December 04, 2022]. doi:10.24911/SJP.106-1597356690


AMA (American Medical Association) Style

Heaton PA, Shalaby MS, Vaina CL, Brown SE, Gradhand E, Paul SP. Accessory scrotum with perineal lipoma/hamartoma mimicking penoscrotal pseudoduplication. Sudan J Paed. 2021; 21(2): 219-223. doi:10.24911/SJP.106-1597356690



Vancouver/ICMJE Style

Heaton PA, Shalaby MS, Vaina CL, Brown SE, Gradhand E, Paul SP. Accessory scrotum with perineal lipoma/hamartoma mimicking penoscrotal pseudoduplication. Sudan J Paed. (2021), [cited December 04, 2022]; 21(2): 219-223. doi:10.24911/SJP.106-1597356690



Harvard Style

Heaton, P. A., Shalaby, . M. S., Vaina, . C. L., Brown, . S. E., Gradhand, . E. & Paul, . S. P. (2021) Accessory scrotum with perineal lipoma/hamartoma mimicking penoscrotal pseudoduplication. Sudan J Paed, 21 (2), 219-223. doi:10.24911/SJP.106-1597356690



Turabian Style

Heaton, Paul Anthony, Mohamed Sameh Shalaby, Camelia Laura Vaina, Sharon Elizabeth Brown, Elise Gradhand, and Siba Prosad Paul. 2021. Accessory scrotum with perineal lipoma/hamartoma mimicking penoscrotal pseudoduplication. Sudanese Journal of Paediatrics, 21 (2), 219-223. doi:10.24911/SJP.106-1597356690



Chicago Style

Heaton, Paul Anthony, Mohamed Sameh Shalaby, Camelia Laura Vaina, Sharon Elizabeth Brown, Elise Gradhand, and Siba Prosad Paul. "Accessory scrotum with perineal lipoma/hamartoma mimicking penoscrotal pseudoduplication." Sudanese Journal of Paediatrics 21 (2021), 219-223. doi:10.24911/SJP.106-1597356690



MLA (The Modern Language Association) Style

Heaton, Paul Anthony, Mohamed Sameh Shalaby, Camelia Laura Vaina, Sharon Elizabeth Brown, Elise Gradhand, and Siba Prosad Paul. "Accessory scrotum with perineal lipoma/hamartoma mimicking penoscrotal pseudoduplication." Sudanese Journal of Paediatrics 21.2 (2021), 219-223. Print. doi:10.24911/SJP.106-1597356690



APA (American Psychological Association) Style

Heaton, P. A., Shalaby, . M. S., Vaina, . C. L., Brown, . S. E., Gradhand, . E. & Paul, . S. P. (2021) Accessory scrotum with perineal lipoma/hamartoma mimicking penoscrotal pseudoduplication. Sudanese Journal of Paediatrics, 21 (2), 219-223. doi:10.24911/SJP.106-1597356690





Most Viewed Articles
  • Rheumatic heart disease in North Darfur: an alarmingly high burden and control initiative
    Nagwa Salih, Ishag Eisa, Daresalam Ishag, Intisar Ibrahim, Sulafa Ali
    Sudan J Paed. 2018; 18(1): 24-27
    » Abstract » doi: 10.24911/SJP.2018.1.4

  • Feeding growth restricted premature neonates: a challenging perspective
    Siba Prosad Paul, Emily Natasha Kirkham, Katherine Amy Hawton, Paul Anthony Mannix
    Sudan J Paed. 2018; 18(2): 5-14
    » Abstract » doi: 10.24911/SJP.106-1519511375

  • Congenital brain malformations in Sudanese children: an outpatient-based study
    Inaam Noureldyme Mohammed, Soad Abdalaziz Suliman, Maha A Elseed, Ahlam Abdalrhman Hamed, Mohamed Osman Babiker, Shaimaa Osman Taha
    Sudan J Paed. 2018; 18(1): 48-56
    » Abstract » doi: 10.24911/SJP.2018.1.7

  • Evaluation of Science. [eng]
    Adnan Mahmmood Usmani; Sultan Ayoub Meo
    Sudan J Paed. 2011; 11(1): 6-7
    » Abstract

  • Medical education and services in an extreme environment
    Mustafa Abdalla M. Salih, Mohammed Osman Swar
    Sudan J Paed. 2018; 18(1): 2-5
    » Abstract » doi: 10.24911/SJP.2018.1.1

  • Most Downloaded
  • The role of micronutrients in thyroid dysfunction
    Amir Babiker, Afnan Alawi, Mohsen Al Atawi, Ibrahim Al Alwan
    Sudan J Paed. 2020; 20(1): 13-19
    » Abstract » doi: 10.24911/SJP.106-1587138942

  • Why mothers are not exclusively breast feeding their babies till 6 months of age? Knowledge and practices data from two large cities of the Kingdom of Saudi Arabia
    Hafsa Raheel, Shabana Tharkar
    Sudan J Paed. 2018; 18(1): 28-38
    » Abstract » doi: 10.24911/SJP.2018.1.5

  • Inborn errors of metabolism associated with hyperglycaemic ketoacidosis and diabetes mellitus: narrative review
    Majid Alfadhel, Amir Babiker
    Sudan J Paed. 2018; 18(1): 10-23
    » Abstract » doi: 10.24911/SJP.2018.1.3

  • Relactation in lactation failure and low milk supply
    Anita Mehta, Arvind Kumar Rathi, Komal Prasad Kushwaha, Abhishek Singh
    Sudan J Paed. 2018; 18(1): 39-47
    » Abstract » doi: 10.24911/SJP.2018.1.6

  • Severe desquamation in Kawasaki disease: Is it somehow protective?
    Jubran Theeb Alqanatish, Amir Babiker
    Sudan J Paed. 2017; 17(2): 56-59
    » Abstract » doi: 10.24911/SJP.2017.2.7

  • Most Cited Articles
  • Anti-diabetic medications: How to make a choice?
    Amir Babiker, Mohammed Al Dubayee
    Sudan J Paed. 2017; 17(2): 11-20
    » Abstract » doi: 10.24911/SJP.2017.2.12
    Cited : 8 times [Click to see citing articles]

  • Broad beans ( Vicia faba ) and the potential to protect from COVID-19 coronavirus infection
    Mutasim I. Khalil, Mustafa A. Salih, Ali A. Mustafa
    Sudan J Paed. 2020; 20(1): 10-12
    » Abstract » doi: 10.24911/SJP.1061585398078
    Cited : 4 times [Click to see citing articles]

  • Challenges in the diagnosis and management of Pediatric Rheumatology in the developing world: Lessons from a newly established clinic in Yemen
    Haifa Ali Bin Dahman
    Sudan J Paed. 2017; 17(2): 21-29
    » Abstract » doi: 10.24911/SJP.2017.2.2
    Cited : 4 times [Click to see citing articles]

  • Pattern of malaria in hospitalized children in Khartoum state
    Hasan Awadalla Hashim, Eltigani Mohamed Ahmed Ali
    Sudan J Paed. 2017; 17(2): 35-41
    » Abstract » doi: 10.24911/SJP.2017.2.4
    Cited : 4 times [Click to see citing articles]

  • Commitment to the wellbeing of children worldwide. [eng]
    Mustafa Abdalla M Salih; Satti Abdelrahim Satti
    Sudan J Paed. 2011; 11(2): 4-5
    » Abstract
    Cited : 4 times [Click to see citing articles]