Supernumerary nostrils together with oesophageal atresia and patent ductus arteriosus

Downloaded from https://journals.lww.com/clindysmorphol by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3z484KQalTVETncwKJTq3oIukANfFw4Ygi6Bwd5rp328= on 08/28/2020 Downloadedfrom https://journals.lww.com/clindysmorpholby BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3z484KQalTVETncwKJTq3oIukANfFw4Ygi6Bwd5rp328=on 08/28/2020 Original article 269

Supernumerary nostrils together with oesophageal atresia

and patent ductus arteriosus

Varim Numanoglu

, Bahri Ermis

, Ahmet Dursun

, Fatih Battal

, Zuhal Ornek

,

Sait Mesut Dogan

and Birol Ugur

Supernumerary nostril is an extremely rare congenital anomaly. It can be unilateral or bilateral and it sometimes occurs in association with other congenital abnormalities. We describe a newborn infant with bilateral supernumerary nostrils together with oesophageal atresia and patent ductus arteriosus, an association that has not been

reported previously in the literature. Clin Dysmorphol

16:269–270 c _{2007 Lippincott Williams & Wilkins.}

Clinical Dysmorphology2007, 16:269–270

Keywords: oesophageal atresia, patent ductus arteriosus, supernumerary nostril

Departments ofa_{Pediatric Surgery,}b_{Pediatrics and Division of Neonatology,} c_{Medical Genetics,}d_{Cardiology and}e_{Ear Nose Throat, Karaelmas University,}

School of Medicine, Zonguldak, Turkey

Correspondence to Dr Bahri Ermis, MD, Division of Neonatology, Department of Pediatrics, Karaelmas University, School of Medicine, 67600 Kozlu, Zonguldak, Turkey

Tel: + 90 3722663674; fax: + 90 372 2610155; e-mail: bahriermis@yahoo.com

Received8 December 2006 Accepted 11 April 2007

Case report

A preterm infant boy (34 weeks) was born by vaginal delivery to a 27-year-old mother (G3P2) who had polyhydramnios during pregnancy. Apgar scores were 5 and 7 at 1 and 5 min, respectively. Neither a family history of congenital anomalies nor parental consanguinity was reported.

At birth, the weight of the infant was 2138 g (50–90th percentile), length was 46 cm (50–90th percentile), and head circumference 32 cm (50–90th percentile). Physical examination showed bilateral supernumerary nostrils, which were situated above the normal nostrils and opened to ipsilateral nasal cavities (Fig. 1). The remainder of the nasal anatomy was normal. The infant

had mild respiratory distress with intercostal retraction and excessive oral secretions. An attempt to pass a nasogastric tube (8Fr) into the stomach was not success-ful. This suggested oesophageal atresia, which was later confirmed by a barium swallow (Fig. 2). Echocardiogram showed a patent ductus arteriosus, which closed following ibuprofen therapy. The child underwent surgery, with primary anastomosis between the two ends of the oesophagus and ligation of the tracheo-oesophageal fistula.

Fig. 1

At birth, the patient has bilateral supernumerary nostrils, which were situated above the normal nostrils and opened into the ipsilateral nasal cavities.

Fig. 2

A barium swallow examination showed a dilated oesophageal pouch.

0962-8827 c 2007 Lippincott Williams & Wilkins

Discussion

Supernumerary or accessory nostrils are a very rare anomaly, with only a few cases reported in the literature. Both unilateral (Tawse, 1920; Onizuka and Tai, 1972; Nakamura and Onizuka, 1987; Williams et al., 1998; Hallak et al., 2001) and bilateral (Lindsay, 1906; Chen and Yeong, 1992) cases have been described, some with other craniofacial malformations, such as a facial cleft. The accessory nostril may or may not communicate with the ipsilateral nasal cavity. Although various aetiologies have been suggested (Lindsay, 1906; Erich, 1962; Nakamura and Onizuka, 1987; Reddy and Rao, 1987), the exact cause is unclear. Lindsay proposed the theory of dichotomy by atavism or parallel evolution (Lindsay, 1906). In 1987, Reddy and Rao suggested that the extranostrils arose as a result of an accessory placode or pit, which may be either above or below the normal nasal placode. In the same year, Nakamura and Onizuka (1987) described a case of supernumerary nostril, and they suggested that the defect was due to a localized abnormality in the lateral nasal process. In 2005, Sinha et al. (2005) reported a case of supernumerary nostril coexisting with congenital cataract, both hypothesized to

be due to a defect in the migration of cells from the frontal prominence.

In summary, we describe for the first time a newborn infant with bilateral supernumerary nostrils, together with oesophageal atresia.

References

Chen MT, Yeong EK (1992). Supernumerary nostrils. Br J Plast Surg 45:557. Erich JB (1962). Nasal duplication. Report of case of patient with two noses.

Plast Reconstr Surg 29:159.

Hallak A, Jamjoom H, Hosseinzadeh T (2001). Supernumerary nostrils: a case report and review. Aesthetic Plast Surg 25:241–243.

Lindsay B (1906). A nose with supernumerary nostrils. Trans Pathol Soc London 57:329.

Nakamura K, Onizuka T (1987). A case of supernumerary nostril. Plast Reconstr Surg 80:436.

Onizuka T, Tai Y (1972). Supernumerary nostril: case report. Plast Reconstr Surg 50:403.

Reddy KA, Rao AK (1987). Triple nostrils. A case report and review. Br J Plast Surg 40:651.

Sinha R, Das S, Sikder B, Ray S, Bit UK (2005). Supernumerary nostril with congenital cataract. Ear Nose Throat J 84:716–719.

Tawse HB (1920). Supernumerary nostril and cavity. Proc R Soc Med (Laryngol) 13:28.

Williams A, Pizzuto M, Brodsky L, Perry R (1998). Supernumerary nostril: a rare congenital anomaly. Int J Pediatr Otorhinolaryngol 44:161.

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