LATERAL PROBOSCIS –A RARE CASE

AUTHOR- Dr. Paresh sodhiya Associate Professor (Neurosurgery)

Dr. Sashi Sankar Sharma Associate Professor (Pedia surgery)

Dr. Sachin Verma Associate Professor (Plastic surgery)

Department of Surgery, M.G.M Medical college and M.Y.

Hospital Indore ,Madhya pradesh

CO-AUTHOR- Dr. Rajkumar Singh Jat

AIM- To share the rare case of lateral proboscis of a PND-0/full term/LSCS delivery/female
/3kg/first child in order/born of non consanguineous marriage.

OBJECTIVE- We present the radiological,sonological, various malformation, pathological

association,embryological aspect,surgical aspect & literature review of lateral proboscis.

INTRODUCTION- Proboscis Lateralis is a very rare congenital anomaly characterized by a

rudimentary tubular, nose-like structure most of the cases arising from the medial portion of the
orbital roof . Lateral proboscis is a rare anomaly, with a reported incidence to be less than
1:10,0000 .
Incidence in male appears to be higher than in females, with a 3:1 male/female ratio. The reason
for this sex difference is unknown [1]. The diagnosis was first described by Foster in his
monograph entitled “Congenital Malformations of the Human body” in 1861. He described an 8-
month-old girl with the lateral proboscis, heminasal aplasia, and cyclopean eye [3]. Later in
1884, Selenkoff reported this anomaly in the autopsy findings of a 34-year old Finnish farmer
[4].
It is a fleshy club like structure with a narrow epithelial lined tract in the center. Its proximal end
is attached in the area of medial canthus.The classification of lateral proboscis suggested by
Koo is the following:
 Group I: lateral proboscis with normal nose (least common);
 Group II: lateral proboscis with an ipsilateral deformity of the nose (second in frequency);
 Group III: lateral proboscis with ipsilateral deformity of the nose, eye and or ocular
adnexa (the most common type);
 Group IV: lateral proboscis with ipsilateral deformity of the nose, eye and or ocular
adnexa, plus cleft lip and/or palate.
 For the complete evaluation of this anomaly CT scan is important which allows assessment of
growth of facial and skull bones as well as CNS development.
Management should start early in childhood to avoid psychosocial consequences related to this
deformity. Complete aesthetic outcome is delayed until late teens when growth of nasal skeleton
is almost complete [5].For the heminose reconstruction, use of proboscis itself is the best option.
Later secondary procedures are required to correct skeleton deformity and groove of the inset of
the proboscis with normal heminose [6].
EMBRYOLOGICAL ASPECT
This condition is caused by the developmental failure or absence of medial and lateral nasal
processes, resulting in fusion of the maxillary process with the contralateral frontal process [1].
The various localization points of PL may be at the embryonic fusion line between the anterior
maxillary process and the frontonasal process, level 1, supramedial canthal area; level 2, medial
canthal area; level 3, inframedial canthal area; level 4, supranostril area (Fig. 1) [2].

PATHOLOGICAL ASSOCIATION
The presence of a proboscis can be associated with several anomalies which include:

 trisomy 13
 holoprosencephaly
 cebocephaly
 ethmocephaly 3
 cyclopia: often below the level of the proboscis
MATERIAL & METHOD
A new born child
All routine investigation

All sonological ,radiological investigation & cardiac evaluation.

CASE REPORT
A. PATIENT INFORMATION- PND-0 /Full term /LSCS delivery/Female/3.0 kg/
First order female child / Born of non consanguinous marriage. Belonging to
lower middle class. .Mother having no history of any chronic illness(DM/HTN) or
intake of associated drugs. Mother has taken Iron Folic acid during ANC. Patient
has passed meconium and urine.
B. LOCAL EXAMINATION- 3×1×1 cm tubular structurefrom medial canthus of
left eye with dimple at tip with few drops of serosanguneous discharge over left
upper eyelid and nose .left external nasal has narrow opening. left eyeball not
visualised .externally deviated right foot. no cleft lip and cleft palate. Gc -avg
afebrile. Pulse 120 /min. Chest -B/l clear .Per abdomen-soft, nontender .CRT <3
secs .CTR =+avg.
FIGURE 1,2,3,4..
 C. RADIOLOGICAL ASPECT- CT imaging are necessary for investigate the asociated
malformation .

CT BRAIN PLAIN- (26/09/2020)

1)Mild hydrocephalus with bilateral colpocephaly and bilateral periventricular

white matter Transependymal CSF seepage.

2).?Corpus callosum agenesis.

3) Elongated soft tissue trunk like exophytic appendage like structure projecting from medial
portion of roof of left orbit-S/o proboscis lateralis.

Figure 1,2,3,4
HRCT THORAX AND ABDOMEN (26/09/2020)

Hemivertebra involving T10 vertebral body causing focal scoliosis with convexity
to right side.

MRI CRANIOSACRAL SPINAL

D- 2D ECHO AND USG WHOLE ABDOMEN(28/09/2020)- DORV/VSD/Mild pulmonary

stenosis/Secondary osteo secundum/ASD/Malposition of great vessel.

No remarkable abnormality seen on USG

E- SURGICAL ASPECT- Proboscis is a typically obvious deformity of the facial structures,
which can be diagnosed at birth or prenatally by ultrasound .The treatment for lateral proboscis is
surgical repair after complete facial growth. However, due to the complex nature of this
malformation, achieving an esthetic result is often a challenge. A prosthetic is suitable
management until final repair and reconstruction are possible. It shows the best colour and
texture match with the opposite normal heminose. Many authors have suggested extirpation of
the proboscis and later reconstruction with various tube pedicle flaps .
There are several nasal reconstruction methods using the proboscis as a donor .The tube has been
used in various ways. Decorticated tube is passed through subcutaneous tunnel made in the
region of absent heminose. Rounded and constricted appearance of the new opening is the
problem with this method. It requires further corrections of nostril with z-plasty. Khoo Boo-
Chai has suggested dilatation of proboscis before starting the reconstruction to avoid narrow
opening and size discrepancies. But this will require repeated dilatations at regular intervals and
proboscis will loose its suppleness. So we find opening the tube and attaching directly to normal
heminose in staged fashion is the best option. Aesthetic correction and framework formation can
be delayed till late teens when Growth of nose has completed.

DISCUSSION
Proboscis lateralis is arare congenitally acquired facial abnormality characterised by soft tissue
tube or trunk like appendages projecting from surface of face,most frequently rooted in the
medial canthus region. Proboscis lateralis typically manifests as a soft, 2- to 3-cm-long and 1-cm
diameter trunk like process originating from the region of the medial canthus. It is
characterized by a central tract lined with squamous and ciliated respiratory epithelium that
typically expresses clear mucus from the blind dimple at its distal end. Although PL has been
described in the absence of other congenital anomalies, it is characteristically accompanied by
ipsilateral heminasal hypoplasia or aplasia and rarely by choanal atresia [4]. Ocular and/or
ocular adnexal findings as well as cleft lip and/or palate are the most common anomalies seen
in conjunction with PL and have been used as the basis for a classification system [5]. Group 1
consists of isolated PL without other associated anomalies (9%). Group 2 consists of PL with
associated ipsilateral nasal defect (23%). Group 3 consists of PL with associated ipsilateral nasal
and ocular and/or ocular adnexal defect (47%). Group 4 includes the features of group 3 with
the addition of cleft lip and/or palate (21%). Embryologic development of the face and nose is a
complex process. The nasal placodes appear to function as the primary organizers for the
developing nose. The nasal placodes invaginate into the frontonasal process, separating this
structure into the medial and lateral nasal processes. The precise embryologic mechanism
responsible for the development of PL has not been defined. Popular theories include imperfect
fusion of the lateral nasal and maxillary processes and aberrant fusion of the maxillary process
of the affected side to the medial nasal process (globular process) [6]. Rontal and Duritz
correctly point out that these theories do not adequately explain the associated anomalies and
suggest a primary insult to the nasal placode as the likely mechanism for PL development

CONCLUSION

In conclusion, Proboscis lateralis is a rare congenital anomaly with a characteristic appearance.

Radiographs and Computed tomography are complementary in determining the extent of the
bony and soft tissue components of the anomaly. Proboscis lateralis is most commonly
associated with developmental anomalies of the ipsilateral nasal and ocular structures.
Complete surgical excision at the base of the proboscis is desirable as a primary procedure if
there is adequate ipsilateral nasal development or as a delayed excision if the proboscis is used
in nasal reconstruction. Definitive cosmetic reconstruction with bone or cartilage should be
planned at a later age keeping in mind the possibility of further growth and development of the
nasal complex.

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